Castleman’s disease (CD) in trachea is rarely reported to date. This report introduces a case of CD in trachea with emphasis on its radiological presentation onCT. A female patient was admitted at our emergency department with dyspnoea. Plain and enhanced CT revealed a mass with distinct border and marked enhancement. Tracheotomy was planned and it turned out to be CD of hyaline type on pathological examination. Two similar case reports on intratracheal CD from 1954 to 2015 were reviewed as we searched in PubMed using key words “endotracheal Castleman’s disease” or “Castleman’s disease in trachea” or “Castleman’s disease in tracheal”. This will be the third case report of CD in trachea in English literature. In this case report, the radiological appearance of CD on multiple imaging modes is reviewed. Lesions that should be taken into consideration in differential diagnosis are mentioned. The two main surgical methods for such lesions are briefly described. Contrast-enhanced CT revealed marked homogeneous enhancement  on the lateral wall of the trachea . It was ancement , with thl centre . Immunoh2 It can occur at any site with lymphatic tissue throughout the body. Of all lesions reported, 70% were in the chest, 15% were in the neck and the remaining were in the abdomen and pelvis.3 CD may be asymptomatic and discovered incidentally. It can present with painless lymphadenopathy or other symptoms related to structures compressed nearby.3 CD in trachea is rarely reported. Like other lesions in the trachea, obstruction of the airway and progressive dyspnoea may be the main symptoms, but it is far from diagnostic.CD, which was first described in 1954 by Benjamin Castleman, is an uncommon lymphoproliferative disorder of uncertain causes.4 even for chest radiologists. Preoperative diagnosis of such focal lesions in trachea remains challenging for several reasons. On one hand, various lesions including inflammatory diseases, neoplastic lesions and systemic lesions can occur.5 Squamous cell carcinoma and adenoid adenoma have the highest occurrence rate.5 Squamous cell papilloma, non-Hodgkin’s lymphoma and papillomatosis should be taken into consideration in differential diagnosis, as they can occasionally be seen in clinical practice.5 On the other hand, even though some focal tracheal lesions have very low morbidity and are reported in case reports, they can share similar clinical and radiological presentations with CD. These focal hypervascular lesions may include paraganglioma and haemangioma. Besides, another reason that makes accurate preoperative diagnosis challenging is that CD may have various presentations according to its pathological type.As tracheal lesions have an overall low prevalence, it is easy for radiologists and doctors not to pay enough attention to them, thus making tracheal lesions “forgotten zones” in chest imaging,6 On MRI, they usually exhibit as heterogeneous T1 and T2 hyperintensity signals compared with the skeletal muscle.7 Prominent flow voids, which imply the existence of feeding vessels, may be seen. On positron emission tomography-CT imaging, the lesions can show a high uptake of radiolabelled [18F]-2-fludeoxy-D-glucose, thus mimicking malignant adenopathy.8 Ultrasound plays an important role in detecting superficial lesions, which usually exhibit as single, well-defined, hypoechoic solid masses with no cystic necrosis inside.6On imaging, the classic CT finding of hyaline vascular-type CD is a solitary enlarged lymph node or localized nodal mass that demonstrates intense homogeneous enhancement. Approximately 10% of the lesions have internal calcifications, with characteristic coarse or distinctive branching patterns.10 have been reported in English literature, both of them presented with respiratory distress and both lesions were resected with rigid bronchoscopy.10 Tracheotomy remains a classic treatment for similar lesions in trachea, and bronchoscopy is another solution widely used in recent years. Rigid bronchoscopy is a less invasive method, while tracheotomy enables more complete excision of lesions, especially for potentially malignant cases. In this patient, malignant lesions were suspected before surgery and tracheotomy was arranged to remove the lesion.CD originating from the trachea is rare. Only two casesCD can occur at any site with lymphatic tissue; a mass or a group of masses with a distinct margin and an obvious enhancement on CT might be an implication of CD.A suggestive preoperative diagnosis of lesions in trachea can help doctors make more appropriate operative plans.The patient agreed the doctors could use and publish her disease related article with personal information deleted.


Elastofibroma is a benign soft tissue tumor characterized by the presence of elastic fibers in a stroma of collagen and mature adipose tissue. It is reported to have a prevalence of 2.73%, as shown by a study through computed tomography (CT) images. However, multiple elastofibromas are uncommon.We report a case of concomitant bilateral elastofibroma in the infrascapular and gluteal regions. A 63-year-old male patient presented with a 6-month history of gradually increasing painless swellings in the upper back. On physical examination, firm, painless bilateral infrascapular masses were identified; these masses were more noticeable on forward arm flexion. Contrast-enhanced computed tomography showed well-defined bilateral infrascapular masses deep to the serratus anterior muscles as well as poorly defined bilateral gluteal masses with attenuation similar to that of the adjacent skeletal muscle. Magnetic resonance imaging revealed heterogenous masses with internal fatty streaks, consistent with elastofibroma. The histopathological diagnosis of elastofibroma was established based on the results of image-guided core-needle biopsy. The patient underwent surgical excision of both infrascapular elastofibromas with no post-operative complications. As the gluteal masses were incidental, surgical management was not warranted.The presence of multiple elastofibromas is unusual. This report describes a rare case of multiple elastofibromas and its typical imaging features, and alerts us that elastofibromas are not exclusive to the periscapular region. Elastofibroma is a benign soft tissue tumor characterized by the presence of elastic fibers in a stroma of collagen and mature adipose tissue. This tumor was first reported in 1961 in the 12th Histopathology Scandinavian Congress by Järvi and Saxen who briefly described four cases of this tumor and called it elastofibroma [Although previously believed to be rare, elastofibroma has a prevalence of 2.73%, as shown by a large retrospective study through computed tomography (CT) images [We report a case of a 63-year-old man who presented with a 6-month history of a gradually increasing swelling on the right upper back. Although the swelling was painless, the patient experienced some discomfort because of its size. He had also observed a comparatively smaller swelling on the left side of the same region. Furthermore, he recently experienced unintentional weight loss. He reported no history of heavy manual work, trauma, or malignancy during his lifetime, and his past medical history was unremarkable.Physical examination revealed the presence of a palpable, non-tender, firm mass with limited mobility located beneath the scapular tip of each shoulder. However, it was more prominent on the right side. The masses were more noticeable on forward arm flexion. No palpable lymph nodes were noted, and the back and shoulder exhibited a normal range of motion. Routine laboratory investigations showed no abnormal findings.The patient underwent contrast-enhanced CT, which showed well-defined, bilateral, solid masses located deep to the serratus anterior muscles in the infrascapular region Fig. a. The riMagnetic resonance imaging (MRI) was performed using a 1.5-Tesla scanner, and T1- and T2-weighted axial and coronal scans were acquired without contrast administration. The bilateral infrascapular lesions showed muscle-like signal intensity on both sequences (Fig. On the basis of the clinical and radiological appearances of the infrascapular and gluteal masses, a presumptive diagnosis of elastofibroma was made. However, considering the patient’s anxiety and history of weight loss, biopsy was recommended during a multidisciplinary oncology meeting. A histopathological diagnosis of elastofibroma was established through core-needle biopsy of the right infrascapular mass Fig. .Fig. 3EThe diagnosis was discussed with the patient, and he decided to undergo surgical resection of the infrascapular masses. However, the gluteal masses were asymptomatic and did not warrant surgical management.Both infrascapular masses were completely excised. The masses were not encapsulated and exhibited a rubbery consistency. Histopathological analysis revealed the presence of fibrous collagenous tissue, occasional fibroblasts, and a large number of eosinophilic fibers intermixed with islets of mature adipose tissues. The resected lesions had clear surgical margins. At the follow-up visit, the patient was asymptomatic and showed no functional limitation in the movement of the shoulder joint.Elastofibroma is often observed beyond the sixth decade of life , 5. AlthThe classic site of elastofibroma is the scapular region between the sixth and the eighth ribs and the inferior angle of the scapula, beneath the serratus anterior, latissimus dorsi, and levator scapulae muscles . It is mBesides concomitant infrascapular and infra-olecranon elastofibromas, multiple elastofibromas are uncommon with few cases reported in the English medical literature . NagaminDifferent environmental and genetic explanations have been postulated to explain the development of elastofibroma; however, the exact pathogenesis remains unclear. It has been suggested that elastofibroma develops due to repetitive mechanical trauma, which induces reactive hyperproliferation and degeneration of fibroelastic tissue . The advIn most cases, elastofibroma is asymptomatic and is diagnosed incidentally. Few cases may present with palpable masses, discomfort, snapping of the scapula, or functional limitations. Symptoms caused by impingement of the brachial plexus may also be observed .Imaging is essential and may preclude the need for biopsy, particularly if the lesions occur at their typical locations and have the typical imaging features . RecogniPlain radiographs are usually normal, but they may show a raised scapula or a soft tissue density. Ultrasound shows alternating patterns of hypo- and hyperechoic lines parallel to the chest wall with no significant vascular flow on Color Doppler . CT scanBecause elastofibroma is an incidental finding in most cases, surgical treatment is often not required and regular follow-up is not needed as malignant transformation has not been previously reported . CompletThis report describes a rare case of multiple elastofibromas and its typical imaging features; our findings demonstrate that elastofibromas are not exclusive to the periscapular region.


Small bowel perforation is rare in dermatomyositis (DM). However, it is associated with high rates of morbidity and mortality. In line with the SCARE criteria, we describe a case of jejunal perforation for a DM patient .A 63-year-old woman had been treated for DM with high dose steroid 1 month prior to the onset of severe abdominal pain. Computed tomography (CT) revealed free air in abdominal cavity and fluid around the small bowel. She was diagnosed with small bowel perforation and underwent emergency surgery. Emergent surgery showed perforated jejunum which was resected. Pathologic reports revealed mesenteric small-vessel vasculitis with a perivascular inflammatory cell infiltration.Perforation of the small bowel for DM patients is rare. However, the early diagnosis of bowel perforation is difficult in DM because it can mimic other gastrointestinal manifestations such as ileus, ischemic colitis and peritonitis. To minimize mortality via an early diagnosis and a timely treatment, it is important to examine the patient’s clinical history and employ a proper medical imaging modality such as CT even when lab findings are nonspecific and atypical. The peak incidence of DM occurs with a bimodal distribution of 5–14 and 45–60 years of age . The inc23/μL (absolute neutrophil count 4470/μL), and platelets 50 × 103/μL. She had high-grade fever (39 °C). Although her purse rate was high (118 beats/min), blood pressure (123/80 mmHg) was normal. Following an emergent surgical consultation, the patient was diagnosed with pan-peritonitis due to small bowel perforation and promptly transferred to the Department of Surgery, whereby a diagnostic surgical exploration was performed. A 2 × 1 cm sized perforation in the antimesenteric border of proximal jejunum was identified (Candida parapsilosis. Cultures continuously remained positive despite intravenous administration of fluconazole (400 mg daily). Finally, she developed multiorgan failure and expired 11 days after surgery.A 63-year-old female patient presented to the Department of Internal Medicine with a fever (>38 °C) that had persisted for two days and lower abdominal pain. The patient was admitted to the hospital due to gait disturbance that started two months earlier. Brain electromyography, brain magnetic resonance imaging, and nerve conduction study failed to reveal the cause of gait disturbance. Positron emission tomography showed mild uptake of fluorodeoxyglucose in the soft tissue around the nose and posterior neck muscle area at the C1 level. Skin biopsy showed hyperkeratosis, mucin deposition and upper dermal perivascular mononuclear infiltrates . In addientified . A moderentified . Upon opentified -A. In adentified -B. After3DM is a rare, chronic inflammatory disease. It is caused by vasculitis determined by humoral factors with subsequent inflammatory cell accumulation, mainly T CD4+ and B cells, which infiltrate myocytes leading to its vacuolization and degeneration  . The diaAlthough GI manifestations occur in up to 50% of DM patients, most cases show symptoms such as dysphagia, esophageal reflux, esophageal dysmotility, delayed gastric emptying, and decreased intestinal motility ,10. DM-iThe pathologic examination of intestinal vessels in this case showed small-vessel vasculitis with intra-vascular thrombosis. We conjecture that there are two factors jointly contributing this vasculopathy. The first point is endothelial injury due to DM itself. The existing studies reported that 44% of patients with DM and PM are found with anti-endothelial antibodies such as IgG and IgM . These aThe treatment for spontaneous small bowel perforation of the DM is based on the same principles as other perforations of the intestine. Initial management of small bowel perforation consisted of intravenous fluid therapy, cessation of oral intake and intravenous broad-spectrum antibiotics. Surgical options range from conservative to abdominal exploration including primary repair, enterostomy and segmental resection. Since the first laparoscopic repair of small bowel perforation was performed in 2005, pure laparoscopic or extracorporeal laparoscopic-assisted resection of a perforated small bowel has been widely performed . In thisThe key clinical feature of this case is sudden onset abdominal pain with normal WBC count in DM patient. In conclusion, intestinal perforation among DM patients has been extremely rare since the development of steroid therapy. However, despite high-dose steroid therapy, bowel perforation can be caused by ischemic changes due to vasculitis. Therefore, immediate examinations such as CT scans should be conducted when bowel perforation or ischemic change is suspected. We argue that it is important to pay more attention to GI manifestations and consider more careful examination when treating DM patients.The researchers claim for no conflicts of interests.The author received no specific funding for this study.This study was approved by the IRB in Jeju National University Hospital.Written informed consent was obtained from the patient's children for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Donghyoun Lee & Wooseong Jeong wrote the manuscripts. Chang Hak Hyun confirmed pathologic reports. Jinseok Kim reviewed the literature.NA.Jinseok Kim M.D., Ph.D.All additional data are available.Not commissioned, externally peer-reviewed.


The case of a 75-year-old female with invasive extramammary Paget's disease of the anal margin, without involvement of the anal canal, is reported. The patient underwent wide local excision of the lesion with reconstruction with a double V-Y flap, a biopsy of the inguinal sentinel node, and a laparoscopic temporary colostomy. No guidelines exist on the treatment of this rare disease, and both wide local excision and abdominoperineal resection have been proposed. In the present case, the absence of invasion of the anal canal, also confirmed by intraoperative biopsies on the resection margins, and of local lymph node metastasis, as confirmed by the sentinel lymph node biopsy, allowed a sphincter-sparing operation with good functional and oncological results. Mucinous adenocarcinoma of the perianal skin is an extremely rare disease . It usuaWe present a case of a local excision of a mucinous adenocarcinoma of the anal margin and reconstruction with a bilateral V-Y flap.A 75-year-old female patient presented at the surgical consultation for perianal hitching and bleeding. The patient's complaints started 12 months prior, with progressive worsening. Her past medical history included hypothyroidism, glaucoma, and hemorrhoidectomy 30 years previously. Her family medical history included hepatic cirrhosis (father) and coronary artery disease (mother) but no cancer. Physical examination showed a 3 cm plaque-like eczematous lesion, circumferentially involving the perianal skin and extending to the anal verge . A biopsThe day before the operation, a lymphoscintigraphy was performed which showed uptake in the right inguinal region. The patient underwent surgery the following day under general anesthesia. A sentinel inguinal lymph node was retrieved at the right groin region. A laparoscopic lateral colostomy was performed on the sigmoid colon. The patient was then placed in a jackknife position. The neoplasia was resected with 1 cm of free margins over the skin and the anal verge, including the dermis and the subcutaneous tissue. Once the excision was completed, multiple biopsies inside the anal canal were taken to exclude invasion. Then, stiches were placed at the four cardinal points on the mucosa of the anal canal. A double V marking was drawn, and the flaps were dissected with vascular pedicle preservation, based on random inferior gluteal perforator vessels and peripheral skin undermining . The flaThe pathological report was mucinous adenocarcinoma of the perianal skin involving the subcutaneous tissue with pagetoid spread with negative resection margins . The les5After 3 months , random Perianal or anal margin cancers are defined as cancers that arise distally from the dentate line and can be entirely seen by gentle traction to the buttocks ; they arImmunohistochemical studies can help differentiate primary from secondary PD since the presence of CK7 and GCDFP15 suggests primary PD while CK20 and CDX2 are more consistent with secondary PD . In the The management of PPD is not standardized and includes noninvasive approaches  as well as surgical treatments  , 11–13. This rare disease can be successfully treated with wide local excision after a throughout preoperative workup and intraoperative exclusion of anal canal involvement, thus avoiding a permanent colostomy. Attention should be paid to exclude concomitant malignancies and to differentiate primary from secondary disease through immunohistochemistry on the resected specimen.


Pancreatoblastoma (PB) is a rare pancreatic neoplasm which arises when a group of pancreatic cells start to go through uncontrollable growth. The diagnosis of PB is challenging due to its vague symptoms. The initial diagnosis is made by imaging, afterwards the management is usually by resection of the tumor with or without chemotherapy which depends on the size and grade of the tumor. We report a case of a nine-year-old girl who was diagnosed with pancreotoblastoma and underwent complete resection with chemotherapy. Pancreatic tumors are conditions which arise when a group of pancreatic cells start to go through uncontrollable growth . PancreaPancreatoblastoma management varies according to the stage. Until now around 200 cases were reported, and the surgical intervention plays a significant role in attaining complete remission . PrognosA nine-year-old girl complained of mild abdominal pain in the last three years. The patient had a history of several admissions with diagnosis of urinary tract infection. Before one month, the abdominal pain started to increase in intensity and the mother started to notice loss of weight. She was diagnosed with hepatoblastoma and was referred to our hospital for further management.On admission the clinical examination showed underweight looking girl; chest and cardiovascular examination was unremarkable. Abdominal examination showed a mass occupying most of the left upper quadrant region. In addition, laboratory tests showed abnormal tumor markers of alpha-feto-protein (AFP) 45.6 IU/mL  and cancer antigen 125 (CA 125) of 44.1 IU/mL . Chest CT scan was done and showed a huge heterogeneously enhancing epigastric lesion measuring 6.2 x 8.7 x 9.4. Multiple mesenteric enlarged lymph nodes, the largest of which measured 1 cm x 1.7 cm. The lesion was probably arising from the liver; however, it is inseparable from the pancreas (Figure The patient underwent laparotomy to obtain biopsy for this lesion. During surgery the tumor was adherent to the left lobe of the liver segments II and III; the adherence was under the surface which made it very difficult to distinguish if the tumor is arising from the liver or invading it. The histopathology report showed fragments of dense fibrous tissue with hyper-cellular proliferation of small round blue cells with mild to moderate nuclear pleomorphism.The cells were arranged in sheets forming numerous cords and rossettes with focal areas of acinar type architectural differentiation. No papillary cords were identified. None of the rossettes have central vessels. There were areas of syncytial arrangement of cells with more abundant pink cytoplasm which were consistent with squamoid nests. The nests were vaguely circumscribed, however, did not demonstrate any keratinization. Foci of foamy cells were identified, but perivascular hyalinization was not identified. No cholesterol clefts were identified or eosinophilic globules Figure .Immunohistochemical stains demonstrated diffused cytokeratin A1, A3 positivity, prominent multifocal synaptophysin positivity and focal chromogranin positivity. Multiple foci are positive for CD10. ER and PR are negative. AFP negative, S100 negative. Scattered mitosis approximately 2-3 10/HPF are present. The biopsy confirmed the diagnosis of PB stage III. The patient was started on chemotherapy, and she received doxorubicin. And she was on PLADO protocol. After six cycles of chemotherapy a CT scan showed significant changes with decrease in size measuring 3.4 x 3.3 x 3.5. after that the patient underwent subtotal pancreatectomy and resection of tumor (Figure Although PB is one of the common pancreatic tumors, still pancreatic tumors in children are considered rare. Since Becker  reportedPancreatoblastoma is usually incidentally found because most of the time it is asymptomatic. In a study by Xu et al.  where thAs per our case, the patient presented with multiple attacks of abdominal pain which gave rise to multiple differential diagnoses, one of which was PB. An abdominal mass was only identified radiologically. Moreover, most reported cases presented with abdominal pain and PB was only diagnosed incidentally via imaging. According to the size and nonmetastases of our case, chemotherapy of PLADO protocol was used. For instance, one of the reported cases used neo-adjuvant chemotherapy to regress the metastatic lesions and the tumor . SimilarWe recommend commencing a multicenter clinical trial in order to have sufficient data to construct a standardized treatment protocol for PB cases.Pancreatoblastoma is a rare malignant neoplasm that originates from primitive cells. With an incidence of around 0.004 per 100,000 cases every year. PB diagnosis is challenging due to its nonspecific clinical manifestations. Management of this tumor is mainly by surgical resection with or without chemotherapy which depends on its size and stage.


Coley, in 1892, reported 14 cases of a hydrocele in women. He described this “affection” as being “too rare an anomaly to deserve consideration. The rarity of this finding continues to be described in more current literature of 400 cases.42-year-old woman presented to the clinic with a palpable mass in her left inguinal region. On physical examination, a soft-consistency, mobile mass of about 4 cm in size was seen in the left inguinal region. The cystic lesion which was seen to be originated from the inguinal canal was excised in the exploration made by suspending the round ligament by passing through the anatomical folds with the incision made from the left inguinal region. The defect was repaired with prolene mesh after high ligation. Patient was discharged on the 1st postoperative day.In women, a round ligament is attached to the uterus close to the origin of the fallopian tubes, and the extension of the parietal peritoneum follows the round ligament as it passes to the inguinal canal through the internal ring. Hydroceles of the canal of Nuck were not conclusively diagnosed until surgery was performed on a suspected inguinal hernia. The treatment of Nuck canale hydroceles are surgery. Ligating the prosessus vaginalis and excision of the cyst in surgical treatment will prevent recurrences.Nuck canal cysts should be considered in the differential diagnosis of cases of female patient's complaints of swelling in the inguinal region. He described this “affection” as being “too rare an anomalyto deserve consideration” . The cys2A 42-year-old woman presented to the clinic with a palpable mass in her left inguinal region which was noticed 1 month prior. The mass had not been present in infancy oradolescence. History of trauma and operations were not found in the patient's history. There was a cyst aspiration story from 2 months ago. On physical examination, a soft-consistency, mobile mass of about 4 cm in size was seen in the left inguinal region. During the Valsalva maneuver, the mass did not change in size and shape. The patient's laboratory findings  were within the normal range. Ultrasonography revealed a hypoechoic cystic mass with a size of 40 × 50 mm in the left inguinal area without any vascular flow and no peristalsis .Picture Abdominal magnetic resonance imaging (MRI) was performed to examine the communication between the cystic mass and peritoneal cavity, and the precise anatomy around the cystic mass.It was found that the cystic mass in the inguinal canal included thin septa, and hydrocele of the canal of Nuck was suspected because of the low and high signal intensities observed on the T1- and T2-weighted images, respectively. Only the wall and septa were contrast-enhanced. The cystic lesion which was seen to be originated from the inguinal canal was excised in the exploration made by suspending the round ligament by passing through the anatomical folds with the incision made from the left inguinal region .The defe3In women, a round ligament is attached to the uterus close to the origin of the fallopian tubes, and the extension of the parietal peritoneum follows the round ligament as it passes to the inguinal canal through the internal ring. This evagination of the parietal peritoneum, named the canal of Nuck, is known as the female counterpart of the processus vaginalis in men. The cystic enlargement is probably due to the imbalance of the secretion and absorption of the secretory membrane that covers the processus vaginalis. This imbalance may be caused by a change in lymphatic drainage as a result of trauma or infection, although most cases are idiopathic . There aThe majority of the reported cases of hydroceles of the canal of Nuck were not conclusively diagnosed until surgery was performed on a suspected inguinal hernia .Women's hydroceles usually manifest themselves as a painless palpable mass in the groin area. Suspicion may arise if the mass is translucent, doesn't disappear in the supine position -like in our patient- or if it becomes more pronounced with valsalva. Imaging studies may help in pre-operative diagnosis but most of the cases of hydrocele of canal of nuck finally diagnosed on surgical exploration. Cyst of the canal of Nuck demonstrates varied appearances in sonography. In the literature sonographic appearance of hydrocele of the canal of Nuck shows thin walled, well defined, echo free, cystic structure varying from an anechoic, tubular, sausage, dumbbell or comma-shaped, “cyst within a cyst’’ to a multicystic appearance . MagnetiOn pathological examination, the Nuck duct cyst is defined as a cyst that is filled with light-colored fluid and has smooth muscle tissues on its walls and is laid with single-layer cuboidal mesothelial cells .Nuck canal cysts should be considered in the differential diagnosis of cases of female patient's complaints of swelling in the inguinal region.No conflicts of interest were declared.We have no supportive funding.I certify that this kind of manuscript does not require ethical approval.Written informed consent for publication of his clinical details and clinical images was obtained from the patient.Uğut topal.-Ahmet gökhan sarıtaş study concept, writing the paper, final decision to publish, data collection.Abdullah ülkü.- study concept, data collection.Atılgan tolga akçam.- data collection and analysis.Ahmet Gökhan Sarıtaş, Abdullah Ülkü.Not commissioned, externally peer reviewed.


Foreign body (FB) aspiration into the tracheobronchial tree is an emergency in the pediatric department, particularly in children aged <3 years. FB granulation tissue is commonly found in children with FB aspiration. However, scarring in the bronchus caused by a FB is rare. We herein report a case involving aspiration of a plastic whistle toy with scarring in the bronchus. The scar tissue was successfully removed by interventional bronchoscopy combined with a flexible electrosurgery probe and carbon dioxide cryotherapy. Aspiration of a foreign body (FB) into the trachea and bronchus is common among children, especially in infants and young children . ComplicWe herein present a rare case of bridging scar tissue formation caused by a FB (a plastic whistle) that resulted in narrowing of the left main bronchus. The FB and scar tissue were removed by interventional pulmonology modalities. The patient's guardians provided written informed consent for the publication of this case report and any accompanying images. Approval for this report was granted by the ethics committee of Children's Hospital, Zhejiang University School of Medicine.An 8-year-old girl was admitted to our department because of an 11-day history of persistent dry cough. Her medical history was not significant. The girl and her parents denied any history of choking or FB aspiration. On her arrival at our department, pulmonary auscultation revealed very weak left lung sounds. Chest computed tomography revealed a soft tissue density at the left lower lobe bronchus (LLLB) with stenosis of the left main bronchus and emphysema of the left lower lobe .Airway exploration by fiberoptic bronchoscopy under sedation and local anesthesia through the nasal route was performed to locate the suspected bronchial lesion. Bronchoscopy revealed that the stenosis of the left main bronchus was caused by a bridging scar tissue . The steIn the operating room, the patient admitted that she had aspirated a plastic whistle 6 months previously. A laryngeal mask was used during general anesthesia, allowing the patient to breathe spontaneously. A swivel adapter was used to connect the proximal end of the laryngeal mask to the T-piece anesthesia system. A flexible fiber bronchoscope (4.9 mm outer diameter) was inserted via the swivel adapter. A flexible electrosurgery probe  was then inserted through the working channel of the bronchoscope. The target scar was endoscopically visualized and cut by the probe. A grasping forceps was introduced via the suction channel of the bronchoscope to remove the FB. However, lack of sufficient pulling force prevented removal of the FB. A rigid bronchoscope was then introduced, and we successfully removed the plastic whistle from the LLLB using rigid forceps . Carbon Three months after the surgery, the patient's ventilatory function test results were normal. Bronchoscopic examination showed good patency of the truncus and LLLB .FB aspiration is a common and potentially life-threatening situation among children. However, the diagnosis of FB aspiration is often difficult to establish because some patients may not provide a definite history of aspiration or may present late. In addition, patients may be misdiagnosed with chronic pneumonia, bronchitis, asthma, or malignancy. Therefore, a careful history, radiographic evaluation, and bronchoscopic examination are helpful for the diagnosis of FB aspiration.The features of tissue reactions to FBs include granulation tissue formation, endobronchial stenosis, strictures, edema, and airway distortion; in contrast, scarring in the bronchus is rare . The forRigid bronchoscopy is considered the traditional bronchoscopic technique of choice for FB removal in children. It allows excellent control of the airway, provides a large working channel, and permits the use of a wide variety of extraction instruments for removal of FBs and thick mucous plugs. However, a rigid bronchoscope cannot be passed to the distal airways. In addition, visualization of the upper lobe bronchi is difficult. Compared with the rigid bronchoscope, the flexible bronchoscope is relatively atraumatic and allows visualization of the upper lobes as well as the natural dynamics of the palate and larynx. The laryngeal mask airway is a safe and effective adjunct to fiberoptic bronchoscopy under general anesthesia in children. Its larger internal diameter than that of a tracheal tube permits the use of a relatively large fiberoptic bronchoscope without a significant increase in airway resistance .Accumulated data have shown the promising value of flexible bronchoscopy in the extraction of airway FBs. In our department, FBs were removed by flexible bronchoscopy with a disposable grasping forceps or biopsy forceps with a high success rate of 90% . The advThe recovery of the left main bronchus was observed after removal of the FB. Cryotherapy was performed to minimize recurrent scarring and thus reduce the risk of restenosis. Cryotherapy involves insertion of a cryoprobe through the working channel of the bronchoscope to create a freeze-thaw effect on the target tissue. This process induces coagulation necrosis of the selected tissue and destruction of the lesion . CryotheOur case demonstrates that bronchoscopy with multiple modalities  is effective for the diagnosis and removal of FBs. Similarly, Fang et al. reported that flexible bronchoscopy with different modalities  showed a high success rate (90%) in removing FBs . CollectFB aspiration is often challenging in terms of diagnosis and treatment. Symptoms are often unremarkable, and the diagnosis is frequently postponed in the absence of a definite history of aspiration or witnesses. Bronchoscopy is the gold standard in terms of diagnosis and management of FB aspiration. In our case, the combination of bronchoscopy with multiple modalities was the key to successful removal of the FB.


Benign esophageal tumors are relatively rare, and a neurofibroma in the esophagus is extremely rare. Dysphagia is the most common clinical manifestation in patients with esophageal neurofibroma, and no cases of giant esophageal neurofibroma with severe tracheal stenosis have been reported.18F-fluorodeoxyglucose (FDG)-positron emission tomography image revealed an upper mediastinal homogeneous mass and left supraclavicular lymph node with increased FDG accumulation. We performed endoscopic ultrasound-guided fine-needle aspiration biopsy; however, a definitive diagnosis could not be determined. During further investigation, her shortness of breath suddenly worsened and she suffered from wheezing. Because of risk of smothering, we decided to perform quasi-urgent lifesaving surgery. Under the preparation of extracorporeal membrane oxygenation (ECMO) when tracheal intubation fails, bronchial blocker was inserted over the tracheal stenosis and the left-lung ventilation was performed via intubation alone. Under general anesthesia, the patient was placed in the left lateral position and we performed right thoracotomy. The tumor strongly adhered to the trachea; however, the trachea or recurrent laryngeal nerves were not damaged in the surgery. Following esophagectomy, we performed gastric conduit reconstruction through the posterior mediastinum, and hand-sewn anastomosis was performed in the left neck. Immunohistochemical staining was positive for S-100 but negative for c-KIT, CD34, α-SMA, and desmin; these morphological and immunohistochemical characteristics were consistent with the diagnosis of neurofibroma.A 73-year-old woman presented with shortness of breath, and computed tomography scan exhibited a giant mediastinal tumor causing severe tracheal stenosis. An upper gastrointestinal endoscopy revealed a giant submucosal lesion without mucosal changes located 18–23 cm from the incisor teeth. It is often difficult to diagnose esophageal neurofibroma preoperatively. The preparation of ECMO could be considered in patients with severe airway obstruction for safe tracheal intubation. This is the first case of life-threatening giant esophageal neurofibroma with severe tracheal stenosis. Benign esophageal tumors are relatively rare, and leiomyoma accounts for 80% of these tumors; however, they are extremely difficult to diagnose preoperatively . A defin18F-fluorodeoxyglucose (FDG)-positron emission tomography imaging revealed an upper mediastinal homogenous mass and left supraclavicular lymph node with increased FDG accumulation  biopsy was conducted in order to provide a definitive diagnosis. Three EUS-FNA specimens revealed spindle cell tumors; however, a definitive diagnosis was not determined with immunohistochemical staining. Immunohistochemical staining was negative for c-KIT, CD34, cytokeratin AE1/AE3, p53, and desmin, but a partial positive for S-100. The patient was required to undergo surgery with general anesthesia for further investigation or treatment; however, tracheal intubation was considered to be a very high risk at the second hospital due to the severe tracheal stenosis. Hence, she was referred to our hospital for further treatment after 5 months of visiting the second hospital.At our hospital, we reviewed previous findings, and the differential diagnoses were leiomyoma, gastrointestinal stromal tumor (GIST), and neurogenic tumor. Although EUS-FNA specimens revealed spindle cell tumors, malignant lymphoma was not excluded completely because of left supraclavicular lymph node with increased FDG accumulation. A definite diagnosis could be reached by performing left supraclavicular lymph node dissection under general anesthesia if the giant tumor was malignant lymphoma. However, tracheal intubation could be impossible because of severe tracheal stenosis, and extracorporeal membrane oxygenation (ECMO) is considered to be life-saving when tracheal intubation fails. The catheters were inserted into internal jugular vein and femoral vein before inducing anesthesia. Under the preparation of VV-ECMO when tracheal intubation fails, single-lumen tracheal tube was inserted over the tracheal stenosis, and the ventilation was performed via intubation alone. Under general anesthesia, left supraclavicular lymph node was dissected; however, the dissected lymph node revealed only inflammation and malignant lymphoma was excluded completely. Her symptom of shortness of breath was not so serious although severe tracheal stenosis was observed; hence, there was no urgency of treatment. However, her shortness of breath suddenly worsened after a few days of lymph node dissection and she suffered from wheezing. We decided to perform quasi-urgent lifesaving surgery. Preoperative diagnosis was GIST because of spindle cell and increased FDG accumulation, and differential diagnoses were leiomyoma and neurogenic tumor. Because the tumor was too large to perform enucleation, esophagectomy was first considered.Again, under the preparation of VV-ECMO when tracheal intubation fails, bronchial blocker was inserted over the tracheal stenosis and the left-lung ventilation was performed via intubation alone. Under general anesthesia, the patient was placed in the left lateral position, and right thoracotomy through the fourth intercostal space was performed. Tumor exploration showed the giant tumor occupying the upper mediastinum and heavily compressing her trachea Fig. . First, The resected tumor was soft in elasticity, measuring 90 × 50 × 50 mm Fig. . No signBenign esophageal submucosal tumors (SMT) are relatively rare, and leiomyoma is the most common, accounting for > 80% of these tumors . NeurofiA few reports have described localized esophageal neurofibromas, and Nishikawa et al. summarized 16 cases , 11–16. In summary, dysphagia most commonly manifests in patients with esophageal neurofibromas. Our case is the first report of a patient suffering from wheezing with severe tracheal stenosis and requiring quasi-urgent surgery. The utility of ECMO in patients with severe airway obstruction was reported in the systematic review . From thIt is often difficult to diagnose localized esophageal neurofibroma using diagnostic imaging; thus, histopathologic diagnosis of neurofibroma is necessary in most cases. We hesitated to perform radical surgery without a definite diagnosis as tumor resection and separation from the trachea seemed to be difficult. If the giant tumor was malignant lymphoma, chemotherapy would be effective, and the patient would be saved without radical surgery. It is well known that malignant tumor or malignant lymphoma had increased FDG accumulation; however, Watanabe et al. reported benign esophageal SMT such as esophageal schwannoma had also increased FDG accumulation . The hisSurgery is the primary treatment for esophageal neurofibroma if clinical symptoms are present. Enucleation of esophageal neurofibromas is sufficient treatment if the tumor is small, and the thoracoscopic approach could be adapted for such small tumors , 21–23. It is often difficult to diagnose esophageal neurofibroma preoperatively. The preparation of ECMO could be considered in patients with severe airway obstruction for safe tracheal intubation. This is the first case of life-threatening giant esophageal neurofibroma with severe tracheal stenosis.


Appendicorectal fistula can be a cause of chronic abdominal pain, forming years after an occult episode of appendicitis. It can be diagnosed with Colonoscopy and Magnetic Resonance Imaging, and successfully treated surgically with laparoscopic appendicectomy and stapled segmental cuff resection of the rectum. Due to the uncommon presentation of appendiceal fistula affecting the gastrointestinal tract and the challenges it presents to a surgeon, we discuss the symptoms, preoperative investigations, and our surgical approach to the first reported case of an appendicorectal fistula.Important pathologies of the appendix include acute appendicitis, appendicorectal diverticulitis, inflammatory bowel disease, and malignancy. Appendicitis is a common emergency presentation, and delayed diagnosis or untreated appendicitis can lead to perforation, abscess formation, peritonitis, and rarely death. A rare but recognized complication of acute appendicitis is the phenomenon of appendiceal fistula.the primary perforation of the appendix to an adjacent hollow viscus or to the skin.”Appendiceal fistula was first described in 1846 when an appendicocutaneous fistula was discovered at autopsy.Reported appendiceal pathologies leading to appendiceal fistula include acute appendicitis, periappendiceal abscess, incomplete appendicectomy,Until now, there was no known report of a fistula occurring between the appendix and rectum. Due to the uncommon presentation of appendiceal fistula affecting the gastrointestinal tract, and the challenges it presents to a surgeon, we discuss the symptoms, preoperative investigations, and our surgical approach to the first reported case of an appendicorectal fistula.2A 23‐year‐old female experienced an episode of acute abdominal pain that led to a computed tomography (CT) scan of the abdomen. The CT revealed no intra‐abdominal pathology and her pain resolved spontaneously. However, the CT did demonstrate a long pelvic‐oriented appendix with the tip adjacent to the rectum Figure . She wenAbdominal examination revealed mild lower abdominal tenderness, and rectal examination was normal. Pelvic ultrasound was negative. Proctalgia fugax was suspected. A colonoscopy was performed which demonstrated patchy inflammation around the appendiceal orifice and an upper rectal defect suspicious for a fistula. The defect appearance was similar to an open diverticulum with some mucopurulent exudate at the edges as well as a granulation polyp Figure . A MRI oThe patient was referred for a surgical opinion. Together with her surgeon, the patient elected for surgical resection of the appendix and rectum. At the time of the operation, an 80 mm appendix was found extending from the cecum to the anterior rectum and posterior vaginal vault. The tip was dissected sharply from the rectum using an energy device, and a routine appendicectomy was performed. A defect persisted within the rectum and vaginal vault with chronic inflammatory changes around the cut edges Figure . A decisHistological examination of the appendix demonstrated features of mild acute on chronic appendicitis with crypt architectural distortion and numerous non‐necrotising granulomata and lymphoid aggregates present throughout the appendiceal wall. Granulation tissue was adherent to the serosal surface of the distal tip of the appendix and the rectal wall showed perforation with granulation tissue adherent to the deep margin extending through the wall to the mucosal surface, consistent with fistula formation.3A review of the literature demonstrates that a common presentation of “spontaneous” appendiceal fistula is chronic abdominal pain measured in years. Appendiceal fistula formation should therefore be considered in patients with chronic abdominal pain and a suspected chronic inflammatory process: a distant yet memorable episode of pain may reflect an episode of acute appendicitis. Other risk factors for appendiceal fistula include persistent or recurrent urinary tract infection; previous abdominal surgery; a chronic draining cutaneous sinus or an abscess requiring repeat incision; and drainage. There is also a notable population in patients with cystic fibrosis.Optimal surgical treatment of appendiceal fistula will of course be determined by the underlying pathology and structures involved. In our case, the investigation of a suspected case of proctalgia fugax led to the discovery of an appendicorectal fistula by colonoscopy and MRI, which may have been the sequelae of an episode of acute appendicitis on a long pelvic‐oriented appendix years earlier. As there was no evidence of malignancy, a local resection of the appendix and rectum was deemed appropriate. The closure of the inevitable defect in the rectum was achieved by novel stapled segmental cuff resection which owes its origins to and is a recognized treatment of endometriosis.4This is the first reported case in the published literature of an appendicorectal fistula following what appears to be an acute on chronic episode of acute appendicitis. Appendiceal fistula is a rare phenomenon that is important to diagnose and can be challenging to treat. Awareness of the types of appendiceal fistula and the various surgical options assists a general surgeon to ensure positive outcomes. Stapled segmental cuff resection of a rectal defect is a novel method of closure and treatment of appendicorectal fistula.None declared.SM: Primary author, reporting and literature searches. JK: Critical revision, final approval. PG: Critical revision, final approval. BM: Critical revision. NL: Critical revision.


Chondromyxoid fibroma-like osteosarcoma (CMF-OS) is an exceedingly rare subtype of low-grade central osteosarcoma (LGCO), accounting for up to 10% of cases and making it difficult to diagnose. CMF-OS is frequently misdiagnosed on a radiological examination and biopsy, even after the initial operation. Its treatment is a controversial issue due to its low-grade classification and actual high-grade behavior.We retrospectively reviewed the medical charts of more than 2000 osteosarcoma patients between 2008 and 2019; 11 patients with CMF-OS were identified, of which six patients were treated by our institution with complete clinical characteristics, including treatment and prognosis, radiological and pathological features were reviewed. Three males and three females with a median age of 46 (range 22–56) years were pathologically proven to have CMF-OS. The radiological presentation of CMF-OS is variable, thus radiological misdiagnoses are common. However, one must not ignore a malignant radiologic appearance. The most distinctive pathological feature conferring the diagnosis of CMF-OS is the presence of osteoid production directly by the tumor cells under a chondromyxoid fibroma (CMF)-like background. Differential diagnoses based on comprehensive data from CMF, LGCO, chondrosarcoma (CHS), conventional osteosarcoma (COS), etc., are needed. All patients were treated with an operation and chemotherapy, and one patient received additional radiotherapy. Nevertheless, recurrence and metastasis are common in CMF-OS patients. Relatively invasive biological behavior of CMF-OS is against the low-grade classification of this disease.It is important to recognize CMF-OS and distinguish it from CMF, CHS, COS and other LGCOs. CMF-OS has a relatively poor prognosis despite its low-grade classification. Conventional osteosarcoma (COS) is the most common primary high-grade sarcoma of the skeleton in which neoplastic cells produce bone , 2. It hThere are also several rare histological subtypes of osteosarcoma, including the giant cell rich variant, the osteoblastoma-like variant and the epithelioid variant, etc., in which four variants were defined by Mirra et al.  as low-gWorld Health Organization classification of tumors, Pathology and genetics of tumors of soft tissue and bone in 2002 [CMF-OS was defined as a subtype of COS in the  in 2002 , but was in 2002 .Herein, we report a case series of six CMF-OS patients with detailed clinicopathological and radiological features and review the literature to increase our recognition of this rare tumor. The differential diagnosis, treatment and prognosis of this tumor are also discussed.We retrospectively investigated the electronic charts of patients with a histological diagnosis of osteosarcoma admitted to Shanghai Jiao Tong University Affiliated Sixth People’s Hospital  between January 2008 and May 2019. From among more than 2000 osteosarcoma patients, only 11 were proven to have CMF-OS, of which five were consulting cases and thus additional clinical details and follow-up data are not available. Accordingly, six patients were recruited for the final analysis. The pathological diagnoses were carefully reviewed by two pathologists (QJ and HZ) who specialized in musculoskeletal pathology for 10 and 30 years based on haematoxylin-eosin staining and additional immunohistochemistry (IHC) staining. Imaging findings were reviewed by a radiology resident (JZ) and a radiologist (WY) with 30 years of image diagnostic experience of musculoskeletal diseases based on all available data. The relevant clinical features and related treatments were retrospectively reviewed, and follow-up data were collected from electronic charts, hard copy medical records and telephone calls. The review of the literature available via the PubMed, Embase and MEDLINE in English and China National Knowledge Infrastructure and Wanfang Data in Chinese revealed ten case reports with pathologically confirmed CMF-OS and had sufficient data to extract [The patients’ baseline characteristics are summarized in Table A 21-year-old female presented with a one-month history of pain and weakness of her left knee. A physical examination (PE) showed obvious swelling and limitations in mobility. Significant lab test results included an elevated alkaline phosphatase (ALP) level of 240 U/L. Computed radiography (CR) showed an osteogenic lesion in the distal femur and an associated soft tissue mass. Computed tomography (CT) findings were similar to those of CR. Magnetic resonance imaging (MRI) demonstrated abnormal signals in the distal femur on T1-weighted and T2-weighted images  showed an expansive lesion with sclerosing and cystic changes that was considered fibrous dysplasia or a chondrogenic tumor. CT scan after enhancement demonstrated bone destruction and a soft tissue mass, and the patient was diagnosed with a fibrogenic or chondrogenic tumor  with detailed information were reviewed. CMF-OS involved almost equally in both gender, in contrast to what is typically observed in COS; however, this could be the result of the deficient number of patients. Of these patients, 12 were adults, and three were pediatrics, with ages ranging from 13 months to 56 years (median 37 years), distinct from that observed in COS. In our cases, there are three patient over 50 years suffered from CMF-OS, demonstrated that CMF-OS can occur in middle-aged adults.As a subtype of osteosarcoma, CMF-OS is less commonly found in the long bones of the extremities. In the present case series, only three tumors were located surrounding the knee, the typical site of COS, including the distal femur and proximal tibia. The rest were found in the proximal femur. Accordingly, CMF-OS is more commonly found in craniofacial areas, where COS is uncommonly involved. In fact, seven of fifteen tumors in our summary were located in the craniofacial area. Occasionally, CMF-OS can be found in other sites, including short and flat bones, but it is rare for osteosarcoma to be found in such sites, making it easy to mistake for other lesions. Of the ten cases described previously, only one tumor reported by Chow et al.  was founMost patients presented with a symptomatic mass, swelling or pain, while one patient discovered the asymptomatic mass at an annual medical test. Tumors in the craniofacial area can initiate oral or nasal problems. Although ALP and lactate dehydrogenase (LDH) levels may increase in patients with osteosarcoma and have been proven to be prognostic factors for osteosarcoma , 12, theWe observed several distinct radiological features that enable recognition in an appropriate clinical setting. As a tumor comprised of several components, the radiological presentation of CMF-OS is variable. CMF-OS can present as an osteolytic, osteogenic or expansive lesion with or without calcification with soft tissue masses. Evidence of well, moderately or poorly defined delineation and the appearance of a thin, broken or destructive cortex are also found. A lesion presenting as a large, destructive, poorly defined, mixed lytic and blastic mass that transgresses the cortex and forms a very large soft tissue mass , similarUnfortunately, most of the tumors showed with no distinguishable characteristics, making it difficult to identify their histological origin. However, their relatively invasive appearance indicated to be potentially malignant. CMF-OS involving the craniofacial area (as case 5) is common and confusing. It sometimes could be misdiagnosed as CMF, which is considered as an intermediate tumor with a much better prognosis. The majority of CMFs show radiological features of benign bony lesions consisting of internally lobulated to lobulated translucent osteolytic defects with scalloped and sclerotic margins . In contSeveral distinct pathological features may enable the recognition of such tumors in the appropriate clinical setting. Grossly, a glistening and mucoid cut surface of the tumor reflects a highly myoxid stroma. Microscopically, it shows a CMF-like appearance, including loose aggregates of cells in a highly myxoid stroma background and segregated into lobules by the fibrovascular septa. Furthermore, the most distinctive feature conferring the diagnosis of osteosarcoma is the presence of osteoid production directly by the tumor cells . MoreoveDifferential diagnoses of CMF-OS include CMF, chondrosarcoma (CHS), other LGCOs and COS, as shown in Table Surgical resection is the primary treatment for CMF-OS. All of the cases we reviewed here were removed; nevertheless, only four patients received chemotherapy –6, 8 whiFour of nine patients reported disease-free survival of only up to 6 months , 8–10, wCMF-OS is an extremely rare variant that is more commonly found in atypical sites of osteosarcoma with confusing clinical, radiographic and pathological features. Difficulties in diagnosis are the first hindrance to an appropriate treatment. Many patients did not reach to the correct diagnosis until the first surgery was completed and had to suffer the second one. The involvement of the craniofacial area may also play an important role in its poor prognosis. Almost half of tumors reported were found in the craniofacial area, where complete resection is difficult to accomplish. Indeed, osteosarcomas involving axial skeleton as an adverse factors for prognosis . Last buIn conclusion, a case series of six patients with CMF-OS and related literature were reviewed in this study. The features that enable the diagnosis of CMF-OS were summarized, emphasizing a differential diagnosis between CMF, CHS, COS and other LGCOs. An appropriate treatment selection based on its biological behavior and probable cause of its relatively poor prognosis were discussed, highlighting the necessity for clarifying its classification.Additional file 1: Figure S1. Figure-case-3 (a), (b) Axial CT on the bone and soft tissue windows through the right foot showed destruction and sclerosing of the bone and an associated soft tissue mass with rings and patched calcification. (c), (d) Coronal T1-weighted images demonstrated a homogeneous isointense to hypointense lesion to muscle tissue. (e), (f) Heterogeneous hyperintense lobular lesions were observed on coronal PD-weighted images.Additional file 2: Figure S2. Figure-case-4 (a) Coronal T2-weighted images demonstrated a heterogeneous hyperintense lesion to the grey matter. (b), (c) Before and after contrast administration, the lesion was isointense to hypointense on axial T1-weighted images and then showed heterogeneous enhancement.Additional file 3: Figure S3. Figure-case-5 (a), (b) Axial CT on the bone and soft tissue windows through the right mandible after resection showed no soft tissue mass.Additional file 4: Figure S4. Figure-case-6 (a), (b) Axial T1-weighted and coronal PD-weighted images through the left maxilla demonstrated a soft tissue mass with a heterogeneous signal. (c) Several nodules found on axial CT were considered metastases.


Intravenous antihypertensives were utilized to control his blood pressure. Hemodialysis was initiated given the degree of renal dysfunction. The patient subsequently developed hemolytic anemia, requiring the transfusion of multiple units of packed red cells. He developed acute respiratory failure leading to intubation, but was thereafter liberated from the ventilator following clinical stabilization. Uncontrolled hypertension precipitated by pheochromocytoma can cause microangiopathic hemolytic anemia and renal insufficiency. This case is notable not only for the occurrence of this rare presentation, but also for the severity of manifestations in a young male with no known significant comorbidities.Pheochromocytoma is a rare adrenal tumor that is classically associated with the triad of paroxysmal tachycardia, diaphoresis, and headaches. However, it can have myriad manifestations. We present a case of a 31-year-old male who presented with abdominal pain, hypertensive emergency, and renal failure. Abdominal imaging demonstrated a left adrenal mass. Plasma metanephrines (153 pg/ml,  Pheochromocytoma is a rare form of catecholamine-secreting adrenal tumor with an estimated annual incidence of about three to eight per million . About 3The patient is a 31-year-old African American male who presented to the ED with a chief complaint of upper abdominal pain. This abdominal pain had begun two weeks prior and was progressively worsening. He described it as a mid-epigastric dull ache, aggravated by eating, and lacking in radiation or alleviating factors. He reported associated nausea, vomiting, and nonbloody diarrhea, as well as a weight loss of 8 pounds over the same period of time, which is something he attributed to poor oral intake. A complete review of systems was limited as the patient was lethargic and slow to answer questions. His medical history was limited to an episode of pancreatitis about 5 years prior to presentation. He had not seen a doctor for years prior to presentation, but was told that he had high blood pressure at a plasma donation center a few years before. He took no medications apart from over the counter ibuprofen as needed for pain. His family history was significant only for hypertension in his mother. He had a 10-pack year smoking history, and biweekly use of marijuana. He denied consumption of alcohol and other illicit drugs.μL, n < 10), normocytic anemia , thrombocytopenia , deranged renal function , proteinuria , mild hematuria with metabolic acidosis (pH of 7.25), and anion (gap of 20). A CT scan of the abdomen demonstrated acute colitis, and a low density left adrenal mass measuring 4.4 × 4.4 × 4.5 cm  and normetanephrines , thus confirming the diagnosis of pheochromocytoma presenting as hypertensive emergency. Hemodialysis was resumed and he was started on the appropriate antihypertensive medications. Once deemed clinically stable, the patient was transferred to a tertiary care center for evaluation of surgical resection of the tumor.The following day, during a session of hemodialysis, the patient developed acute respiratory distress requiring intubation for airway support and resumption of nicardipine infusion for hypertensive emergency. A chest X-ray demonstrated bilateral alveolar infiltrates and pleural effusion. High sensitivity troponin was found to be elevated and a transthoracic echocardiogram showed mildly impaired left ventricular function with an ejection fraction of 50% and no significant valvular abnormalities. Over the next 48 hours, his oxygen needs improved and he was subsequently extubated. The workup for secondary hypertension revealed elevated plasma metanephrines  and thrombocytopenia due to endothelial injury and subsequent destruction of RBCs and platelets . The subA review of literature shows us several cases of reported renal failure in the setting of pheochromocytoma secondary to rhabdomyolysis , 10 or cA final note must be made on the diagnostic utility of plasma metanephrine and normetanephrine in pheochromocytoma. The Endocrine Society (ES), in their clinical practice guidelines for pheochromocytoma and paraganglioma (PPGL)  recommenPheochromocytoma is a disease with a myriad of possible presentations. In a previously healthy young patient, presenting with a triad of malignant hypertension, hemolytic anemia and renal failure, pheochromocytoma must be considered as part of the differential.


Kidney cancer is the third most common urologic malignancy and a 2% annual increase in the incidence has occurred over the past two decades, largely because of the increased utilization of imaging. The majority of these tumors are small, so the indications for nephron-sparing surgery and for minimally invasive surgery are continually expanding. Complex kidney lesions, such as those completely endophytic, are still a challenge even for experienced surgeons.Our objective is to demonstrate the operative technique for laparoscopic partial nephrectomy with the aid of intra-operative ultrasound in a man with a totally endophytic renal lesion.Case: A 52 years old man, asymptomatic, with incidental renal mass of 2.9 cm, completely endophytic (R.E.N.A.L score 9p) submitted to partial laparoscopic nephrectomy. Surgical time was 2 hours, with 20 minutes of ischemia. Pathological anatomy confirmed tumor of clear cells T1a, free margins.


A central odontogenic fibroma (COF) is a rare tumor of odontogenic origin with a diverse histopathology found in both the mandible and the maxilla. It can often be difficult to diagnose; therefore, it is necessary to evaluate the clinical, radiographic, and histopathological analyses of COF and co-relate them in a manner to make definitive diagnosis easier for the treating physician. Herein, we report and discuss the first known case of central odontogenic fibroma in Pakistan: a 16-year-old boy presenting as a hard bony painless swelling of the left mandibular region. It appeared as a large, well-defined unilocular radiolucency on the orthopantomogram, making it indistinguishable from other radiolucent tumors of the mandible. Histologically, the lesion consisted of nests of odontogenic epithelium in between the fibroblastic stroma, confirming a definitive diagnosis of COF. It was treated by conservative surgical excision followed by curettage and no postoperative complications were reported. A central odontogenic fibroma (COF) is a rare and benign tumor accounting for only 0.1% of all odontogenic tumors . It may On clinical examination, it usually presents as a non-tender swelling showing cortical expansion ,5-6. HowRadiographically, the COF may be multilocular or unilocular with distinct borders although, in some rare cases, a mixed radiolucent and radiopaque appearance with undefined borders has also been observed .A histological examination of the lesion reveals mature collagen interspersed with fibroblasts. The collagenous tissue is moderately dense to dense and nests of inactive odontogenic epithelium in strands may also be present . BecauseIn this case report, we describe a case of COF in a 16-year-old male, occurring in the left posterior mandibular region. We also highlight the clinical and histological features of this rarely occurring odontogenic tumor.A 16-year-old boy reported to the clinical department of Armed Forces Institute of Dentistry (AFID), Rawalpindi, Pakistan, with a slow-growing swelling on the left side of his face for the past two years. He had no other active complaints apart from a slight discomfort upon mastication. His past medical, family, and social history were considered non-contributory to the case.Upon extraoral examination, marked facial asymmetry extending from the left parasymphysis to the angle of the mandible on the left side of the face was noticed, but no ulceration or change of color of the overlying skin was observed. The submandibular lymph node of the left side was palpable Figure .An intra-oral inspection revealed a swelling extending from tooth 32 to the ramus of the mandible (involving the retromolar area of the left side), causing a displacement of teeth 34, 35, and 37. A bicortical expansion of the mandibular plates was noticed, but there was no evidence of paresthesia.An orthopantomogram of the patient showed a large unilocular radiolucency extending from the left parasymphyseal area of the mandible up to the ramus; the inferior alveolar canal was displaced toward the lower border of the angle of the mandible. Root resorption of teeth 33, 34, 35, and 37 was seen along with the residual roots of tooth 36. The margins of the lesion appeared well defined Figure .Considering the clinical and radiographic evidence, a differential diagnosis of an odontogenic keratocystic tumor, unilocular ameloblastoma, and odontogenic myxoma was made.An incisional biopsy revealed a fibro-osseous lesion and the section of the soft tissue showed fragments of fibrocollagenous tissue with a mild lymphoplasmacytic infiltrate. Sections from the bony tissue showed trabeculae of lamellar and woven bone with osteoblastic rimming and intervening spindle cell stroma.Surgical excision of the lesion was planned two months after the biopsy. The lesion, along with spongy bone, was removed after being exposed intra-orally through the extraction site of teeth 34, 35, 36, and 37  dressing was applied Figure  and the The histopathological report concluded the presence of tissue fragments lined by stratified squamous epithelium with dense lymphoplasmacytic infiltrate. Other fragments from the specimen revealed loose spindle-shaped cells in fibrocollagenous stroma with a myxoid appearance in some areas. Moreover, nests of odontogenic epithelium were also seen in between the fibroblastic stroma Figure .Complementing previous findings with conclusive histopathological results, a final diagnosis of central odontogenic fibroma was established. The patient was scheduled for further follow-ups; on the third-month follow-up, a significant decrease in the patients extraoral swelling was observed Figure .COF can appear to be similar to other odontogenic tumors . HoweverThe occurrence of COF has been reported to be more common in females than males  and is uThe most common clinical sign is the swelling of the maxilla or mandible while pain and paraesthesia are less frequently observed . MoreoveDaniels et al. reported that radiographically, smaller-sized lesions often appeared unilocular whereas the larger lesions, exhibiting more aggressive behavior, such as root resorption of adjacent teeth and expansion of the cortical plates, were often multilocular . In our Histopathologically, COF has been categorized into two types: an epithelium-poor (simple type) and an epithelium-rich type, also referred to as complex or World Health Organisation (WHO) type . The simSections from the specimen of our patient showed fragments of tissue lined by stratified squamous epithelium with loose spindle-shaped cells in a fibrocollagenous stroma. It exhibited a myxoid appearance along with nests of odontogenic epithelium. These findings from our biopsy report allowed us to reach a definitive diagnosis of central odontogenic fibroma of the complex/WHO type.It is important to include other benign tumors in the differential diagnosis of COF because odontogenic myxomas and enlarged, contiguous dental follicles may also show relatively uniform stellate or spindle-shaped cells embedded in a fibrocollagenous matrix . FurtherThe current recommended treatment for COF is conservative surgical enucleation followed by the curettage of the cavity. The recurrence rate is not common and a good prognosis has been witnessed in most cases -3,5-7.We report the first known case of COF, an uncommonly occurring odontogenic tumor, in Pakistan. This tumor is difficult to diagnose because of its non-specific clinical signs and symptoms. It is also generally confused with other tumors of odontogenic origin, as was seen in our case, which was clinically diagnosed as an odontogenic keratocystic tumor, leading to a hindrance in diagnosis and management. Apart from its histopathological classification into simple and complex/WHO types, there are no clear clinical or radiographic diagnostic guidelines to reach a definitive diagnosis of COF. Complete enucleation and curettage of the lesion seem to be the treatment of choice with a good prognosis and few chances of recurrence have been seen so far.


A 50-year-old Japanese man with a two-year history of a painless right scrotal mass visited our hospital. Considering laboratory findings and computed tomography, the patient was diagnosed with an uncharacteristic testicular tumor. No metastases were present on radiographic study at the first visit. Emergent high radical orchiectomy was performed, and the tumor was identified as a squamous cell carcinoma (SCC) of a testicular epidermal cyst. He is alive without recurrence or metastasis six months after surgery. Testicular SCC is an extremely rare tumor. This is the third case of testicular SCC associated with an epidermal cyst in English literature. A large proportion of testicular cancers are seminomas, and most nonseminomas include embryonal carcinomas and yolk sac tumors . We encoA 50-year-old Japanese man with a two-year history of a painless right scrotal mass visited our hospital. He was previously healthy and did not take any medications. His scrotal mass was elastic and hard and had no translucency. We palpated normal testis and epididymis in the contralateral scrotum.We ordered blood laboratory examination and computed tomography (CT). Laboratory data were largely unremarkable and testicular tumor markers were not elevated : 2.9 ng/mL; beta human chorionic gonadotropin (hCG): <0.1 ng/mL). CT revealed a testicular tumor with cystic structure in the right scrotum. The wall of the cystic structure was thickened and enhanced with contrast medium. The tumor size was 48 x 48 x 42 mm . ConsideEmergent high radical orchiectomy was performed. The operation time was 36 minutes and there was minimal bleeding. The resected tumor was cystic and filled with the brown pus-like fluid. He was discharged from our hospital on the second postoperative day.The specimen was submitted for pathological examination. Hematoxylin-eosin staining revealed SCC developing from the cyst in the parenchyma of the testis . The neoAfter discharge, he was followed up with CT and tumor marker (SCC). At six months after operation, SCC was within the normal range (1.1 ng/mL) and CT showed neither recurrence nor metastasis.Most primary testicular tumors are germ cell tumors, and primary testicular SCCs are very rare, with only three cases reported in the English literature –5.The incidence of secondary (metastatic) testicular tumor has been reported as 0.06%, with origins including the lung, prostate, gastrointestinal tract, skin (melanoma), and kidney , 6. We tWith regard to the diagnosis of testicular SCCs, it is common to suspect a malignant transformation of a teratoma or from other origins. In this case, the carcinoma developed from an intraepithelial tumor of the cyst, and the cyst did not have cutaneous appendages, bone, or cartilage. CT also revealed no other neoplastic lesions. From the abovementioned information, the tumor was diagnosed as a primary tumor that developed from an epidermal cyst.Up to now, three cases of primary SCCs of the testis have been reported in the English literature –5. BryanShah et al. collected 141 cases of testicular epidermal cysts , with paIn general, squamous cell carcinogenesis has been thought to be related to chronic irritation and inflammation. However, with regard to testicular SCCs alone, it is unclear whether such mechanisms exist.Due to its rarity, there are no guidelines or prognostic data available. However, as previously mentioned, epidermal cysts have the potential for malignant transformation. All SCC cases developing from epidermal cysts have been reported in patients over 50 years old , 5. TherWe reported the third case of testicular SCC associated with an epidermal cyst in English literature.


Primary angiosarcoma of the adrenal gland is both a rare and aggressive malignancy. Differentiating it from more common adrenal masses such as adrenal adenomas, adrenal cortical carcinomas, and metastatic carcinomas is one of several diagnostic challenges. Immunohistochemical analysis is imperative to arrive at the correct diagnosis. Treatment typically involves surgery and adjuvant chemotherapy, but prognosis remains poor. Angiosarcoma is a rare high-grade malignancy that originates from the endothelium of blood and lymphatic vessels. It accounts for less than 1% of all soft tissue sarcomas -3. PrimaA 58-year-old man with a history of type one diabetes mellitus presented to his primary care physician with hematochezia, left-sided abdominal pain, and fatigue for two months. A complete blood count was significant for anemia with a hemoglobin of 8.8 g/dL. He was subsequently admitted to an outside institution where he underwent a colonoscopy which revealed two sigmoid polyps consistent with tubular adenoma and a hyperplastic polyp. One week following discharge, he continued to have left-sided abdominal pain which prompted admission to our institution. Here, a computed tomography (CT) scan of his abdomen revealed a large, heterogeneous, partially necrotic retroperitoneal mass measuring 15 cm x 10.9 cm x 13.9 cm arising from the left adrenal gland as well as prominent retroperitoneal lymph nodes  scan following two cycles of therapy showed several hypermetabolic soft tissue nodules in the surgical bed and left retroperitoneum concerning for disease recurrence. Due to the presence of only one viable kidney following his nephrectomy, ifosfamide was avoided and he was subsequently started on palliative chemotherapy with single-agent doxorubicin. A PET scan following three cycles of doxorubicin therapy revealed interval disease progression in his surgical bed and metastatic disease in the abdominal soft tissue inferior to the stomach as well as to the right adrenal gland. His chemotherapy regimen was then switched to gemcitabine with vinorelbine for three cycles. He continued to have disease progression on scans and decided to transfer his care to another institution, where he was later started on carboplatin therapy. However, CT imaging following two cycles of carboplatin therapy showed extensive retroperitoneal metastatic disease progression with a new mass in the distal colon. Because he did not show any response to these four lines of therapy generally used for angiosarcoma, he was started on etoposide-doxorubicin-mitotane therapy which is typically reserved for advanced adrenal cortical carcinoma . During Adrenal angiosarcoma is a highly uncommon and aggressive malignancy which is difficult to diagnose. It was first described by Kareti et al. in 1988 . It is mHowever, making the diagnosis of primary adrenal angiosarcoma is challenging for several reasons. First, it is a very rare malignancy and is often not considered in the initial differential diagnosis of an adrenal mass. Therefore, it is important to consider and potentially rule out these other causes which include adrenal cortical carcinoma, pheochromocytoma, adrenal adenomas, primary hyperaldosteronism, lymphoma, metastatic adenocarcinoma, metastatic melanoma or metastatic angiosarcoma. There is often confusion with carcinoma of the adrenal gland, especially when the stains are not conclusive as in this case. It is an important distinction to make, because these diseases are treated differently. Furthermore, it is sometimes difficult to identify on biopsy due to the level of necrosis and hemorrhage found in specimens Figures -2B 7]. . 7]. TheThere is no standardized treatment protocol due to the low incidence of this malignancy. Patients with resectable masses usually undergo adrenalectomy. Due to the aggressive and infiltrative capacity of the disease, other visceral organs in the vicinity often need to be removed as was the case of our patient. Median overall survival is higher in those with localized disease following surgery . AdjuvanOur case highlights the importance of using appropriate laboratory tests and immunohistochemical studies to differentiate between the various types of adrenal masses. Treatment of this malignancy, like its diagnosis, remains challenging due to the absence of a standardized treatment protocol and the often aggressive capability of this disease. A multidisciplinary approach through the expertise of medical oncology, surgical oncology, and pathology is essential for disease management.


Extrinsic compression with resulting obstruction of the iliac veins is recognized as a crucial cause of deep vein thrombosis (DVT). We report a case of impacted ureteric stone causing DVT.A 65-year-old man presented with left lower limb swelling that diagnosed and treated as extensive DVT. Further evaluation revealed incidental left severe hydroureteronephrosis and impacted ureteric stone compressing iliac vessels. Immediate percutaneous nephrostomy done followed by left ureterolithotripsy 6 weeks later.several iliofemoral venous thromboses have been reported due to extrinsic compression by various pathologies, but to best of our knowledge, this is the 1st report of the impacted ureteric stone causing life-threatening proximal DVT.Isolated unilateral iliofemoral DVT may raise the suspicion of pelvic pathology including ureteric stone. Thrombo22) with extensive pitting edema of the left lower limb from the groin to the knee joint with calf tenderness. Color Doppler ultrasound revealed an extensive DVT involving common iliac, external iliac and common femoral vein as well as superficial femoral down to popliteal vein. He was treated by bed rest, elevation with bandaging of left leg, 6000 IU of low molecular weight heparin subcutaneously twice a day and further evaluation was performed to find the underlying etiology. Investigations including hematological, immunological, biochemical, lipid profile, protein S and protein C were normal. Abdominal and pelvic ultrasound (US) showed incidental finding of severe left hydroureteronephrosis with almost lost of cortical thickness, for that abdominal and pelvic computed tomography (CT) scan revealed marked left-sided hydroureteronephrosis and an impacting stone measuring (18 × 10 × 10 mm) at the level of L5/S1 (A 65-year-old man referred to the emergency department for evaluation of the lower extremity swelling associated with pain for four days. Physical examination demonstrated an overweight man (BMI 29.6 kg/mof L5/S1  with sigof L5/S1 . Next daof L5/S1 . Next da3In a review of normal anatomy, the ureter enters the pelvis, where it crosses anteriorly to the iliac vessels, which usually occurs at the bifurcation of the common iliac artery into the internal and external iliac arteries. Here, the ureters are within five cm of one another before they diverge laterally .Studies have demonstrated that there is an anatomical predilection for the left iliofemoral vein to form thrombi as an acute setting . It was Extrinsic compression leading to iliofemoral venous thrombosis has also been attributed in several cases caused by various pathologies, but to best of our knowledge, this is the 1st report of the impacted ureteric stone causing life-threatening proximal DVT . In the 4Isolated unilateral iliofemoral DVT may raise the suspicion of pelvic pathology including ureteric stone. In this situation, definitive management should deal with the pelvic diseases.There is no conflict of interest.None to be stated.Approval has been given by Ethical committee of Sulaymaiyah Surgical Teaching Hospital.NO 25.2018.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Design and idea: Mohammed Abed Al Kadum Hassan, Rawa Hama Ghareeb Ali, Fahmi H. Kakamad, Okba F. Ahmed and Hewa Mahmood Toffeq.Drafting: Rawa Hama Ghareeb Ali, Hewa Mahmood Toffeq and Okba F. Ahmed.Data aquision: Rawa Hama Ghareeb Ali, Okba F. Ahmed, Fahmi H. Kakamad.Final revision: Mohammed Abed Al Kadum Hassan, Rawa Hama Ghareeb Ali, Fahmi H. Kakamad, Okba F. Ahmed and Hewa Mahmood Toffeq.Not applicable.The corresponding author is the guarantor of submission.Not commissioned, externally peer reviewed.


Hydatid disease is a worldwide zoonosis endemic in many countries. Liver echinococcosis accounts for 60–75% of cases and may be responsible for a wide spectrum of complications in about one third of patients. Some of these complications are potentially life-threatening and require prompt diagnosis and urgent intervention. In this article, we present our experience with common and uncommon complications of hepatic hydatid cysts which include rupture, bacterial superinfection, and mass effect-related complications. Specifically, the aim of this review is to provide key imaging features and diagnostic clues to guide the imaging diagnosis using a multimodality imaging approach, including ultrasound (US), computed tomography (CT), magnetic resonance (MR), and endoscopic retrograde cholangiopancreatography (ERCP). Rupture of hepatic hydatid cyst occurs in about 35% of cases: “waterlily sign,” “snake/serpent sign,” or the “snowstorm pattern” suggest contained rupture of hepatic hydatid cyst; intracystic fat suggests communicating rupture of hepatic hydatid cyst within the biliary tree.Superinfection of hepatic hydatid cyst may lead to higher incidence of postoperative complications and life-threatening complications.Hepatic hydatid cysts may grow in size and lead to significant mass effect on adjacent structures, including portal vein, hepatic veins, biliary tree, right diaphragm, stomach, and kidney.Echinococcus tapeworm . Hyd. Hyd7]. metastatic hydatidosis—and may manifest dramatically as a life-threatening condition with anaphylactic shock or acute abdomen due to chemically induced peritonitis [Communicating rupture of hepatic hydatid cysts into adjacent viscera, peritoneal cavity, or pleural cavity is known as direct rupture and occurs in about 6% of cases . Direct itonitis . CT and itonitis . Communiitonitis  or from itonitis . The ideitonitis . In patiitonitis .Fig. 9ASuperinfection may complicate ruptured hepatic hydatid cysts in up to 25% of cases , 20, 21.Hepatic hydatid cysts may gradually increase in size of about 1 cm in the first 6 months, and then of 2–3 cm each year depending on compressibility of surrounding structures . HepaticThe diagnosis of hepatic hydatid cyst is based on epidemiology , presence of pathognomonic features at imaging, and, in some cases, confirmation by various immunodiagnostic methods. Only in few cases histopathology or parasitology directly showing protoscolex or hooklets in cyst fluid is needed. Specifically, if US or CT imaging findings allow a confident diagnosis of hepatic hydatid cyst, no further test is usually needed, and patients can be treated depending on the stage of echinococcosis. Conversely, if reliable US or CT imaging diagnosis is not possible, MR imaging and further serological test can be performed to reach final diagnosis. In patients with history of hepatic echinococcosis, the onset of symptoms including fever, nausea and vomiting, jaundice, and raise of liver hepatic test should prompt the suspicion of complications. In regard to complications, US should be performed as first imaging technique, but it is usually not sufficient for the assessment of complications of hepatic hydatid cysts. Depending on availability and patient compliance, CT and MR imaging—including MRCP—may be performed alone or in combination for a more comprehensive diagnostic assessment of complications of hepatic hydatid cysts. In addition, an invasive combined diagnostic and therapeutic approach is usually indicated in specific cases including ERCP in case of suspicion of communication with biliary tree and surgery for peritoneal dissemination.Complications of hepatic echinococcosis occur in about one third of patients and may potentially be life-threatening if not promptly diagnosed. Clinical diagnosis of complicated hepatic hydatid cysts may be difficult because of non-specific symptoms. Adequate knowledge of diagnostic imaging clues of complications of hepatic echinococcosis is crucial to guide the clinical, radiological, and therapeutic management.


Synchronous occurrence of different types of neoplasms is not very frequent, representing around 6% of all cases of cancer. Usually there is a lack of information on how to treat these patients, especially when both types of cancers are also uncommon. No cases of synchronous gallbladder adenocarcinoma and gastric gastrointestinal stromal tumor have been published before.We present the case, management and follow-up, of a 66-year-old female with incidental diagnosis of a pT2NxMx gallbladder adenocarcinoma after elective cholecystectomy that latter, during staging, was also diagnosed with GIST. Total gastrectomy, wedge resection of the liver and lymphadenectomy were performed due to the new findings. Adjuvant chemotherapy for 36 months was indicated. After 16 months of the treatment she has no signs of recurrence.Gastrointestinal stromal tumors (GISTs) had a turnaround in the end of the 20th century after the introduction tyrosine-kinase inhibitor to the adjuvant treatment and now the trend is to extend it up to 36 months in selected patients. Gallbladder adenocarcinoma is an uncommon cancer but the incidental diagnosis is increasing with the popularity of laparoscopic cholecystectomy and, thus, specific management should be offered for these patients, what frequently includes a complementary surgery. Although, GISTs may be associated with another synchronous tumor in 20% of the cases, the simultaneous occurrence with gallbladder cancer is incredibly rare.Simultaneous occurrence of gastric GIST and gallbladder adenocarcinoma has not been reported before and, thus, any information about it may help in the management of those patients. In 1921, Owen showed 4.7% of cases of multiple tumors in a 3000 case-series [Gallbladder adenocarcinoma and gastric gastrointestinal stromal tumor (GIST) are both uncommon types of cancer and its simultaneous occurrence has not been reported previously. We report a case of a woman with incidental diagnosis of gallbladder adenocarcinoma after elective cholecystectomy with a subsequent diagnosis of GIST during the staging.Patient has signed an informed consent and agrees with the following article in its entirety, also, this publication was approved by a Local Research Committee, is registered in the national research database and has been reported according to SCARE criteria .2A 66-year-old black woman was submitted to elective laparoscopic cholecystectomy on August 2016, when she had asymptomatic cholelithiasis diagnosed on a routine abdominal ultrasound. Her past medical history included just arterial systemic hypertension with irregular treatment. She denied any other comorbidities and did not have any previous surgeries. The surgery and her recovery were uneventful.The pathological analysis of the gallbladder revealed an infiltrative neoplasia with large nucleus causing architectural destruction and intestinal metaplasia. It was reported as an adenocarcinoma of 1,1 cm with free margins, subserosal invasion, with no lymphatic or perineural involvement, staging pT2NxMx .Fig. 1GaIn October 2016 a staging abdominal computerized tomography(CT) was performed and showed irregular wall thickening in the proximal gastric segment with exophytic component close to the lesser curvature, measuring 5 × 4,5 cm . No hepaOne week later an upper gastrointestinal endoscopy found a submucous lesion 3 cm below the cardia, with central ulceration and indurated edges . HistopaOn November 2016, total gastrectomy with Roux-en-Y anastomosis, wedge resection of the gallbladder bed (resection of IVb and V liver segments) and hepatic hilar lymphadenectomy were executed. Pathological analysis revealed a 4,5 × 3,5 cm GIST with 12 mitosis in 5 mm² (20 high power fields), high histologic grades (G2) and free margins. Thirty lymph nodes were evaluated, and none showed signs of malignancy. Liver segments were not compromised. GIST was staged as a pT2N0M0. The patient was classified as high-risk of recurrence since the mitotic count was greater than 10/50 high power fields (NIH modified classification) and, thus, adjuvant chemotherapy was considered beneficial for her.After the surgery, the patient developed esophagojejunal fistula which was managed conservatively. She also had asymptomatic pulmonary thromboembolism in the right descendent interlobar artery, which was treated with anticoagulation.On February 2017 she started a 36 months treatment with Imatinib mesylate  400 mg daily and so far, after 16 months follow-up, she has no signs of recurrence, with CEA, CA19-9 and abdominal/chest CTs with no abnormalities.3kit gene have an important role in the oncogenesis of GISTs with more than 95% of GISTs expressing c-kit [Gastrointestinal stromal tumors (GISTs) had a turnaround in 1998 when it was identified that the gain-of-function mutations of the c-ng c-kit . The useng c-kit .Nowadays, GIST is an atypical case of solid tumor which receives adjuvant therapy for more than one year. In 2012, a multicentric research conducted in four European countries allocated equally 400 patients between a group that received Imatinib for 12 months and another group that received it for 36 months. Individuals who received 36months had greater RFS  and longer overall survival . [p = 0.004 and p = 0.003 respectively) when compared to T2 tumor without liver involvement [® and European Society for Medical Oncology recommend that all postoperative incidentally diagnosed gallbladder cancers above T1a should do gallbladder bed resection and lymphadenectomy including porta hepatis, gastrohepatic ligament and retroduodenal lymph nodes since simple cholecystectomy has a 5-year survival rate of only 20–40% [Gallbladder adenocarcinoma is also rare and ranks sixth for all gastrointestinal tumors. It has an important geographical variation, with higher incidence rates among American and South American Indians . It is aolvement . Currenty 20–40% .Several reports evaluating the simultaneous occurrence of GISTs and synchronous gastrointestinal neoplasms have been released over the last decade. Approximately 10–20% of patients with GISTs have a second primary tumor . The firIn summary, patients with GIST may have unknown pathogenic mechanisms that may increase the risk of developing other types of cancer. The proper identification and description of these links carry immense clinical implications, from screening and prevention to diagnosis and treatment. Due to the rarity of GIST and gallbladder adenocarcinoma synchronous occurrence, its evaluation and management become very challenging.The authors do not have any conflict of interest in this research.This study did not have a funding source.Local Research Committee of Hospital Israelita Albert Einstein approved the publication of this case report under the reference number 2.946.926. The study has been also registered in the national research database (Plataforma Brasil).The patient agreed and signed an informed consent allowing the publication of his data including radiology and pathology images.Paulo Henrique Peitl Gregório: Study concept, writing paper, data collection and outpatient clinic consultation.Lucas Seiti Takemura: Writing paper, literature review and data collection.André Luiz Vilela Galvão: Writing paper, literature review and data collection.Giuliano Campolim Gagliotti: Surgeon in both procedures, outpatient clinic consultation and data collection.Rodrigo Camargo Leão Edelmuth: Surgeon in both procedures, outpatient clinic consultation and data collection.Vanderlei Segatelli: Pathological analyzis and photography of specimens and paper reviewer.This study has been registered in the national research database (Plataforma Brasil).Registration number (CAAE):99493018.3.0000.0071.Paulo Henrique Peitl Gregório - Division of General Surgery, Hospital Israelita Albert Einstein, São Paulo, Brazil.Not commissioned, externally peer-reviewed.