Glomus tumors are uncommon mesenchymal neoplasms originating from modified smooth muscle cells in the glomus body. They are generally small, solitary lesions found in the distal extremities. Rarely, involvement in the abdominal viscera can occur. In such cases, hematemesis/melena and epigastric discomfort are the most common initial symptoms. Although gastric glomus tumors can demonstrate malignant behavior, criteria for identifying malignant potential have yet to be established. We present a rare case of gastric glomus tumor in an otherwise healthy 41-year-old female. The patient initially presented with a significant upper GI bleed requiring a 4 U PRBC transfusion for stabilization. An upper endoscopy with endoscopic ultrasound identified an ulcerated, submucosal mass thought to be consistent with GI stromal tumor (GIST). Once clinically stable, she was scheduled for elective resection. However, prior to resection she experienced a second hemodynamically significant upper GI bleed and underwent emergent laparotomy with distal gastrectomy. Pathologic examination revealed a 3 cm glomus tumor. Gastric glomus tumors are rare solitary submucosal tumors for which preoperative diagnosis is challenging and can be confused with a GIST. Local resection with negative margins is the preferred treatment and the exact diagnosis relies heavily on histopathological examinations. Currently, there are no clear guidelines regarding the staging and malignant potential of glomus tumors of the stomach. Glomus tumors are rare mesenchymal neoplasms that arise from modified smooth muscle cells within the glomus body. They are typically small, solitary tumors and account for approximately 2% of all soft tissue tumors . The gloThe patient is an otherwise healthy 41-year-old female who initially presented with a hemodynamically significant, but self-limited, upper GI bleed. She required 4 units of packed red blood cells in total for stabilization. An upper endoscopy was performed which showed an ulcerated gastric mass . A subseBased on the above imaging findings, the diagnosis was thought to be most consistent with gastrointestinal stromal tumor (GIST) and the patient was scheduled for elective resection. However, on the day prior to her scheduled resection she experienced recurrent upper GI bleeding with hemodynamic instability. An emergent exploratory laparotomy was performed and the antral mass was identified along the greater curvature of the stomach just proximal to the pylorus. It was determined that a simple wedge resection would have likely resulted in gastric outlet obstruction. Thus, a distal gastrectomy with roux-en-y reconstruction was performed. She subsequently had an uneventful hospital course and was discharged home 5 days postoperatively.Interestingly, on pathologic examination the tumor was shown to be a 3 cm, low-grade gastric glomus tumor. All surgical margins were negative. Tumor histology demonstrated a low-mitotic rate (2 mitotic figures per 50 high power field) with no nuclear atypia or lymphovascular invasion . ImmunohThe first case of gastric glomus tumor was described in 1951 with sparse reports in the literature since that time . In one Gastric glomus tumors share many features with other gastric submucosal lesions, making preoperative diagnosis difficult. They are often initially misdiagnosed as the more common GIST, only to be definitively diagnosed by immunohistochemistry on final pathologic specimen. Patients are typically symptomatic at time of presentation, with gastrointestinal bleeding and epigastric discomfort being common . AlthougPostoperative microscopic examination typically demonstrated round, uniform glomus cells within a hypervascular surrounding tissue. Immunohistochemical staining is characteristic for the expression of the mesenchymal markers synaptophysin, smooth muscle actin, laminin, and vimentin. Noticeably absent is CD 117 expression, thus distinguishing glomus tumor from the more common GIST [Given the rarity of disease, there are currently no established guidelines for the staging, optimal treatment, or clinical follow-up for these tumors. The diagnostic criteria for malignant gastric glomus tumor remains controversial. Tumor size, necrosis, mitotic activity, and atypical mitotic features are generally accepted as factors suggesting malignant propensity. Interestingly, both nuclear atypia and lymphovascular invasion are common findings and do not necessarily indicate malignant potential. It has been suggested that size > 5 cm is more predictive of malignant behavior than mitotic rate or nuclear atypia . TreatmeGastric glomus tumors are rare solitary submucosal tumors with a generally good prognosis that may present as an acute GI bleed. They occur in adults of all ages with a significant female predominance and are typically located in the gastric antrum. Preoperative diagnosis is challenging and often impossible. Given the rarity of these tumors, there are currently no established guidelines for staging, treatment, or follow-up. However, local resection with negative margins is generally considered the treatment of choice. Long-term surveillance is recommended given the small but unpredictable potential for malignant behavior.


Primary pulmonary meningiomas represent a rare tumor entity. Few cases have been reported in the English medical literature, and they have almost all been solitary and benign in nature, with the exception of several extremely rare cases. We report herein a case of PPM that raised suspicion of a pulmonary metastatic tumor initially, as it was depicted as a single, round, small, ground-glass opacity pulmonary nodule on a chest computed tomography scan, in a 55-year-old man with a history of buccal cancer. Increased awareness of the clinical and radiologic characteristics of this rare category can assist a multidisciplinary team to perform adequate management.  Primary pulmonary meningiomas (PPMs) are rare tumors and only sporadic cases have been reported. Most PPMs are solitary pulmonary nodules, incidentally detected on chest plain film or chest computed tomography (CT). We report a case of PPM herein, which raised suspicion of a pulmonary metastatic tumor initially.A 55-year-old man was referred to our Chest Department due to abnormal pulmonary nodules located in the left upper and lower lung lobes, which were detected on a chest CT scan. He had a past history of left buccal cancer without distant metastasis more than ten years ago, for which he received surgery and local radiotherapy. He underwent regular follow-up by contrast-enhanced CT scanning of the head and neck without local recurrence. With the exception of buccal cancer, the patient denied any history of other neurologic or pulmonary disorders. He underwent a health examination, and a chest CT scan revealed a 4.5 mm ground-glass opacity nodule in the left upper lobe and a 16 mm part-solid ground-glass nodule in the left lower lobe of the lung . Three mIn histopathological study, the nodular lesion in the left lower lobe of the lung proved to be granulomatous inflammation. Microscopically, the tumor in the left upper lobe of the lung was composed of nests of round to spindle-shaped cells, which presented in a focal whorl pattern . The tumPPM, defined by the typical histological features of meningioma in the absence of CNS lesions, is a rare neoplasm. Only 45 cases were described in the English medical literature to 2015 [Most PPMs present as an asymptomatic solitary lung nodule and are detected incidentally on chest plain film or chest CT. Few cases have been reported to be symptomatic and present with hemoptysis . On chesHistologically, PPMs appear as well-circumscribed tumors without any bronchial or pleural involvement. Microscopically, these tumors usually present with spindle-shaped or ovoid cells arranged in lobules or a whorl pattern . Absenceμm to 3 mm) that maintain the original architecture of lung parenchyma, whereas PPMs present as nodular lesions that substitute for the parenchyma. MPMNs may have a relationship with chronic lung disease, whereas they may not be congenital cases due to absence in pediatric lung specimens [Two hypotheses regarding the pathogenesis of PPMs have been proposed: they may arise from pluripotential subpleural mesenchyma or from minute pulmonary meningothelial-like nodules (MPMNs) . Recentlpecimens . Immunohpecimens ; howeverThe treatment for pulmonary meningioma is most commonly surgical resection. Wedge resection is ideal for peripheral lesions. On the contrary, lobectomy is suitable for lesions located in the central lung . Recent In conclusion, a greater knowledge of the clinical and radiological features of this rare entity is important to achieve more accurate and improved management. PPM should be listed in the differential diagnoses of single or multiple pulmonary nodules, even though it may present as a ground-glass opacity nodule on a CT scan.


Rhinophyma  is a rare, disfiguring disease characterized by a progressive hypertrophy of the soft-tissues of the nose with its increased volume, mainly in the lower half, and often associated to an end-stage of severe acne rosacea. The cause of rhinophyma is unknown, but it is believed to be the end stage of acne rosacea. The diagnosis is easy to ascertain based on the clinical features of the disease. In advanced cases, medical management is believed to be inferior to the results seen with surgical treatment. Surgical management is divided into 3 approaches: full-thickness excision followed by split-thickness skin graft, full-thickness excision followed by full-thickness skin graft, and decortication or partial excision. Decortication techniques include dermaplaning, dermabrasion, cryosurgery, electrosurgery, laser surgery and sharp blade excision. We reporte a case treated by a decortication technique: a 60-year-old man who presented at the dermatology department with a 20-year history of rhinophyma. He had never undergone any laser or surgical interventions. No other medical problem was reported, and there was no family history of rosacea. In collaboration with the Department of Plastic Surgery, rhinophymectomy using a scalpel and electroscalpel was undertaken. The procedure was performed with the patient under general anesthesia. A scalpel with a no. 15 blade was used. The postoperative period was satisfactory. The first signs of skin reepithelialization appeared 2 weeks after the intervention. At a 2 month-follow-up visit, a scar was present on the nasal tip, but the overall nasal contour was restored.


Multiple endocrine neoplasia type 1 (MEN1) is an autosomal-dominant inherited disorder that is classically characterized by the presence of neoplastic lesions of the parathyroid glands, the anterior pituitary gland, and the pancreas. However, MEN1 with concomitant pheochromocytoma is extremely rare.MEN1 gene. He underwent distal pancreatectomy, splenectomy, cholecystectomy, right adrenalectomy, abdominal subcutaneous tumor excision, and cutaneous tumor biopsy for the purpose of tumor volume reduction. Extended right posterior segmentectomy with partial hepatectomy of S2, S3, and S8 was performed to resect residual tumors 9 months after the initial surgery. Although a newly formed liver metastasis was found 19 months after the hepatectomy, he is still alive 4 years and 4 months after the initial surgery.We report a case of MEN1 concomitant with pheochromocytoma. A 44-year-old Japanese man, who had undergone total parathyroidectomy due to primary hyperparathyroidism at the age of 18, was referred to our hospital with a complaint of a large abdominal tumor. He was diagnosed as having a giant insulinoma (maximum diameter 18 cm) in the pancreatic tail, five other non-functional neuroendocrine tumors in the pancreatic body and tail, multiple liver metastases of pancreatic neuroendocrine tumors, a pituitary prolactinoma, non-functional adrenal cortical adenomas, a pheochromocytoma in addition to a subcutaneous neurofibroma, and a cutaneous fibroma. The genetic screening revealed a deletion mutation at codons 83–84 in exon 2 of the We reported an extremely rare case of giant insulinoma and simultaneous occurrence of pheochromocytoma and adrenal cortical adenoma in the ipsilateral adrenal gland in a patient clinically and genetically diagnosed as having MEN1. MEN1 gene was identified in 1997 and is located on chromosome 11q13 encoding a 610-amino acid protein, MENIN [Multiple endocrine neoplasia type 1 (MEN1) is an autosomal-dominant inherited disorder that is classically characterized by the presence of neoplastic lesions of the parathyroid glands, the anterior pituitary gland, and the pancreas . Furthern, MENIN . Insulinn, MENIN . An incin, MENIN , most ofn, MENIN .We report herein a case of MEN1 with a giant insulinoma of the pancreatic tail, non-functional adrenal cortical adenomas, and an ipsilateral pheochromocytoma.2 on admission. He also complained of sleep apnea and bilateral nipple discharge. He was not aware of the hypoglycemic symptoms before the admission, but he frequently encountered the symptoms under nutritional management after the admission. Laboratory data of blood chemistry were normal as well as the tumor markers, such as carcinoembryonic antigen and carbohydrate antigen 19-9, whereas neuron specific γ-enolase was high (19.1 ng/ml). Hormonal examinations revealed a hypoglycemia, a hyperinsulinemia , a hyperprolactinemia (serum prolactin level 871.2 ng/ml), and elevated urinary catecholamines. On an enhanced thin-slice abdominal computed tomography (CT), a large tumor was detected in the pancreatic tail, showing heterogeneous enhancement with partial calcification and cystic components. Its maximum diameter was about 18 cm  identified a heterogeneous pituitary adenoma, 3.1 cm in diameter.A 44-year-old Japanese man was referred to our hospital complaining of a large tumor that had been accidentally pointed out in a medical examination as being an esophageal submucosal tumor. Relevant family history brought to light that his father had been diagnosed as having MEN1. Our patient had undergone total parathyroidectomy and autologous transplantation for primary hyperparathyroidism at the age of 18. His height and weight were 187 cm and 120 kg, respectively, with a body mass index of 34 kg/m8 cm Fig., b. OtheMEN1 gene mutation, were identified within his family. Furthermore, the genetic examination revealed a deletion mutation (g.249_252delGTCT) at codons 83–84 in exon 2. He underwent distal pancreatectomy, splenectomy, cholecystectomy, right adrenalectomy, abdominal subcutaneous tumor excision, and cutaneous tumor biopsy for the purpose of tumor volume reduction. Six tumors in the resected pancreas were diagnosed as being pancreatic NETs. The giant tumor in the pancreatic tail was insulinoma, and NET G2 as the Ki67 labeling index was 4.2 % (Fig. His pedigree diagram is shown in Fig. During the postoperative outpatient follow-up, he developed diabetes mellitus and required treatment with an oral antidiabetic drug. For the treatment of his pituitary prolactinoma, cabergoline was administered. Octreotide therapy for the multiple liver metastases of the NETs began 2 months after the operation. After the ninth administration of the octreotide, three new lesions were detected in the liver on a CT scan. His general condition was judged to be tolerable to undergo liver resection, and the radiological examinations showed that there was no recurrence except the liver. Complete surgical resection of liver metastases was therefore achieved by an extended right posterior segmentectomy with partial hepatectomy of S2, S3, and S8. Immunohistochemical examination revealed that liver metastases were derived from a glucagon-positive tumor of the pancreatic NET. The Ki67 labeling index of these lesions ranged from 1.5 to 5.4 %.Nineteen months after the hepatectomy, a CT scan revealed a small nodule in the S4 of the liver. Twenty-three months after the hepatectomy, gadoxetic acid- or gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid-enhanced MRI demonstrated multiple liver tumors. We started everolimus therapy following a diagnosis of unresectable pancreatic NET recurrence. He is still alive after 4 years and 4 months after the initial surgery, although he is suffering from worsened diabetes mellitus, a pituitary prolactinoma that led to a hemorrhage into the pituitary tumor, and an increasing size of the left nonfunctional adrenal tumor.MENIN gene that predisposes the development of endocrine and non-endocrine tumors with variable penetrance [MEN1 is an autosomal-dominant hereditary syndrome caused by germline mutations of the netrance , 6. The netrance , 6, 7. Tnetrance , whereasnetrance . There anetrance –12, and More than a half of the patients with a giant insulinoma reportedly had a liver metastasis at the time of diagnosis. Liver metastases of the present case were estimated to be derived from the glucagon-positive cells of the pancreatic NET. This was consistent with the consensus that non-functional tumor have a higher incidence of liver metastases than insulinoma . On the An incidence of adrenal involvement in patients with MEN1 is about 20 % , most ofIn conclusion, we reported an extremely rare case of a giant insulinoma and simultaneous occurrence of pheochromocytoma and adrenal cortical adenoma in the ipsilateral adrenal gland in a patient clinically and genetically diagnosed as having MEN1.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent form is available for review by the editor-in-chief of this journal.


We present the case of a 10-year-old boy with the sudden onset of a large, painless left neck mass. Findings on magnetic resonance imaging (MRI) and fine needle aspiration (FNA) biopsy suggest a cystic lesion, most likely of thymic origin. Cervical thymic cysts are a rare form of cervical mass, which are easily overlooked in the differential diagnosis of children presenting with painless neck masses. A combination of CT and MRI investigations can be helpful in differentiating thymic cysts from other congenital and neoplastic masses, but the definitive diagnosis of thymic cyst requires histopathological documentation of thymic tissue. Surgical excision is considered the management of choice for thymic cysts, and no cases of postoperative recurrence have been reported. Cervical thymic cysts (CTC) are epithelial-lined cysts that most commonly present during the first decade of life, with a slight preponderance towards males . Since CCTCs generally present as a slow-growing, painless neck mass, and can be accompanied by a combination of stridor, hoarseness of voice, and/or dysphagia . CT, MRIA 10-year-old boy presented with sudden onset of a large, painless, left neck mass. There was no history of fever, night sweats, malaise, dysphagia, or difficulty breathing. Aside from an upper respiratory tract infection three weeks ago, he had no recent illnesses and no prior episodes of neck swelling or infection. He had a history of pulmonary hypertension but was otherwise healthy. The patient was up to date on all vaccinations. There was no family history of congenital or acquired cystic lesions. Physical examination of the neck revealed a mass on the left, which was firm but not fluctuant, tender, or erythematous. He had full neck range of motion. Based on these findings alone, we were unable to reach a definitive diagnosis and, thus, decided to proceed with magnetic resonance imaging (MRI) of the neck mass. Informed patient consent was obtained from the child's parent.Contrast-enhanced magnetic resonance imaging (MRI) of the neck revealed a peripherally enhancing, multi-septated, cystic lesion that arose in the superior mediastinum and extended into both sides of the neck, deep to the sternocleidomastoid muscles (SCM) bilaterally, and anterior to the carotid sheaths Figure . The lefUltrasound-guided fine needle aspirations (US-FNA) of the cystic and solid lesions in the left neck each revealed a hypocellular, proteinaceous fluid with few histiocytes, degenerating cells, and inflammatory cells, and without evidence of malignant cells. No definitive evidence of thymic tissue containing Hassall’s corpuscles was obtained. After aspiration of cystic fluid was performed, no apparent neck mass was visible. Further therapeutic options were discussed with the patient’s family , and they opted to observe and follow up two months later. At his two month follow-up, no increase in swelling was noted, and the patient reported no pain or decreased range of motion. We plan to follow-up again in six months, or sooner if the mass returns.Cervical thymic cysts are rare, painless neck masses that can present suddenly in children . CTCs caThe differential diagnosis of cervical thymic cyst is broad and includes thyroglossal duct cyst lymphovascular malformations, branchial cleft cyst, and laryngocele, as well as benign tumors  and malignant tumors  . CT can Surgical excision is the management of choice for CTCs, and there have been no reported recurrences after complete excision in the pediatric population . Most CTCervical thymic cysts are a rare form of cystic mass that can easily be overlooked in the differential diagnosis of painless neck masses in children. CT, MRI, and FNA are all helpful investigations in the diagnosis of cervical thymic cysts, but a definitive diagnosis requires identification of thymic tissue containing Hassall’s corpuscles. If accurately diagnosed, cervical thymic cysts can be effectively managed with surgical excision with little chance of postoperative recurrence.


A 45-year-old woman was under study for one year of abdominal pain associated with 4kg weight loss and chronic constipation. An entero magnetic resonance found a mass of 8x10cm on medium ileum suggesting a phytobezoar, with no proximal bowel dilatation. The woman was scheduled for programmed resection. Two weeks after, she presented on our emergency department with an acute abdomen. Exploratory laparotomy showed a bowel perforation of the phytobezoar, and a segmental resection was performed. No more bezoars were found on exploration. She did well after surgery and was discharged home on 6th post-operative day. Pathology revealed a MALT Lymphoma . The overall proliferation index was low with Ki-67 immunoreactivity in approximately 10% of cells. The bone marrow biopsy showed no evidence of involvement by lymphoma. The patient started therapy with rituximab infusions . In two years follow up she remains asymptomatic and without recurrence. Primary ileal MALT lymphoma is rare, and has not been associated with a specific infectious disease. Bezoar is an unusual cause of small bowel obstruction accounting for 0.4-4% of all mechanical bowel obstruction. The common site of obstruction is terminal ileum. We believe that the primary MALT lymphoma caused a small mechanic obstacle that made alimentary fibers to accumulate and cause the phytobezoar. The perforation required a segmental resection, which finally led to the diagnostic of the MALT lymphoma.


Diaphragmatic hernia is a potential complication of esophagectomy, which usually occurs as a hiatal hernia and more frequently after minimally invasive esophagectomy. Parahiatal hernia is a rare form of diaphragmatic hernia, and to the best of our knowledge, parahiatal hernia after esophagectomy has not been previously reported. Here, we report a case of parahiatal hernia after esophagectomy that was successfully managed laparoscopically.A 73-year-old man underwent thoracoscopic esophagectomy for esophageal cancer with gastric tube reconstruction via the posterior mediastinum. Postoperative morbidity was ileus, which required conservative treatment, and intestinal obstruction for which operation with laparotomy was necessary. He was admitted with abdominal pain and vomiting at 15 months after esophagectomy. Abdominal X-ray revealed colon gas in the intrathoracic space. A barium enema examination showed a transverse colon incarcerated in the intrathoracic space. The patient was preoperatively diagnosed with hiatal hernia after esophagectomy, and laparoscopic hernia repair was performed. During the surgery, the hiatus was found to be intact, and the defect was clearly separated from the left crus of the diaphragm. Parahiatal hernia was the operative diagnosis. The incarcerated colon was repositioned in the abdominal cavity, and the defect was repaired using a composite mesh.Laparoscopic surgery was found to be effective for the diagnosis and repair of parahiatal hernia. Diaphragmatic hernia is a potential complication of esophagectomy, which usually occurs as a hiatal hernia and more frequently after minimally invasive esophagectomy. –4. ParahClostridium difficile enteritis was a postoperative morbidity, with hepatic portal venous gas and sepsis on postoperative day (POD) 10. Left pleural effusion was pooled and treated with drainage between POD 11 and 25. The patient recovered and was discharged on POD 28. He was then identified as having intestinal obstruction with volvulus at the site of the removed jejunostomy catheter and underwent surgery with laparotomy to correct this in August 2015.A 73-year-old man was admitted to our hospital with a diagnosis of esophageal squamous cell carcinoma in the middle thoracic esophagus in October 2014. The clinical diagnosis was T4b (left main bronchus) N2M0 stage IIIC carcinoma, according to the seventh edition of the Union for International Cancer Control TNM Classification of Malignant Tumors. Accordingly, he was initially treated by triple induction chemotherapy comprising docetaxel, cisplatin, and 5-fluorouracil. Febrile neutropenia and neutropenic enterocolitis were observed as adverse events. Downstaging to T3N2M0 stage IIIB carcinoma was achieved after two courses of chemotherapy. Thoracoscopic esophagectomy for esophageal cancer with gastric tube reconstruction via the posterior mediastinum was performed in January 2015. An abdominal approach was applied using open laparotomy. A jejunostomy catheter was placed for early postoperative enteral nutrition. Ileus due to Subsequently, the patient was admitted to our hospital having presented with abdominal pain and vomiting in April 2016 (15 months after the esophagectomy). The abdominal X-ray revealed colon gas in the intrathoracic space Fig. . The lefThere are two common forms of esophageal hiatal hernia: “sliding hiatal hernia” and “paraesophageal hernia” . AnotherIt is difficult to diagnose a parahiatal hernia preoperatively. It is difficult to make a distinction clinically between parahiatal and hiatal hernia, and the former is usually diagnosed by intraoperative findings. Scheidler et al. indicated that specific roentgenographic findings helped to distinguish between parahiatal and hiatal hernias, i.e., the position of the intrathoracic herniated abdominal viscus was moved noticeably to the left of the midline . Though C. difficile enteritis after esophagectomy. The weight of the patient was 46 kg upon the first admission to our hospital with a 7-kg decrease during the previous 3 months. Furthermore, the patient showed a weight loss to 40 kg during neoadjuvant chemotherapy preoperatively. Although the patient did not show a drastic weight loss postoperatively, it was thought that he was under prolonged malnutrition, which might have made the tissue fragile. In addition, we thought his ileus might be related to parahiatal hernia formation. The patient underwent emergency surgery on the same day of the onset of bowel obstruction 7 months after esophagectomy. He suffered ileus with pneumatosis intestinalis and hepatic portal venous gas after esophagectomy, and he was not able to ingest a meal for a while. Continuous abdominal pressure due to ileus and subileus might gradually have extended the injured part of the diaphragmatic musculature and might have induced the parahiatal hernia.Usually, diaphragmatic hernia after esophagectomy occurs as a hiatal hernia . We perfIn recent reports, almost all cases of parahiatal hernia were treated by laparoscopic repair of the hernia Table  6, 8–14–148–14. This was a rare case of parahiatal hernia following thoracic esophagectomy involving gastric tube construction via the posterior mediastinum. Laparoscopic surgery was found to be effective for the diagnosis and repair of a parahiatal hernia.


Bezoars are relatively rare foreign bodies of gastrointestinal tract and often cause ileus and ulcerative lesions in the stomach and subsequent bleeding and perforation due to their size and stiffness. Therefore, the removal of bezoars is essential and recent development of devices, the endoscopic removal procedure, is often applied. However, due to their stiffness, simple endoscopic removal failed in not a few cases, and surgical removal has also been used. Recently, the efficacy of a combination therapy of endoscopic procedure and dissolution using carbonated liquid has been reported. To develop the safe and effective removal procedure, we carefully reviewed a total of 55 reported cases in this study including our 3 additional cases, successfully treated with dissolution with endoscopic fragmentation. In summary, the data showed the efficiency in the combination therapy, treating the larger size of bezoar and reducing the length of hospital stay. To the best of our knowledge, this is the largest pragmatical and clinical review for the combination therapy of dissolution and endoscopic treatment for bezoars. This review should help physicians to manage bezoars more efficiently. Bezoars are relatively rare abnormal concretions of indigestible organic material in the gastrointestinal tract, with a reported incidence of 0.07%–0.4% –3. They Bezoars are hard masses comprising indigestible food and vegetable fiber or hair found in the gastrointestinal tract, particularly in the stomach. Although the precise incidence of bezoars is unknown, their reported incidence varies from 0.07% to 0.4%. Bezoars are classified into 4 types according to their contents: phytobezoars, trichobezoars, pharmacobezoars, and lactobezoars [Phytobezoars are caused by the fibers in food, the most common type. The main food types underlying this condition are celery, grape, prune, pineapple, raisins, and particularly persimmons. The tannin in the food reaction with gastric acid resulted in the polymerization and formation of the mass containing various proteins.Trichobezoars are composed of hair and caused by psychiatric disorders including trichotillomania and trichophagia mainly found in the young women. The hairs form the hair ball with mucous and food in the stomach.Pharmacobezoars are caused by medication and its insoluble synthetic compounds, for example, cellulose acetate. The modification on the coating to stabilize the medicine in the stomach using a polymer barrier may lead to the insolubility and be responsible for bezoar formation.Lactobezoars are mainly caused by the undigested milk protein found in milk-fed infants. The number of cases is decreasing; however, the reason is not clarified.Among these bezoars, phytobezoars are the most common type and the number of cases reported depended on the culture of food intake; they are found especially in the east and west Asian countries where the residents have the habit of eating persimmons. Therefore the reports for the management and therapeutic strategies have been reported from these countries with treating various cases. However, it is obvious that any indigestible materials including not only food but also foreign bodies which can cause mass with mucus and so forth can be the etiology of the bezoar.Bezoars can be found in any age groups depending on the types. However, as the frequency of the phytobezoars is much higher than the other types of bezoar, they tend to be found in the adults . Among 5The treatment modalities for bezoars include endoscopic therapy with fragmentation, dissolution using carbonated liquids, medical treatment involving enzymatic dissolution, and surgery . The enzWith the report of successful treatment of gastric bezoar using endoscopic procedure and carbon oxide fluid, we carefully reviewed a total of 55 patients, including our 3 additional patients, treated by dissolution with (combination therapy) or without endoscopic fragmentation (dissolution monotherapy) , 19–34  to develWe evaluated age, gender, medical history, and associated complications . Median In addition, to evaluate the usefulness of combination therapy, we retrospectively compared the etiology, total administration amount of carbonated liquids, endoscopic procedure time, length of hospital stay, and outcomes between dissolution and combination therapy . There aOver recent years, drinking green juice and eating healthy foods containing tannin have become very popular for fitness. However, we should keep in mind that an excessive consumption of tannin can lead to the formation of a phytobezoar. In the combination group, the average administration amount of carbonated liquids was 4,456 mL . This difference might be due to the size of bezoars, since the median size of bezoars was approximately 4.5 cm in dissolution monotherapy, whereas it was 7.0 cm in combination therapy group . However, as the limitation of this literature review, the size of each bezoar was estimated as an occupied percentage of the stomach lumen in some patients; therefore, we could not evaluate accurate measurements . The aveIn terms of outcomes, one patient  underwent surgical removal following the dissolution monotherapy because of intestinal obstruction owing to fragmented bezoars arising because of the procedure using Coca-Cola  reportedA 63-year-old Japanese woman visited a hospital with anorexia and heart burn. She had no history of gastritis or any other disease, except appendicitis. Upper gastrointestinal endoscopy revealed a huge bezoar in the patient's stomach, which was not detected in a 1-year prior examination. The patient was referred to our hospital. Her physical examination revealed upper abdominal tenderness and laboratory results were within normal ranges. The patient reported a habit of drinking green juice extracted from vegetables every day. This juice contained a high volume of catechin, a type of tannin.Upper gastrointestinal endoscopy revealed a black-colored bezoar measuring approximately 5 cm in the greater curvature of the gastric body, with no ulcers or other complications in the gastrointestinal tract . Next, wA 70-year-old Japanese man underwent annual gastrointestinal endoscopy as a follow-up screening process after a distal gastrectomy performed 15 years prior for gastric cancer. He was referred to our hospital because the upper gastrointestinal endoscopy revealed a huge bezoar in his remnant stomach. He had no symptoms and laboratory results revealed no abnormal findings upon admission. However, he had consumed 2-3 persimmons every day for 3-4 months from autumn to winter in the previous year.There were no ulcers or other complications in his remnant stomach, and a black-colored bezoar measuring approximately 5 cm was observed in the greater curvature of the upper gastric body . The stiAn 80-year-old Japanese man underwent endoscopy to investigate symptoms of epigastric discomfort over the previous month. Examination revealed the existence of a huge bezoar and he was referred to our hospital. His physical examination revealed mild epigastric pain and tenderness and laboratory results were within normal ranges. He also had consumed persimmons over the previous year. Endoscopy showed a hard, black-colored bezoar measuring approximately 4 cm in the stomach complicated with a gastric ulcer (A2 stage) in the lesser curvature of the gastric angular area Figures . We collBezoars are hard masses comprising indigestible food and vegetable fiber or hair found particularly in the stomach. They are classified into 4 types according to their contents . PhytobeIn this study, we reviewed a total of 55 patients, including our 3 additional patients, treated by dissolution with or without endoscopic fragmentation , 19–34 . In term in vitro. Small pieces of bezoar were laced into individual tubes containing different carbonated liquids. In case 2, Juice X had the most superior solubility among the liquids tested. The bezoar was softened and fragmented easily following the administration of Juice X at a dose of 500 mL every day for 2 days. Diospyrobezoars are often resistant to dissolution because of their stiffness and are usually endoscopically or surgically removed. In the present study, the diospyrobezoars found in case 3 were treated using the combination therapy. However, the contribution of the endoscopic procedure is relatively better using various devices. This experience suggests that it may be effective and reasonable to determine the therapeutic strategy for each phytobezoar to investigate the therapeutic effect of dissolution therapy by several carbonated liquids in advance. Differences between liquids reflect differences in the concentration of carbon dioxide, pH value, and contents such as sugar, thereby contributing to differences in solubility, although it is not disclosed from the company. It is reasonable to determine the best carbonated liquid prior to the procedure to reduce the hospital stay, the procedure time, and risks of the procedure, leading to the safe and effective removal. Further cases are necessary to be studied as randomized prospective study.In our representative 3 cases, we investigated the solubility of bezoars by testing various carbonated liquidsWe carefully reviewed 55 cases including our 3 cases of gastrointestinal bezoars to summarize the knowledge and data for developing the safe and efficient therapeutic strategy. The data showed the efficiency in the combination of dissolution therapy using carbonated liquid and endoscopic procedure which resulted in the shorter period of the hospital stay. Therefore the selection of the carbonated liquid prior to the intervention will further help patients to be treated in safe and efficient manner by reducing the endoscopic procedure time. In conclusion, the results of our literature review should help physicians to treat gastric bezoars in a much more effective manner.


Pancreatic acinar cell carcinoma (PACC) is a rare tumor. Surgical resection is the treatment of choice when feasible, but there are no clear recommendations for patients with advanced disease. Liver-directed therapy with Y-90 selective internal radiation therapy (SIRT) has been used to treat hepatic metastases from pancreatic tumors. We describe a case of PACC liver metastases treated with SIRT. 59-year-old man was admitted with an infiltrative, solid lesion in pancreatic tail diagnosed as PACC. Lymph nodes in the hepatic hilum were enlarged, and many metastatic liver nodules were observed. After partial pancreatectomy, the left and right lobes of the liver were separately treated with Y-90 resin microspheres. Follow-up imaging revealed that all hepatic nodules shrank by at least 50%, and 3 nodules disappeared completely. Lipase concentration was 8407 U/L at baseline, rose to 12,705 U/L after pancreatectomy, and declined to 344 U/L after SIRT. Multiple rounds of chemotherapy in the subsequent year shrank the hepatic tumors further; disease then progressed, but a third line of chemotherapy shrank the tumors again, 16 months after SIRT treatment. SIRT had a positive effect on liver metastases from PACC. In conjunction with systemic therapy, SIRT can achieve sustained disease control. Pancreatic acinar cell carcinoma (PACC) is a rare tumor that accounts for approximately 1% of malignant pancreatic neoplasms –4.Selective internal radiation therapy (SIRT) with yttrium-90 (Y-90) resin microspheres is an alternative treatment for patients with primary or secondary liver malignancies not amenable to resection , 6. DuriIn the context of pancreatic cancer, SIRT has been used as a salvage therapy , 12 or iA 59-year-old man underwent a transabdominal ultrasound to investigate persistent postprandial abdominal pain and was admitted to the hospital with liver nodules of unknown cause. Most laboratory values were close to normal, including the tumor markers alpha-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen 19-9; however, serum lipase concentrations were elevated .An abdominal, contrast-enhanced computed tomography (CT) scan and magnetic resonance imaging (MRI) showed multiple, unresectable hypovascular liver nodules in both lobes Figures . The lar68Ga-DOTATATE) revealed no lesion suggestive of a tumor highly expressing somatostatin receptors, which excluded a diagnosis of well-differentiated neuroendocrine tumors. No extrahepatic metastases were evident.Positron emission tomography- (PET-) CT with 18-fluorodeoxyglucose (18-FDG) showed that the pancreatic and hepatic lesions were hypermetabolic, with a maximum standardized uptake value of 8.5. An ultrasound-guided biopsy of the largest hepatic nodule was performed on the same day as the PET-CT, and the histopathologic analysis was suspicious for PACC. Whole-body PET-CT with a somatostatin analog  decided to resect the primary tumor and treat the liver metastases with SIRT. Systemic chemotherapy was contraindicated because the patient was living abroad and would not be able to attend follow-up appointments in our country. Pathologic (microscopic examination) analysis of the resected pancreatic body and tail showed that the tumor was characterized by marked cellularity and a paucity of fibrous stroma. The neoplastic cells were arranged in solid nests and in some areas formed an acinar arrangement , with roμm, SIR-Spheres; Sirtex Medical Limited, North Sydney, Australia) were delivered to the right hepatic lobe, and 1 month later microspheres were delivered to the left hepatic lobe and a right hepatic artery branch to segments IV/VIII.The patient then underwent SIRT to control the secondary liver lesions. Because of the extent of the liver lesions, SIRT was performed in 2 stages. First, Y-90 resin microspheres (20 to 60 http://apps01.sirtex.com/smac/) [In the first SIRT treatment, a pulmonary shunt of 6.5% was detected. We used the Dose Activity Calculator (m/smac/) , 18 to cIn the second SIRT treatment, a pulmonary shunt of 5% was detected, and a dose of 0.25 GBq (6.7 mCi) was delivered through the right hepatic artery branch to segments IV and VIII (to treat the remaining portion of segment IV), and 0.65 GBq (17.5 mCi) was delivered through the left hepatic artery Figures . A PET-CAfter both SIRT treatments, the patient presented with Grade 1 fatigue that lasted approximately 1 week. Measurement of liver enzymes indicated that no liver damage had occurred (data not shown). Serum lipase concentrations progressively decreased from a high of 12,705 U/L to 344 U/L , indicatThe patient returned to the United States, where he has been under the care of an oncologist at the M. D. Anderson Cancer Center in Houston, Texas. Beginning 6 weeks after the second SIRT , he was treated with folinic acid-fluorouracil-oxaliplatin (FOLFOX) chemotherapy every 2 weeks for 6 cycles. Imaging on April 7, 2016, showed improvement in the hepatic lesions that did not reach the criteria for a partial response, and the FOLFOX treatment was continued. In July 2016, the hepatic lesions progressed; FOLFOX was discontinued, and chemotherapy with capecitabine was initiated. An abdominal MRI 82 days later showed hepatic lesions of 3.3 cm, 1.2 cm, and 1.0 cm in segment II and a 1.5 cm lesion in segment VII. The volume of the right hepatic lobe had decreased from April 7, and multiple millimeter-sized subcapsular and peritoneal lesions were evident. Chemotherapy with capecitabine was continued. Three weeks later, PET-CT confirmed the hepatic lesions and further indicated peritoneal carcinomatosis and involvement of the abdominal and retroperitoneal lymph nodes. A few days later, a CT-guided biopsy was performed, after which the chemotherapy regimen was changed to chronomodulated bevacizumab plus irinotecan with fluorouracil and folinic acid (FOLFIRI). To date, 2 cycles of bevacizumab plus FOLFIRI have been administered. CT images obtained on December 16, 2016 , indicated that the tumors had shrunk considerably . The patPancreatic acinar cell carcinoma is a rare neoplasm, representing 1% to 2% of exocrine pancreatic neoplasms in adults , 20. AltIn PACC, the tumor cells bear a morphological resemblance to acinar cells and express pancreatic enzymes such as trypsin, lipase, chymotrypsin, and amylase , 2. The Surgical resection is associated with longer survival and is therefore always recommended when feasible, although it is rarely curative . In our SIRT with Y-90 resin microspheres is a relatively new treatment modality that has been effective in treating liver metastases from several locations, such as the cervix, breast, lung, colon, rectum, and pancreas –10. In 1In our patient, imaging showed multiple hypovascular liver nodules in both lobes, with no extrahepatic metastases, suggesting that he would be a good candidate for SIRT to attempt control of the liver disease. Follow-up imaging after SIRT revealed that the treatment was effective: all hepatic nodules were reduced in size by at least 50%, and 3 nodules disappeared .Lipase hypersecretion syndrome occurs in a minority of patients with PACC, often in association with hepatic metastases, but can resolve with treatment . Our patChemotherapy with FOLFOX initiated after the SIRT initially shrank the tumors further, but the patient's disease subsequently progressed. Capecitabine treatment did not reverse the progression; however, 2 cycles of chronomodulated bevacizumab plus FOLFIRI dramatically shrank the tumors again, as shown with CT imaging 16 months after the first SIRT treatment.SIRT was successful in reducing the burden of hepatic metastases from PACC; however multiple lines of systemic chemotherapy were needed to sustain the improvement as to treat also the lymph nodes at the hepatic hilum.


Angiomyolipoma (AML) is a tumor composed of variable proportions of adipose tissue, spindle and epithelioid smooth muscle-like cells and abnormal thick-walled blood vessels which are usually benign. AML is relatively common in kidney and liver. Gastric angiomyolipoma (GAML) is extremely rare and only 2 cases were reported in the literature. Diagnosis of AML is difficult owing to its rarity and varied immunohistochemical patterns. Here, we report a case of GAML in a 65-year-old male who presented with an episode of hematemesis and intermittent melena for one week. Endoscopy showed a partially obstructing mass arising from gastric antrum with central ulceration. Mass was removed by wedge resection after laparoscopic anterior gastrectomy. Excised specimen showed a benign 6 × 3 × 3 cm homogenous fatty mass with adipose tissue, smooth muscles and prominent vascularity. Immunohistochemistry stains were positive for desmin, smooth muscle actin (SMA), CD34 and negative for human melanoma black (HMB)-45 antigen and CD117. This case reports the largest and HMB-45 negative GAML so far, which can be a very rare cause of upper gastrointestinal bleeding. Angiomyolipoma (AML) is a relatively rare neoplasm, composed of variable proportions of adipose tissue, spindle and epithelioid smooth muscle-like cells and abnormal thick-walled blood vessels . Though A 65-year-old male presented with an episode of hematemesis and intermittent melena for one week. He reported some stomach upset with decreased appetite, dizziness, and exertional shortness of breath lately. As per his wife, pt also had an episode of syncope, the day before admission. He was diagnosed with diverticulitis a week before, when he presented with fevers, chills, left lower abdominal pain, and was started on oral cephalexin and metronidazole, which he was on day 6 of the antibiotics at the time of presentation. He reported increased use of ibuprofen since he had the diverticulitis, using up to four tablets of 200 mg daily. He denied having any fevers, chills, weight loss or other sites of bleeding. His past medical history included hypertension, hyperlipidemia, cholelithiasis and Abdominal Aortic Aneurysm (AAA). Past surgical history included bilateral visceral hernia repair and vasectomy. There was no history of heart burn or reflux disease. He was a previous smoker with fifty plus pack year smoking history and quit smoking 10yrs ago, consumed alcohol socially. He denied recreational drug use. Family history included coronary artery disease on both sides of the family. He was only on simvastatin at home and did not have allergies to any medications.In the emergency room, he was hemodynamically stable. Nasogastric lavage showed some coffee ground emesis. On physical exam, he was well oriented, chest and heart examination was normal. Abdominal examination showed mildly hyperactive bowel sounds, otherwise normal. No organomegaly was noted. Heme occult was positive. Admission lab work showed hemoglobin of 12.4 mg/dL with hematocrit of 38. Complete metabolic panel was normal. Patient was given nothing by mouth, started on intravenous fluids and protein pump inhibitor (PPI) drip. It was thought to be non-steroidal anti-inflammatory drug (NSAID) related gastropathy or peptic ulcer related bleeding and ibuprofen was stopped. Overnight, he had couple of episodes of melena resulting in hemoglobin drop and needing blood transfusion. Computerized tomography (CT) scan of abdomen/pelvis with contrast that was taken at other hospital for diverticulitis incidentally showed a gastric mass, gall stones, stable AAA . No otheSurgical team was consulted and resection of gastric mass was planned. On laparoscopy, a mass was seen in the distal stomach which appeared to be intraluminal only. Through a small upper abdominal incision, anterior gastrostomy was done and the mass was delivered through the opening. Mass was entirely within the wall of stomach with overlying ulcer. A submucosal resection of the mass was done and sent for frozen section analysis. Intra operatively, it was consistent with intramural lipoma. Gross evaluation of the specimen revealed a homogeneous yellow-tan colored submucosal fatty mass measuring 6 × 3 × 3cm. There were no areas of hemorrhage or necrosis. Microscopic examination showed a circumscribed nodule composed primarily of mature adipose tissue with prominent vascularity . There wAngiomyolipoma (AML) is a relatively rare neoplasm, composed of variable proportions of adipose tissue, spindle and epithelioid smooth muscle-like cells and abnormal thick-walled blood vessels . Though In the gastrointestinal tract, other than in liver, it was reported in duodenum, appendix, pancreas and stomach , 4. GastEUS is the choice of investigation for the evaluation of SMTs , 10. BasThe pathological diagnosis of AML depends on demonstrating three cell lines namely blood vessels, epithelioid or spindle smooth muscle, and mature adipose tissue. The immunohistochemistry profile of AML is very variable. The monoclonal antibody which was previously raised against melanoma cell line, HMB-45 positivity is considered to be the most specific and characteristic feature of AML , 13. HMBIn the first reported case by Helwig et al, patient presented with upper gastrointestinal bleed which showed 2 × 3 cm mass with central ulceration on endoscopy. The second case by Aggarwal et al, was a 40-year-old female who presented with vague abdominal pain, intermittent melena, hematemesis and found to have a 5 × 4cm polypoidal mass along the greater curvature of stomach. In the second case, tumor was positive for HMB-45 antigen and other immunohistochemical stain patterns were not reported. Laparotomy with excision of tumor was done in the first case where as laparoscopic wedge resection was performed in the second case.Gastric angiomyolipoma (GAML) is a tumor with distinct histopathological pattern which can be a very rare cause of upper gastrointestinal bleeding. Continued reporting of these cases is important to understand clinicopathological characteristics and malignant potential. So far, GAML is a benign tumor and complete resection is curative.


Pelvic dystopia of rudimentary multicystic dysplastic kidney as a rare cause of bedwetting in children.We report the case of a 14-year-old Kazakh girl who presented with difficulty in starting the stream of urine and intermittent interruption of the urinary stream while voiding as well as bedwetting, caused by a rare congenital disease (pelvic dystopia of rudimentary multicystic dysplastic kidney). The diagnostic workup, differential diagnosis, and management, and a review of the literature are presented. Persistent since she was 2 years old, bedwetting was stressful for both the parents and child. Initially detected radiologically and endoscopically, a bladder mass was thought suspicious for ureterocele, papilloma, or mixed tumor of the urinary bladder, but surprisingly, turned out to be a pelvic dystopia of the rudimentary multicystic dysplastic kidney. Transvesical excision of this mass was performed.The purpose of this case report is to draw attention to the fact that a persistent case of bedwetting which does not respond to conventional therapy should be subject to further examinations to exclude surgical causes of the disease. Nocturnal enuresis (bedwetting) is defined by the National Institute for Health and Care Excellence (NICE) guidelines as the involuntary wetting during sleep without any inherent suggestion of frequency of bedwetting or pathophysiology . BedwettWe report a rare case of a 14-year-old Kazakh girl with persistent bedwetting which did not respond to conventional therapy for a long period of time. This case was a diagnostic and therapeutic challenge for us because diagnostic radiology and endoscopy were suspicious for ureterocele, papilloma, or mixed tumor of the urinary bladder. In this case, only through a surgical procedure and histological examination was the true cause of bedwetting found. Therefore, good collaboration between surgeons and pathologists is essential for an accurate diagnostic process.In such cases of obscure bladder mass that cause persistent bedwetting, transvesical excision is preferable.We searched for information in sources such as Medline, Embase, and the Cochrane Database of Systemic Reviews using search terms causes of “enuresis” and “bedwetting” as keywords and did not find any reports apprising that dystopia of rudimentary multicystic dysplastic kidney can be the cause of bedwetting.A 14-year-old Kazakh girl who had ongoing difficulties in starting the stream of urine and intermittent interruption of the urinary stream while voiding, and bedwetting since her early years (persistent since she was 2 years old), which was stressful for both the parents and child.Since that time, the girl has been treated by pediatricians using traditional methods  without any success.Her past medical history included regular medical checkups because of agenesis of her right kidney and pelvic ectopy of the single left kidney, a cyst of the bladder, and bedwetting. She had never had hematuria. Surgical interventions had not taken place. She is the second child of five in her family. The other family members do not have health problems.On physical examination she was well. Her blood pressure was 100/60 mmHg. Her pulse was 82 beats/min. A urine analysis was normal. Her renal function was normal with a creatinine of 90 μmol/L. Examination of other systems revealed an agenesis of her vagina.3, and on the right side of the wall a cystic mass 23 × 20 mm in size was visualized (ureterocele?). A contrast media computed tomography (CT) scan revealed agenesis of her right kidney, pelvic ectopy of her left kidney; on the right side of the urinary bladder there was a cystic mass 24 × 17 mm in size ; and agenesis of her uterus and vagina (Mayer-Rokitansky-Küster-Hauser syndrome) was also detected  cystography shows a shadow of reduced density on the right side of the urinary bladder wall 23 × 20 mm in size   of the bladder cause bladder irritation with symptoms of lower urinary tract obstruction and bedwetting. However, we considered the possibility of a relationship of this mass with the right kidney agenesis, because of its location in the same area of the missing right ureteric orifice, so we decided to perform transvesical surgery rather than transurethral surgery.Transvesical access found a round-shaped mass, 24 × 20 mm in size, located on the right extremity of the interureteric ridge, covered by normal mucous membrane of the bladder. This mass was located close to the bladder neck and was protruding into the internal urethral meatus Fig. . We discAfter removing the urethral catheter (at 48 h), our patient was free from her symptoms of bedwetting, difficulty in starting the stream of urine, and intermittent interruption of the urinary stream while voiding.In order to study the tissue with a light microscope, the specimen was fixed in formalin 10%), cut into sections of 5 μm and stained with hematoxylin and eosin. At histology Figs. , and 10,%, cut inAt 3 year, 8.5 month follow-up of the patient, she had no recurrence of bedwetting, difficulty in starting the stream of urine or intermittent interruption of the urinary stream while voiding. She was followed up with clinical and radiological examinations. Retrograde cystogram  showed normal urinary bladder, no cystic mass was seen Fig. .Fig. 11RUsually, most cases of bedwetting are successfully treated by traditional methods of treatment.We have presented a case of a very rare patient with multiple congenital malformations of the urinary and genital system , in which pelvic dystopia of right rudimentary multicystic dysplastic kidney caused bedwetting, difficulty in starting the stream of urine, and intermittent interruption of the urinary stream while voiding. We searched Medline, Embase, and the Cochrane Database of Systemic Reviews using the search terms causes of “enuresis” or “bedwetting” as keywords, and found no report about dystopia of rudimentary multicystic dysplastic kidney being the cause of bedwetting.Usually, most common urological causes of bedwetting are: overactive bladder  or dysfuMost studies indicate that children with more severe form of enuresis are more likely to have persistent problems  and treaüster-Hauser syndrome  is a group of associated findings that may include vaginal aplasia, uterine maldevelopment, and normal ovaries [In 1993, 441 patients with multicystic kidney were registered [ ovaries . Unilate ovaries . Macrocy ovaries . The met ovaries . But in However, the literature does not describe cases where the pelvic dystopia of rudimentary multicystic dysplastic kidney was a cause of bedwetting, which makes our case a rare one.When conventional methods of treatment of nocturnal enuresis in children are ineffective, other causes which require surgical intervention should be excluded. One of the very rare causes may be dystopic rudimentary multicystic dysplastic kidney, which can be successfully treated by transvesical excision.In such difficult cases, where radiologic and endoscopic findings (bladder mass) mislead clinicians with ureterocele, papilloma, or mixed tumor of the urinary bladder, collaboration between surgeons and pathologists is very important when accurate diagnostic search work is required.


Osseous lesions of the tongue, also referred to as osseous choristomas, are benign growths of bony tissue. These lesions are not true neoplasms but rather represent growth of normal tissue at an abnormal location. Clinically, they appear as exophytic masses of the tongue, and they are treated by surgical excision. Lingual osseous choristomas are rare entities, with only 71 reported cases in the literature. We present the case of a lingual osseous choristoma of the tongue base in a 21-year-old female. Of the cases of lingual osseous choristoma reported in the literature, ours is only the fifth case to involve this location. Osseous lesions of the tongue are an extremely rare benign entity, of which there have only been 71 documented cases . InitialA 21-year-old female was referred to the ENT department at Columbia University Medical Center for a “bothersome” lesion of her tongue. The patient's medical history was noncontributory; however, she did report that she was a current everyday smoker. No history of intraoral trauma was reported.Fiberoptic examination revealed a pedunculated, smooth, dome-shaped mass of the tongue base. The mass measured approximately 4 to 5 mm in size and was located right of the midline . The remAt the time of writing, the patient is one month post surgical treatment. At the one-month clinical follow-up visit post surgery, the patient was symptom free and seemed to be recovering well. She will be reevaluated again at 3 months post surgery.Including the current case, there have only been 72 reported instances of lingual osseous choristoma –4. GorinLingual osseous choristomas vary in size from 3 mm to 5 cm and present clinically as hard masses that can be either pedunculated or sessile. In most cases, the covering mucosa exhibits a normal clinical appearance , 7. LesiThe etiopathology of this entity is still unknown, but several theories have tried to explain its pathogenesis. Two hypotheses in particular are widely recognized. The malformation hypothesis argues that the lesion arises as undifferentiated mesenchymal cells at the line of fusion between the developing first and third branchial arches , 8. On tThe differential diagnosis of osseous choristoma is large and may include benign tumors of nerve or soft tissue, thyroglossal duct cyst, lingual thyroid, mucocele, pyogenic granuloma, and malignant tumors , 9. Howe


Pulmonary visceral subpleural hematoma is rare. We report visceral subpleural hematoma of the left diaphragmatic surface following left upper division segmentectomy. This very rare case was difficult to distinguish from thoracic abscess.A 68-year-old man with hypertension had undergone video-assisted thoracoscopic left upper division segmentectomy for suspected lung carcinoma. Deep vein thrombosis of the lower leg was identified and edoxaban, a so-called novel oral anticoagulant, was started on postoperative day 7. The chest drainage tube was removed on postoperative day 12 because of persistent air leakage, but fever appeared the same day. Computed tomography revealed a cavity with mixed air and fluid, so antibiotics were started on suspicion of abscess. Computed tomography-guided drainage was attempted, but proved unsuccessful. Fever continued and surgical investigation was therefore performed. Visceral subpleural hematoma was identified under the diaphragmatic surface of the left basal lung. We excised the pleura, then performed drainage and applied running sutures. The parenchyma and visceral pleura were covered with polyglycolic acid sheet and fibrin glue. Edoxaban was restarted on postoperative day 12 of video-assisted thoracoscopic surgery and no recurrence of hematoma has been revealed.Visceral subpleural hematoma after thoracic surgery is extremely rare. Furthermore, correct diagnosis was difficult and surgery offered a good diagnostic and therapeutic procedure. Pulmonary visceral subpleural hematoma is rare, and has also been reported as pulmonary pseudocyst –4. TheseThe patient was a 68-year-old man with hypertension. He was not receiving anticoagulants and had no history of smoking. Chest X-ray in a regular check-up revealed an abnormal chest shadow and he was referred to our department. A 30-mm ground glass nodule in the left upper lobe S1 + 2) was identified on chest computed tomography (CT) and lung adenocarcinoma was suspected  was initiated. On POD4, we changed the drainage suction water seal. Follow-up ultrasonography of the lower extremity revealed new calf-limited deep vein thrombosis on POD7 and the novel oral anticoagulant edoxaban was started. The thoracic drain was clamped on POD10 and removed on POD12. Fever of 38°C appeared the same day, but no other symptoms were identified. A cavity appeared in the left lower lung field on chest X-ray  was performed. Inferior pulmonary ligament was not sectioned. Air leakage was observed on postoperative day (POD)2 and continuous low-pressure negative suction (−5 cmHa-2 was pOn POD2 after the second operation, the drainage tube was removed. The patient was discharged on POD8. Cultures of fluid and pleural effusion yielded negative results. Edoxaban was restarted on POD12, but no recurrence of hematoma has been found over one year of follow-up in the outpatient clinic Fig. .Fig. 5ChWe reported a very rare case of postoperative visceral subpleural hematoma. Diagnosis was difficult and surgery was required. Subpleural hematoma is classified as either parietal subpleural hematoma or visceral subpleural hematoma. Hematomas of the parietal pleura account for most reports of subpleural hematoma. Visceral pleural hematoma was reported in three of 98 patients who underwent preoperative CT-guided hook wire localization . VisceraThe chest X-ray appearance of visceral subpleural hematoma sometimes resembles that of lung abscess, cavitating tuberculosis, or bronchial carcinoma with cavitation in adults . In partPostoperative visceral subpleural hematoma distant from the operative site is extremely rare. Furthermore, correct diagnosis was difficult. Surgery is a good diagnostic and therapeutic procedure.


Background. Morbidly adherent placenta (MAP) is increasing in incidence and is commonly associated with maternal hemorrhage and cesarean hysterectomy. Uterine artery embolization (UAE) may be utilized in the conservative management of placenta percreta to potentially reduce blood loss. The incidence of complications from UAE in the conservative management of placenta percreta is poorly described. To our knowledge, we present the first reported case of buttock necrosis in this setting. Case. A 39-year-old gravida nine para two with placenta percreta who underwent conservative management with UAE complicated by right buttock necrosis. Conclusion. While UAE may potentially decrease blood loss, it is not without risk. More studies must be performed in order to quantify those risks and determine the clinical utility of UAE.  Morbidly adherent placenta (MAP) is a spectrum of disorders caused by the abnormally deep invasion of placental tissue into the uterine myometrium. It includes placenta accreta, increta, and percreta and is thought to be due to myometrial scarring and abnormal vascularization following uterine surgery, which leads to excessive trophoblastic invasion . While hSuccessful management of MAP hinges upon timely antenatal diagnosis and planning, as well as optimization of surgical management. In the management of placenta percreta, operative morbidity may be decreased by leaving the placenta in situ . DelayedDetermining the clinical utility of UAE in the conservative management of MAP is challenging. Presently there are no evidence-based guidelines on its role and the little data that exists is inconclusive . InformaA 39-year-old gravida nine para two was referred at 30 weeks of gestation for evaluation of MAP. Her history included two uncomplicated low transverse cesarean deliveries, four curettage procedures for pregnancy termination, and two spontaneous abortions. Pelvic imaging showed an anterior placenta with obliteration of the uterine interface, presence of intraplacental vascular lacunae, and increased vascularity proximal to the bladder and loss of the myometrial margin. Findings were suspicious for placenta percreta possibly involving the urinary bladder. The patient was counseled extensively regarding the management of MAP, including conservative management and its associated risks, and comprehensive informed consent was obtained. Planned delivery at 35 weeks and 6 days of gestation occurred under general anesthesia. Immediately prior to delivery, the patient underwent uncomplicated placement of bilateral hypogastric artery balloon catheters and bilateral ureteral stents. At the time of stent placement, systematic inspection of the bladder was noted to be normal on cystoscopy. Intraoperatively, an area of tortuous blood vessels on the uterine serosa was noted extending to a ballooned right broad ligament and to the border of the bladder reflection on the left side, confirming a placenta percreta with broad ligament and possible bladder invasion . Given tShortly after arrival to the postanesthesia care unit the patient reported buttock numbness but was otherwise asymptomatic. The epidural catheter was removed on postoperative day one and shortly thereafter she reported a burning sensation in the right buttock. Physical exam revealed a tender 9 × 11 cm area of ecchymosis in the medial aspect of right buttock with extension to the gluteal crest. Given recent embolization, there was concern for end-artery compromise and potential soft-tissue and skin necrosis. Interventional radiology, vascular surgery, and plastic surgery teams were consulted. Computed tomography angiogram demonstrated patent internal iliac arteries with patent proximal pelvic branches. Over the following week the lesion blistered, necrosed, and unroofed to reveal healthy tissue beneath. The lesion was treated with topical silver sulfadiazine cream and pressure relief. The patient was discharged home on postoperative day seven with close outpatient follow-up.The patient was planned for interval hysterectomy at six to eight weeks postpartum but was briefly lost to follow-up. Shortly thereafter, she presented and was admitted to the hospital when she noted umbilical cord tissue prolapsing from the vagina. She subsequently underwent hysterectomy at ten weeks postpartum . At thatWith the increasing incidence of MAP and associated complications, substantial effort has been made to determine optimal management strategies. Leaving the placenta in situ is one strategy that can be employed in the case of placenta percreta when intraoperative findings indicate a high risk of operative complications. This may allow for a decrease in placental vascularity, which can lower the risk of operative complications at the time of both delivery and delayed hysterectomy . UAE is Planned UAE is most often performed using prepared embolic agents. Agent selection depends on vessel size, desired duration of occlusion, and whether the embolized tissue is to remain viable . Most orThe case discussed herein was complicated by terminal vessel embolization with resultant ischemia of the skin and underlying gluteal soft-tissue. Buttock necrosis is a rare complication after UAE with limited documentation in the literature, almost exclusively in the trauma literature relating to the treatment of pelvic fracture . To dateGelfoam embolization, when employed, is now routinely performed using a slurry under direct visualization with fluoroscopy. This allows for specific and real-time modification of gelfoam placement and cessation if reflux is noted . AlthougWhile there is robust literature evaluating complications of UAE in other populations, there exists fewer data on the incidence of complications in the obstetric population. A recently published literature review aimed to compare the rate and risk factors for the development of ischemic complications after interruption of the hypogastric artery in the vascular surgery, oncology, trauma, and obstetrics and gynecology populations. In this review they reported complication rates and found that, among obstetrics and gynecology patients, ligations resulted in fewer complications when compared with embolization . However


Gastric perforation (GP) in neonates is a rare entity with high mortality. Although the etiology is not completely understood, it mostly occurs in premature neonates on assisted ventilation. Combination of duodenal atresia and gastric perforation is very rare. We present a case duodenal atresia who developed gastric perforation after operetion for duodenal atresia. Analysis of the patient medical record and histology report did not reveal the etiology of the perforation.  A one-day-old full-term male newborn, weighing 3400g, was admitted with suspicion of duodenal atresia due to history of polyhydramnios and antenatal ultrasound findings. An 8Fr nasogastric (NG) tube was inserted and prophylactic antibiotic (ceftriaxone) was initiated. A supine abdominal radiograph and contrast study revealed a double-bubble sign . At laparotomy, the atresia was detected in the pre-ampullary region of the duodenum, and diamond-shaped duodeno-duodenostomy was performed. NG tube was passed across the anastomosis. Abdominal distension, tachypnea and respiratory distress occurred suddenly 24 hours after surgery. Abdominal radiography showed massive pneumoperitoneum. Emergent re-surgery found a linear tear (5 cm) along greater curvature of the posterior gastric wall . The edges of the tear seemed very thin, as the muscular layer was missing. Perforation edges were excised until normal gastric wall was reached (approximately 1 cm from edge) and primary repair of the perforation was performed in two-layer . Further postoperative course was uneventful. Upper GI series performed after seven days  was normal so NG tube was removed, breastfeeding was started, and he was discharged on the 14th postoperative day. Histological analysis of the excised perforation edges showed gastric wall necrosis. Neonatal GP is a rare entity which requires prompt diagnosis and surgical intervention. The exact etiology of the condition is unclear, although various theories have been suggested including congenital absence of the muscular layer of stomach, increased intraluminal pressure due to distal obstruction by atresia, stenosis or bands, positive pressure mask ventilation during resuscitation, all have been proposed. Vigorous NG tube placement occasionally causes GP, which usually appears as a puncture wound or as a short tear. Prematurity and perinatal stresses are the most important risk factors for GP because of a possible low-perfusion state that leads to gastric wall injury. However, predisposing factors can not be identified in around 20% of the patients [1-8]Congenital duodenal atresia complicated by a GP is a very rare condition. According to a case report and literature review by Takebayashi, there were 12 reported cases of GP related to duodenal atresia, between 1940 and 1975, and one additional case was reported afterwards . Only three of the reported 13 cases survived. In our patient, although the stomach had a wide perforation, generalized peritonitis was absent. Preoperative antibiotic use, early diagnosis of the GP and the pre-ampullary type of duodenal atresia might have prevented peritoneal irritation by pancreatic and bilious fluids. Our patient had two risk factors for the neonatal GP: duodenal obstruction and NG tube insertion. However, we do not believe that any of those had caused GP in our patient. The NG tube had a proper length and was placed under direct vision during the first surgery. In addition, the perforation type was different than NG tube related perforation, which generally has a small diameter. Increased intra-gastric pressure due to distal obstruction too cannot stand as an etiology as the stomach was decompressed by NG tube.To conclude, neonatal GP may occur after successful treatment of duodenal atresia without clear etiology. Revision surgery performed as soon as possible and intensive treatment is crucial for good outcome.Source of Support: NilConflict of Interest: Nil


Mucosa-associated lymphoid tissue lymphomas can occur in various parts of the body, and half of mucosa-associated lymphoid tissue lymphomas occur in the gastrointestinal tract. Gastric mucosa-associated lymphoid tissue lymphoma is the most common lymphoma of the gastrointestinal tract and primary rectal mucosa-associated lymphoid tissue lymphoma is very rare. Because of the high radiosensitivity of mucosa-associated lymphoid tissue lymphomas, this condition can be controlled with radiotherapy of approximately 30 Gy alone. However, ovarian dysfunction as an adverse event of radiotherapy for pelvic lesions can become a problem in girls and women. We report a case of a 28-year-old woman with rectal mucosa-associated lymphoid tissue lymphoma who safely gave birth to a baby following 30.6 Gy radiotherapy to her whole rectum.A 28-year-old Japanese woman became aware of bloody stools and was diagnosed as having Lugano I rectal mucosa-associated lymphoid tissue lymphoma. She was referred to our institute and initiated on radiotherapy. However, she expressed a desire to bear children. We used horizontally opposed pair fields for radiotherapy to minimize the irradiation to her endometrium and ovary. A total dose of 30.6 Gy was given in 17 fractions of 1.8 Gy by 10-Megavolt X-ray linear accelerator. As a result, one-third of her uterus and half of her ovary were outside the irradiation field. After approximately 1 year of treatment, positive pregnancy was confirmed and finally she safely gave birth to a baby girl without congenital abnormalities.This report provides hope for girls and women who have undergone irradiation for pelvic mucosa-associated lymphoid tissue lymphomas and who desire to bear children. Mucosa-associated lymphoid tissue (MALT) lymphoma is a low-grade B cell lymphoma, first identified by Isaacson and Wright in 1983 , 3. BecaA 28-year-old Japanese woman became aware of bloody stools from August 2013 and visited a neighborhood clinic dedicated to internal medicine and digestive organs in September 2013. An initial examination by colonoscopy identified elevated lesions with mild bleeding in the Rb section of her rectum , and she revisited the clinic. A gestational sac was confirmed by ultrasound examination, and the second month of pregnancy was diagnosed. Surprisingly, she had conceived naturally. Finally, she safely gave birth to a baby girl without congenital abnormalities in October 2015.Non-gastric MALT lymphomas can occur in the colon, lung, thyroid gland, salivary glands, mammary gland, and ophthalmologic fields. Primary rectal MALT lymphoma is a particularly rare disease, accounting for less than 1% of all MALT lymphomas , 3. RectA critical point of this case was our patient’s desire to bear children, and we wanted to meet her desire, as well as gain good control over her rectal MALT lymphoma. However, when radiotherapy is focused onto the pelvic cavity, ovarian dysfunction as an adverse event of radiotherapy becomes a serious problem. Therefore, we performed radiotherapy using horizontally opposed pair fields, which goes against the era of three-dimensional radiotherapy. Our treatment procedure had two purposes. First, we tried to minimize the amount of irradiation exposure to her ovaries. Although she had frozen embryos stored, we wanted to preserve her ovarian function as much as possible in an effort to give her a chance of a natural pregnancy. Second, we attempted to minimize the amount of irradiation exposure to her endometrium, such that we could maximize the chance of normal implantation if she could not become pregnant naturally and needed to use her frozen embryos.et al. reported that when the whole ovary is irradiated more than 15 Gy, ovarian dysfunction occurs in almost all patients. In addition, when one side of the ovary is preserved, ovarian dysfunction occurs in approximately half of all patients [Schuck patients . The meaHere we report the case of a 28-year-old woman with a rectal MALT lymphoma who safely gave birth to a baby following 30.6 Gy radiotherapy to her whole rectum. This case involves several rare points. First, there is a low incidence of primary rectal MALT lymphoma among cases of MALT lymphoma. Second, ovarian function could be preserved by removing half of her ovary from the irradiation field using horizontally opposed pair fields. Finally, by removing one-third of her uterus from the irradiation field, we could preserve normal implantation, which enabled her to give birth naturally. We believe that this case is extremely rare and will be of global interest, and we are proud to be able to report the control of rectal MALT lymphoma and the safe birth of a healthy child in this 28-year-old woman.


Hemangioma of the liver is a benign hepatic tumor, more common in women than in men, which is typically asymptomatic, solitary, and incidentally discovered. Atypical hemangioma is a variant of hepatic hemangioma with atypical imaging finding features on CT and MRI that can be confused with hepatocellular carcinoma (HCC), intrahepatic cholangiocarcinoma (ICC) and mixed hepatocellular cholangiocarcinoma (HCC-CC).We report a case of atypical hepatic hemangioma mimicking HCC-CC: A 59-year-old man was referred to our hospital for a hepatic lesion that was 4.7×3.6 cm in size and located in segments 2 and 3 of the liver. Serum alpha-fetoprotein (AFP) level increased from 17.03 ng/mL to 374.9 ng/mL. The patient was positive for hepatitis B.Atypical hepatic hemangioma.US, CT, MRI and Tc-99m RBC liver scans were performed. Dynamic contrast-enhanced MRI showed no uptake in the corresponding area during the arterial phase, peripheral nodular enhancement during the portal phase and delayed phase, and hypo-intensity in the central area. An initial diagnosis of HCC-CC was offered based on the history and imaging findings. Finally, percutaneous liver biopsy (PLB) was offered to the patient. Histopathologic examination of the liver lesions revealed nodular cirrhosis and atypical hyperplasia of liver cells with cavernous hemangioma, where numerous old Schistosoma japonicum eggs were found.Accurate diagnosis of the patient obviated the need for surgery. The patient's recovery after liver puncture was uneventful, and he was discharged on the seventh post-operative day.In some cases, accurate preoperative imaging of focal hepatic lesions is essential but insufficient for diagnosis. PLB and histopathological examination are important, especially in patients with suspected malignancy. Cavernous hemangioma is a common type of hepatic hemangioma, which usually presents as a solitary, well-delineated, subcapsular, disclosed nodule. These distinctive structures show a characteristic hemodynamic pattern on enhanced computed tomography (CT) and magnetic resonance imaging (MRI). However, an atypical hemangioma may lack the imaging features that are characteristic of a typical hemangioma. Hepatic hemangioma can therefore be difficult to distinguish from other lesions such as hepatocellular carcinoma (HCC), intrahepatic cholangiocarcinoma (ICC), and mixed hepatocellular cholangiocarcinoma (HCC-CC). We describe a case of atypical hemangioma mimicking HCC-CC that was misdiagnosed before percutaneous liver biopsy (PLB).Hepatic hemangioma is the most common benign neoplasm of the liver. Patients with this condition are typically asymptomatic. The prevalence of hepatic hemangiomas ranges from 1% to 20% in the general population.2The pathology record was retrieved from the Department of Pathology, First Affiliated Hospital, School of Medicine, Zhejiang University. The study was reviewed and approved by the Institutional Review Board of First Affiliated Hospital, School of Medicine, Zhejiang University.35 copies/mL. Plain CT revealed a space-occupying lesion in segments 2 and 3 of the liver, which was suspected to be a liver neoplasm.A 59-year-old male patient presented with a habit of heavy drinking (700 mL/day) for more than 40 years, history of atrial fibrillation for approximately 6 years, and a 28-year history as a known carrier of Hepatitis B (HBV). He had consulted a doctor for weakness and dizziness 2 months earlier. Entecavir and compound glycyrrhizin capsule therapy were administered immediately. The serum level of aspartate aminotransferase (ALT) was 142 U/L , and alpha-fetoprotein (AFP) was 17.03 ng/mL . Serum HBV DNA was detected, and the viral load reached 4.7 × 10A laboratory workup on admission showed that the serum level for total bilirubin was 27 μmol/L with 18 μmol/L of unconjugated bilirubin; ALT was 46 IU/L; alanine aminotransferase (AST) was 42 ng/mL; laminin was 64.5 ng/mL ; albumin/globulin ratio was 1.1 ; and alpha-fucosylated glycosidase was 54 U/L . All other liver function tests were within normal limits. Serum AFP increased to 374.9 ng/mL , and serum ferritin was 789.6 ng/mL. Other tumor markers, including carcinoembryonic antigen, carbohydrate antigen 125, carbohydrate antigen 199 (CA199), and prostate-specific antigen, were within normal limits. Repeat testing for HBV DNA was negative; serum hepatitis B virus surface antigen (dilution) was 2444.80 IU/L; and anti-hepatitis B virus core antibody was 12.85S/C.Abdominal ultrasonography (US) revealed multiple cystic dark regions in the liver. The largest was 9.5 mm in size with thin walls and an enhancement effect. A well-defined, heterogeneously hypo-echoic mass (4.7 × 3.6 cm) was found in the left, lateral liver Fig. . The bouMRI showed that the well-marginated lesion  had slightly low signal intensity on T1-weighted images and slightly high signal intensity on T2-weighted images  SPECT was performed beginning 60 minutes after the injection of Tc-99m RBC. Liver blood pool tomography revealed a round area of homogeneously increased radioactivity in the left hepatic lobe proximal to the hilus hepatis, except in the central area. The result suggested atypical hemangioma Fig. . Meanwhile, the fluctuation of viral load and the high level of AFP suggested HCC. Based on the patient's history of HBV, the possibility of acute exacerbations of chronic hepatitis B could not be ruled out.An initial diagnosis of HCC-CC was offered based on the history and imaging findings. The imaging findings were highly suggestive of ICC. However, the patient's clinical symptoms were not obvious. The patient lacked the following symptoms of biliary tract obstruction: jaundice, weight loss, abdominal pain, fever, pruritus. The level of CA199 was within the normal limit.Schistosoma japonicum eggs were found , the diagnostic criteria of HCC include AFP levels >400 ng/mL and focal lesion >2 cm with arterial hypervascularization.In our case, repeated inspection results of AFP were significantly abnormal: false-positive results could be excluded. The immunohistochemistry analysis of PLB demonstrated that AFP was negative in the lesion, which contradicted the continuous high serum AFP level. Therefore, combining the long history of HBV with the clinical manifestations of weakness and dizziness, we presumed that the nonmalignant serum AFP elevation was caused by the acute exacerbation of chronic hepatitis B. Recently, this conjecture was supported by strong evidence: the latest level of AFP was down to 22.0 ng/mL, close to normal.MRI reexamination Fig.  after 2 5Here, we report a case of atypical hepatic cavernous hemangioma in an HBV carrier with high AFP. The lesion was benign but required differentiation from hepatic malignancies. However, in some cases, accurate preoperative imaging of focal hepatic lesions is essential but insufficient for diagnosis. Relying exclusively on preoperative imaging could lead to misdiagnosis or unnecessary hepatic resection. PLB and histopathological examination are important, especially in patients with suspected malignancy.


Temporomandibular joint (TMJ) ganglionic cyst is an uncommon entity and only a few have been reported in the literature. TMJ ganglion within the external auditory canal presenting clinically as a fluctuating cystic lesion has never been reported. Here, we present a unique case of such a lesion together with otoscopic and radiological images as well as provide a descriptive review of TMJ ganglionic cysts. Ganglion cyst is expansile, synovial fluid-filled lesion of joints, most commonly seen in the wrists, knees, and feet. Reports of these cystic lesions arising from the temporomandibular joint (TMJ) are scarce. When they occur, they are most commonly found in the preauricular region, with the most common presenting clinical features being pain and swelling . ConventA 59-year-old female was referred to a tertiary otorhinolaryngology unit for multiple reasons. Of these, the predominant troubling symptom was bilateral blocked ears, the right being worse than the left. This was associated with intermittent sharp retroauricular pain, a history of chronic tinnitus, and chronic headaches. She denied history of otalgia, otorrhoea, vertigo, hearing loss, TMJ pain, and discomfort even in the setting of mastication.On examination, there was absence of TMJ crepitus. The cranial nerves were intact, and in particular there was no facial nerve weakness or facial paraesthesia. Otoscopy revealed a cystic lesion arising from the anterior inferior wall of the left external auditory canal . The cysA presumptive diagnosis of TMJ ganglion was made. Computed tomography revealed that the cystic lesion communicated with the TMJ . At follGanglion cyst is a benign soft tissue lesion that usually is found near joints most notable wrist, hand, and feet. TMJ ganglionic cyst is an uncommon entity, and only a few have been reported in the literature. TMJ ganglion within the external auditory canal is rare. The median age of patients is approximately 46, and TMJ cysts occur more commonly in the female gender with a ratio of 3 : 1 [The aetiology of ganglionic cysts remains largely unknown. They are thought to come about from myxoid degeneration of the connective tissue encompassing the joint capsule. Trauma and inflammation have been suggested to play a role , 3.The most common symptoms are cystic swelling and pain. Various other symptoms have been reported including neuropathic pain, pain on mastication, pain on awakening, trismus, paraesthesia, TMJ crepitus, and facial nerve weakness . PreauriAs with ganglion elsewhere surgery is a mainstay form of management. However, if diagnosis can be made confidently based on clinical history, examination, and imaging in an asymptomatic patient, a period of surveillance is justified. However, if the patient is symptomatic, surgery with complete resection appears to provide relief of symptoms . The patIn summary, we provide the first report of a TMJ ganglion presenting as a fluctuating cystic lesion within the EAC. This patient did not have any TMJ pain or otalgia. Importantly, the cystic lesion remained stable. Imaging especially CT provides guidance in making the diagnosis. Surgical excision is an option in symptomatic patients; otherwise, conservative management remains a good choice.


Although Central Giant Cell Granuloma (CGCG) is a benign tumor of the jaw and aneurysmal bone cyst seen in children, its aggressive behavior causes extensive loss of hard tissue requiring wide excision and extensive rehabilitation. We report a rare case of concomitant CGCG and aneurysmal bone cyst in a two-year-old male child, involving the coronoid and condylar process. Young age, large tumor, its aggressive nature, and future growth of orofacial region pose a significant challenge in the management of such conditions. For a successful outcome, the systematic approach to the presurgical evaluation and appropriate treatment planning is essential for such conditions. Bony pathology in the region of head and neck in children is often seen as a morbid prognostic value. Since most of the lesions erode the jaws leading to disfigurement, or otherwise, the growth potential in children exaggerates the recurrence of the lesions.Central Giant Cell Granuloma (CGCG) is a benign tumor of the jaw seen in children and young adults. CGCG accounts for approximately 7% of all benign tumors of the jaws; however, the aggressive nature of the lesion causes resorption of the tooth and bone in the area of the lesion leading to massive destruction of the bone, displacement of the erupted tooth, and germs of the unerupted tooth , 2. It iAneurysmal bone cysts (ABC) are expansile osteolytic blood-filled cystic lesions seen commonly in the mandible . FemalesWide excision of the aggressive lesion is the time-tested treatment modality in bony lesions of the jaw because of recurrence, but they usually disfigure the patients face. Rehabilitation following excision can also be challenging given the dynamic changes concerning growth of orofacial region occurring in the children. Hence the management of aggressive tumors of the jaw in children needs to be a continuous process with regular timed intervention keeping growth in the mind. The aim of this article is to report a rare case of concomitant CGCG and ABC in a two-year-old male child in the posterior region of the lower jaw.A two-year-old male child presented with a unilateral swelling on the right side of the face. The patient's mother noticed the swelling four months back, and since then the lesion had been progressively increasing in size. There was no history of trauma, trismus, fever, or familial history of a musculoskeletal disorder associated with the appearance of this swelling. On extraoral examination, the diffuse swelling was present with posterior region of the right side of the lower third of the face. Overlying skin was normal in color and texture with no signs of local inflammation . On palpLaboratory investigations revealed normal serum values of calcium, phosphorus, urea, creatinine, and PTH. Alkaline phosphatase values were marginally raised. Screening radiographs for chest ribs and skull bones did not reveal the presence of such similar radiolucent lesions. On performing an ultrasonography, normal glandular echotexture and multiple benign upper deep cervical lymph nodes were noted bilaterally.Contrast enhanced CT scan showed large multiloculated expansive lesion measuring 4.2 cm anteroposteriorly, 3.5 cm mediolaterally, and 4 cm superoinferiorly, arising from right ramus of mandible extending to involve coronoid and condylar processes. Images showed uniform expansion resulting in the ballooning of the mandibular ramus Figures . The infThe hematoxylin and eosin stain (H&E stain) section revealed a highly cellular stroma comprising abundant multinucleated giant cells, plump spindle cells, and stromal mononuclear cells. The multinucleated giant cells were diffusely distributed throughout the stroma with areas of hemorrhage, few chronic inflammatory cells, and many dilated RBC filled capillaries. Cysts-like spaces were also evident in certain areas which were lined by cellular connective tissue wall suggesting both Central Giant Cell Granuloma and aneurysmal bone cyst-like appearance Figures . AlthougWith the histopathological diagnosis, the resection of right ramus condylar unit of mandible followed by reconstruction with costochondral grafting was planned. The surgical intervention includes curettage or wide excision depending on the nature and behavior of the lesion. The decision of resection was taken given the extensive bony involvement and multiple cortical bone perforation. In our case, since the lesion showed multiloculated radiographic appearance and was aggressive in nature surgical resection was planned over curettage to avoid recurrence.A seven-centimeter-long extraoral submandibular incision was given along the neck crease. By layered sharp and blunt dissection, the inferior border of the mandible was exposed. Upon reflecting masseter and the periosteum, the surgically exposed bone showed ballooning and had a brownish hue. The inferior border of the angle of the mandible and ramus were involved. The bony lesion was resected, into healthy bony limits . Dark reThe behavior of the lesion is a critical factor in deriving a diagnosis of swelling seen in head and neck region in children. If one can categorize the swellings into groups based on the site of lesion, progression, and onset and duration of swelling, then a correct differential diagnosis can be obtained . An acutThe fibroosseous diseases of the jaws represent a diverse group of entities. They commonly include lesions of primary or secondary hyperparathyroidism, fibrous dysplasia including cherubism, Central Giant Cell Granuloma, and aneurysmal bone cyst. The histopathological findings of these lesions may be remarkably similar, and hence they have to be differentially diagnosed by the clinical and radiographic presentation.The clinical presentation of bony hard swelling with normal overlying skin color and normal cutaneous and subcutaneous echotexture on ultrasonography do not favor the diagnosis of neurofibroma, hemangioma, lymphangiomas, and vascular malformation. Certain intraosseous variants of vascular malformation and hemangiomas are reported; the radiographic presentation of multiloculated “soap bubble appearance” as seen in our case does not coincide with such intraosseous lesions of vascular origin. The absence of any intraoral infectious foci rules out osteomyelitis or similar chronic infectious lesions. The normal values of serum calcium, phosphorus, and alkaline phosphatase along with unilateral presentation indicate that the lesion was not cherubism or fibrous dysplasia.Probable diagnosis of Central Giant Cell Granuloma (CGCG) can, therefore, be derived by sequentially ruling out another differential diagnosis. Radiographic “ballooning” of the ramus of mandible and “soap bubble appearance” further support the possible diagnosis. CGCG is predominantly found in children and young individuals but has not been commonly reported in a child as young as our patient. The coronoid and condylar process is rarely involved by the lesion . The hisThe conventional treatment of lesions like CGCG or ABC of the jaw bones is surgical excision either by curettage or en bloc resection depending on the behavior (aggressive or nonaggressive), location, the size of the lesion, and radiographic appearance . Other tWhen conservative options like Denosumab are used a complete response is rarely obtained; hence additional surgery becomes necessary to remove the tumor in case of tumor progression, to remove a remnant, or to remodel bone. Moreover, these drugs have frequent local or systemic side effects such as osteonecrosis and growth deficiencies –13.Because of high recurrence rate of up to 70% after local curettage wide excision of the lesion is preferred as the choice of treatment , 14. A rThe recent follow-up of our case revealed spontaneous regeneration of bone. It is an interesting finding to report since only a few reports exist on the spontaneous regeneration of bone after surgical resection of jaw lesions , 19. It Usually CGCG and ABC are seen with higher female predilection but in our case, it was seen in a very young boy and in unusual site with involvement of coronoid and condylar process. Proper histopathological examination and radiographic examination are much essential in establishing an accurate diagnosis, as in our case histopathological examination reported the presence of an aggressive case of concomitant CGCG and aneurysmal bone cyst. With this kind of unusual presentations, the clinician should always bear differential diagnosis in mind while examining bony swelling in head and neck region in children. With the evidence of spontaneous regeneration of resected portion of the mandible in our case, we suggest that when considering reconstructive options in such aggressive lesion of the jaw in children one must keep the host's growth potential in mind.