We describe the management of a tracheoesophageal fistula due to a damaged tracheal stent, which was first inserted to treat tracheal stenosis. A 29-year-old woman with a history of treated epilepsy had a seizure and suffered from smoke inhalation during a fire. Breathing difficulties appeared and gradually worsened; consultation was obtained two years afterward. After undergoing a thorough examination, the patient was diagnosed with tracheal strangulation. A noncovered, metallic stent was inserted. When the patient was 37 years old, she was admitted to our hospital for the treatment of a tracheoesophageal fistula. We diagnosed it as a tracheoesophageal fistula due to the collapse of the damaged tracheal stent toward the esophageal side, and we decided to perform a mediastinal tracheostomy. Granulation may be formed in the circumference of a stent that has been present for a prolonged period, and removal of the stent may become difficult. This case suggests that insertion of a noncovered, metallic stent is contraindicated for a benign disease. A 37-year-old woman was admitted to our hospital for the treatment of a tracheoesophageal fistula. Medical history revealed that she had epilepsy and took anticonvulsant medication continuously from the age of 10 years. When the patient was 29 years old, she had an epileptic seizure when a fire occurred, and she suffered from smoke inhalation. Chronic coughing and breathing difficulties appeared a few months after the fire. The symptoms gradually worsened, and hospital consultation was obtained two years afterward. As a result of a thorough examination, she was diagnosed as having breathing difficulty due to tracheal strangulation. A noncovered, metallic Ultraflex Tracheobronchial Stent  was inserted approximately 2 years after the fire. Since there was no evidence of congenital disease, the cause of tracheal stenosis was attributed to smoke inhalation based on her history. Although her condition improved after stent insertion, repeated bronchoscopic medical treatment was carried out for modification of the stent and granulation formation.One year after stent insertion, the patient experienced postprandial obstruction. Since the airway narrowing had worsened, she returned to the hospital. An attempt was made to remove the stent completely. However, it was possible to extract only part of the stent due to adhesion of the stent and the trachea. The stent had been inserted in the trachea on the proximal side while the tracheotomy was performed. Subsequently, food was discharged from the tracheotomy, and a tracheoesophageal fistula was diagnosed. For this reason, nutrition was provided primarily via the gastrostoma.When the patient was 37 years old, she was admitted to our hospital for a procedure to remove the stent and close the tracheoesophageal fistula. Computed tomographic (CT) scans showed strangulation of the stent on the proximal side from the tracheotomy. The part of the stent distal from the tracheotomy was not a circular form. The deepest part of the stent was inferior to the left innominate vein . Based oEsophagoscopy revealed the following: stenosis in the cervical esophagus, granulation in the distal side, and the tracheal tube in the distal side . When thWe diagnosed it as a tracheoesophageal fistula due to the fact that the damaged tracheal stent collapsed to the esophageal side, and we decided to operate. The patient was intubated from the tracheotomy and underwent right thoracotomy at the fifth intercostal space. We planned to observe the length of the damaged trachea through the intercostal thoracotomy incision. The operation was due to be stopped when the remaining trachea was shortened. The thickening of pleural membranes and adhesions were observed in the upper mediastinum. First, the upper esophagus was peeled away from the trachea. Adhesions were intense, so that they went to the proximal side. We could peel the esophagus only to the lower end of the stent but no further. At this point, we judged that the respiratory tract reconstruction would require an anterior mediastinal tracheostomy .A U-shaped incision was made just above the isthmus of the thyroid, and a vertical midline incision was made from the bottom of the U incision to the level of the third rib. The sternocleidomastoid muscles were divided at their origins. The pectoralis muscles were reflected off the chest wall bilaterally to expose the ribs and their costal cartilage. With a sternal saw, the anterior thoracic breastplate was resected, including the medial third of the clavicles, the medial segments of the first and second costal cartilages, and the upper third of the sternum to the upper edge of the third rib. The ample removal of the anterior thoracic wall allowed excellent exposure of the thoracic inlet and access to the cervicothoracic esophagus. The trachea was divided gently from the esophagus, and the trachea and the damaged stent were extracted . The lenThe medical treatment for airway narrowing includes laser surgery, balloon extension, a stent, a tracheotomy, and tracheal plastic surgery. The treatment is chosen according to the cause and the condition of the stenosis. Stents, either silicon or metal, are placed so that the trachea and bronchus may remain expanded. Silicon stents, such as the Dumon Stent , have projections for the prevention of migration.pressure on the respiratory tract caused by malignant diseases, such as lung cancer, esophageal cancer, or thyroid cancer , 3;strangulation due to a benign disease as in this example, including smoke inhalation and bronchomalacia , 5;bronchoesophageal fistula.The cases for which stent insertion is needed include those withHowever, the following complications may occur after placement of an airway stent for benign disease: stent migration, sputum retention, and granulation tissue at the ends of the stent. In this case, the noncovered Ultraflex Tracheobronchial Stent was inserted in the tracheal strangulation. A metal stent is made so that insertion can be relatively easy and so there is relatively little sense of incongruity after insertion compared with a stent made from silicon. However, granulation is formed in the circumference of a stent that remains in place for a prolonged period, and removal of the stent may become difficult. This is one of the precautions that should be considered prior to stent insertion for a benign disease. Since there is no cover, it may develop erratic adhesions to the trachea with time. If a stent becomes damaged and deformed, it may become difficult to remove and may cause damage to an adjacent tissue. In this case, the tracheal epithelium had stretched to the inner aspect of the stent. Moreover, the area of the stent that was damaged by the removal operation underwent retroflexion to the esophageal side, causing esophageal injury and a tracheoesophageal fistula. Although the damaged esophagus was restored with sutures without excising the trachea, the stent could not be removed; therefore, the mediastinal tracheostomy was extended.The findings in this case should alert surgeons to the fact that benign strangulation is a contraindication, not an indication, for therapy with a noncovered, metallic stent. Thus, the remaining options for medical treatment for strangulation due to benign diseases include laser ablation, surgical resection, balloon extension, a tracheotomy, tracheal plastic surgery, or use of a covered metallic or silicone stent.This case suggests that insertion of a noncovered, metallic stent is contraindicated for benign disease. A covered or silicone stent must be selected for benign cases.


Atrophic variant of dermatofibrosarcoma protuberans (DFSP) is a distinct form of DFSP.COL1A1-PDGFB fusion gene. Thus, congenital atrophic dermatofibrosarcoma protuberans was diagnosed. No metastasis to the lungs or regional lymph nodes was found on magnetic resonance imaging. Wide local excision and split-thickness skin grafting was performed and neither recurrence nor metastasis has been observed for 5 years and 8 months since the surgery.Here, we report the case of a 19-year-old woman with a small congenital atrophic plaque on the right precordium. The lesion remained atrophic for more than 10 years. Several years earlier, a portion of the plaque became tuberous and enlarged. Physical examination revealed a 25 × 30 mm erythematous atrophic plaque surrounded by three hard, smooth, and orange-colored nodules of varying sizes on the right precordium, along with visible subcutaneous adipose tissue and cutaneous veins. Biopsy of the nodule and atrophic plaque revealed dense proliferation of spindle-shaped tumor cells from the dermis to the subcutaneous adipose tissue, and positive immunostaining for CD34 and vimentin in addition to negative staining for factor XIIIa and α-smooth muscle actin. Reverse transcription polymerase chain reaction (RT-PCR) of the tumor tissue revealed the presence of a This case indicates that a congenital atrophic lesion could represent a quiescent phase of DFSP. Awareness of this rare condition can aid with early diagnosis and thereby improve the prognosis of DFSP. COL1A1) gene with the platelet-derived growth factor β-chain (PDGFB) gene [COL1A1-PDGFB fusion gene is detected in >90 % of cases of DFSP [Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive mesenchymal neoplasm of the skin characterized by high rates of local recurrence after surgical excision but a low risk of metastasis to the lymph nodes and other organs . DFSP isFB) gene . The COL of DFSP  and reprCOL1A1(exon25)-PDGFB(exon2) fusion gene detected by polymerase chain reaction (PCR) confirmed the diagnosis of congenital atrophic DFSP. Recognition of this rare atrophic variant of DFSP can lead to early diagnosis and to improve the disease prognosis.The DFSP lesion is usually present as a tumor that protrudes above the surface of the skin. Atrophic DFSP, a recently identified rare variant of DFSP, appears as a depressed plaque –10. In tThe goal of this report is to highlight the potential clinical variability in the presentation of congenital DFSP and the importance of considering this diagnosis in pediatric patients with atypical cutaneous or subcutaneous tumors.A 19-year-old woman presented with an atrophic plaque and small nodules on the right precordium. The atrophic plaque was present at birth and remained quiescent for more than 10 years. Several years prior, a portion of the plaque became tuberous and enlarged, and the patient presented to our institution. She was otherwise healthy and had no systemic symptoms and no remarkable family history. On initial examination, a 25 × 30 mm erythematous atrophic plaque was seen on the right precordium, along with visible subcutaneous adipose tissue and cutaneous veins  of the tumor tissue revealed gene fusion between exon 25 of DFSP, a cutaneous fibrohistiocytic tumor of intermediate malignancy , constitDFSP usually appears as an indurated exophytic plaque that protrudes above the surface of the skin. Some patients with DFSP have atrophic plaques that might be clinically quiescent but have rarely been reported and characterized in detail. The atrophic variant of DFSP was first reported in 1985 by Lambart et al.  as dermaab initio, and 4) atrophic plaque. Hanabusa et al. [Martin et al.  analyzeda et al.  reportedCOL1A1-PDGFB fusion gene is absent in ML-DDH [Atrophic DFSP is a soft tissue tumor of intermediate malignancy that has no difference in prognosis compared to typical DFSP . Atrophin ML-DDH , which dDFSP is a locally aggressive tumor with a high postsurgical recurrence rate. Surgical resection is the standard treatment for DFSP; however, complete resection is sometimes difficult because the minimum resection margin needed to achieve local control remains undefined . Local rMetastasis is rare occurring in only 2–5 % of cases, and the most common route is hematogenous dissemination to the lungs . AlthougCOL1A1) on 17q with the platelet-derived growth factor β gene (PDGFB) on 22q [PDGFB is constant (exon 2), whereas in COL1A1, the break may occur in any of the exons codifying the alpha-helical region (exons 6–49) [COL1A1-PDGFB fusion variants have been shown to be involved in the pathogenesis of DFSP, with exons 25, 32, and 47 being the most frequently involved [COL1A1 break points within the fusion gene and histopathological subtypes of DFSP or the clinical characteristics [COL1A1 to exon 2 of PDGFB. To our knowledge, this is the fourth reported case of atrophic DFSP in which the COL1A1-PDGFB fusion gene was detected: Exons 25 [COL1A1 were previously reported to be involved in the pathogenesis of atrophic DFSP. These findings imply that even in atrophic DFSP, detection of the COL1A1-PDGFB fusion gene is a powerful tool for molecular diagnosis to differentiate other benign/malignant neoplasms.Translocation of t17;22)q22;q13) is the cytogenetic feature of DFSP. This chromosomal rearrangement leads to fusion of the collagen type I α1 gene  on 22q . In all  is the c exons 6–9 [COL1A1Exons 25  of COL1AHere, we presented the case of a patient with congenital atrophic DFSP with a lesion that had been recognized at birth and remained quiescent for a long period of time before developing into a tumor. This atrophic variant of DFSP is believed to represent an early stage of the lesion, and awareness of this condition can aid in early diagnosis and thereby improve the prognosis of DFSP.Written informed consent was obtained from the patient for publication of this Case Report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


A 37-year-old previously healthy man presented with a localized headache and a progressive enlarging mass in the left frontal area for more than 8 months. He also reported a history of head injury in the same area sustained two years before. On physical examination, the mass (about 28 mm in diameter) was hard to pressure with freely mobile skin above the lesion in the left frontal sus-orbital region. Ophthalmologic and neurologic examinations were normal. Cranial computed tomography scan showed an osteolytic intradiploic lesion with poorly defined margins (A and B). Magnetic resonance imaging showed hyperintense lesion on T2-weighted image and hypointense on T1-weighted image with gadolinium enhancement (C). The lesion was extradural with slight brain mass effect. Surgery consisted of total resection of the bony bluish lesion (D) and cranioplasty. The postoperative course was uneventful. Histological study revealed a cavernous hemangioma of the diploe. Bone hemangioma is a vascular hemartoma: a benign tumor arising from the intrinsic vasculature of the bone, commonly seen in long bones and vertebrae. Skull cavernous hemangiomas are rare tumors for which the origin is not yet clear. We suspected that in our case head injury may have been the cause of cavernous hemangioma in the cranial vault.


Taenia solium. Although cysticercosis is a common disease in some regions of the world and can occur in any body site, oral lesions are rare. In this report, we document the case of oral cysticercosis in a 10-year-old boy who sought treatment for an asymptomatic nodule on the dorsal surface of the tongue. A detailed history, thorough clinical examination, morphological appearance and the histopathologic findings of the excised cyst formed the basis for the diagnosis of the lesion.Cysticercosis is a condition in which a human acts as the intermediate host of the pork tapeworm How to cite this article: Goenka P, Sarawgi A, Asopa K, Gumber P, Dutta S. Oral Cysticercosis in a Pediatric Patient: A Rare Case Report with Review. Int J Clin Pediatr Dent 2016;9(2):156-161. Taenia solium, a pork tapeworm. The life cycle of T. solium is characterized by different stages of development, requiring various kinds of hosts that can appropriately harbor the eggs (proglottids), the oncospheres, the larvae and the adults.12 Cysticercosis in humans is common in the cerebral tissue, subcutaneous tissue, muscle and the eye.2 The pathological conditions manifested are usually the functional disturbance of the infected tissue such as seizure and visual impairment.3 The oral cavity is a rare site of involvement by cysticercosis, even in an endemic area.45 In addition, cysticercosis presenting as a nodule or mass in the tongue is even more rare.6 A correct and precise clinical diagnosis is infrequently established and often confused with other benign lesions of the oral cavity.57 We report here a case of oral cysticercosis that was diagnosed based on the clinical findings, and the morphological and histopathologic appearance of the lesion.Cysticercosis is a condition in which humans act as the intermediate host of A 10-year-old boy reported to the dental clinic with a swelling on the tongue . The lesThe cyst was enucleated under local anesthesia and was sent for histopathologic examination  and 3. TThe patient was referred to the Department of Pediatrics for thorough systemic examination. Computed tomography (CT) of the head and neck was found to be unremarkable. Stool, urine and blood failed to show active parasitosis. Oral antihelminthic drug was planned for the patient. To avoid any immune response to the parasitic byproducts, steroids were started first. Prednisolone 20 mg 8 hourly was administered orally for 5 days. On the 4th day, Albendazole 600 mg once daily was started and was continued for 28 days. The patient was monitored for 24 hours after administration of the first dose of Albendazole. The patient was scheduled for periodic examinations to assess his clinical status, which remained satisfactory for 3 years of follow-up.Taenia solium (tape worm) is a hermaphrodite cestode for which human beings are the only definitive host. The adult worm is composed of the head (scolex) and numerous proglottids and may reach up to 6-10 ft. The proglottids  are liberated by the humans in excreta. Cysticercosis develops when these eggs are ingested by humans and pigs (intermediate hosts) and oncospheres (embryos) are liberated by the action of gastric acid and intestinal juices. Infestation is usually via the oral route after consumption of contaminated food or drinks, or by unclean hands  or, rarely, by reflux of the proglottid from the intestines into the stomach.7 The oncospheres and larvae that are formed in the stomach cross the bowel wall and actively reach destinations like brain, skeletal muscle, eye and subcutaneous structures through blood and lymphatics. Reaching these organs, the larvae become fluid-filled cysts known as the ’bladder worm’ or cysticerci.89 The pictographic description of the tapeworm life cycle has been shown in 10 In a large series of 450 cases, Dixon and Lipscomb11 detected oral involvement in only eight cases (1.8%). The condition in pediatric age group (0-18 years) involving the oral structures is even rarer. A review of few reported cases of cysticercosis in pediatric age group is shown in Although cysticercosis is highly prevalent in some parts of the world , oral and perioral lesions are relatively rare.12 According to the literature, oral cysticerci usually elicit a clinical diagnosis of mucocele, or a benign tumor of mesenchymal origin, such as lipoma, fibroma, hemangioma, lymphangioma, granular cell tumor or a minor salivary gland adenoma.141314 The lesion presented in this case as a firm nodule, thus making lipoma and hemangioma as less likely diagnosis. The consistency of lipoma is usually soft to fluctuant and histologically is composed of adipocytes that are subdivided into lobules by septet of fibrous connective tissue.15 Moreover, hemangioma presents as a flat or raised lesion, usually deep red or bluish which is often traumatized leading to surface ulcerations and secondary infections. Although tongue is the most common intraoral site for lymphangioma, it is a less probable diagnosis as they usually are present since birth or appear at a very early age. The histopathologic findings were also not in favor of either hemangioma or lymphangioma.Although involvement of the tongue musculature by cysticercosis is common in swine, this location is rare in humans. No explanation for this phenomenon has been given, but some authors have suggested that the high muscular activity and metabolic rate of these muscles in humans might act against the lodgment and development of the cysticercus in this location.16 Microscopically, granular cell tumors exhibit round or polygonal cells with small excentrically placed nuclei and abundant pale eosinophilic granular cytoplasm. The cells are usually arranged in unencapsulated sheets but may also be found in cords and nests. The histopathologic picture found in the presented case did not resemble the one described for granular cell tumor. Thus, granular cell tumor was also ruled out as the diagnosis for this case.20Very often the clinical presentation of granular cell tumors is very similar to the one presented in this case, i.e. a solitary, slow growing, painless, smooth and sessile mucosal swelling with a firm texture and color varying from normal or slightly pale to yellowish. The lesion can appear at any age with a peak age of 40-60 years and a female predilection of 2:1.Although larvae or its fragments were neither identified in FNAC nor in histopathologic examination of the postexcision specimen, based on clinical and morphological pointers the lesion was suggested to be a parasitic cyst. Aspiration of a clear fluid with white flakes, intramuscular site, mixed inflammatory response with predominance of eosinophils and plasma cells, presence of palisading histiocytes and calcified structures on microscopic examination were highly suggestive of an intraoral cysticercosis.10 In contrast to the severity of the disease in cerebral, ocular or cardiac sites, oral lesions are usually well tolerated; however, it is important to carry out a detailed study in every case to exclude the presence of the parasite in other sites. In order of frequency, the tissues affected by cysticercosis are subcutaneous layers, brain, muscles, heart, liver, lungs and peritoneum.21 The intensity of the signs and symptoms produced by cerebral cysticerci  depends on the number of invasive oncospheres present and their anatomic location. In some cases, the symptoms may even suggest the presence of a cerebral neoplasm.22 Iridocyclitis, secondary glaucoma and cardiac arrhythmias may also occur.17Oral cysticerci are firm nodules on palpation because of its high intraluminal pressure.2324 The immunodiagnosis of human cysticercosis can be achieved in sera, cerebrospinal fluid and saliva by either enzyme-linked immunosorbent assay (ELISA) or enzyme-linked immunoelectrotransfer blot.2526 Enzyme-linked immunoelectrotransfer blot has a specificity and sensitivity superior to ELISA for the diagnosis of cysticercosis.27 Enzyme-linked immunoelectrotransfer blot for cysticercosis antibodies is highly sensitive in patients with multiple intracranial lesions, but it is less sensitive in patients with single or calcified lesions.28 Apparently, imaging techniques are more reliable than serological tests for the diagnosis of neurocysticercosis.22Radiologic imaging, serology and tissue biopsy can be used to confirm a diagnosis of cysticercosis. Imaging techniques, in particular CT and magnetic resonance imaging (MRI), are of great value to diagnose cerebral cysticercosis.10 In the present case also, a simple surgical excision of the lesion was done, which was well tolerated by the patient.Oral cysticerci are usually easy to excise and the prognosis is good. In all cases, simple surgical excision seems to be sufficient to ensure complete removal of the lesions without postoperative complications. Treatment of multiple cysts may be unnecessary in asymptomatic individuals after confirmation of diagnosis, but in every case a thorough clinical and epidemiological survey has to be done to identify the possible source and magnitude of the problem in a given community.28 Praziquantel (isoquinoline) is a broad-spectrum antihelminthic, whose efficacy to treat parenchymal neurocysticercosis has been confirmed in several long-term follow-up studies throughout the world.29 Clinical trials for the treatment of neurocysticercosis have revealed that both Albendazole and Praziquantel reduce the number of cerebral lesions as demonstrated by serial MRI and CT scans.30 Considering the epidemic nature and the severity of the disease, a future therapeutic alternative to regulate the transmission of helminthic disease could be vaccination.Albendazole is currently the drug of choice for the treatment of systemic cysticercosis. It is an imidazole with antihelminthic properties. It was first used to treat human neurocysticercosis in 1987.


Following 18 cycles, tumor sizes were reduced and 18-fluorodeoxyglucose positron emission tomography (18FDG-PET) examination revealed obvious reduction of tumor FDG uptake. Transarterial tumor embolization (TAE) was performed for the previously unresectable right subphrenic liver tumor, and the other tumors were surgically resected. The resected tumors were diagnosed as liver metastases and a local recurrence of SPT in the postoperative pathological examination, which revealed that the resected tumors were composed of sheets of bland cells, which were positive for CD10, CD56, vimentin, neuron-specific enolase and α-antitrypsin. The postoperative course was uneventful, and the patient is currently under observation at an outpatient clinic; postoperative adjuvant chemotherapy with oral S-1 has continued, and additional TAE is planned. In the future, if the middle segment of the liver becomes enlarged, surgery for the residual right lobe tumor may be possible. This case demonstrates one method of SPT treatment: Preoperative HAI chemotherapy with GEM, plus oral S-1 and TAE. If complete resection can be achieved, the majority of patients with SPT have a favorable prognosis. In patients with unresectable metastases from SPT, it is crucial to conduct systematic multimodal treatment to maximize treatment success.A 33-year-old female was diagnosed with a solid pseudopapillary tumor (SPT) of the pancreas and multiple liver metastases at the Department of Gastroenterological Surgery, Ishikawa Prefectural Central Hospital . Distal pancreatectomy and postoperative systemic chemotherapy with gemcitabine (GEM) and S-1, an oral fluoropyrimidine derivative, was administered, however, liver metastases became enlarged and local recurrence occurred. Therefore, the patient was referred to the Department of Gastroenterologic Surgery at the Graduate School of Medicine  for hepatic arterial infusion (HAI) chemotherapy. Oral S-1 (80 mg/m Solid pseudopapillary tumor (SPT) is a rare, nonfunctional neoplasm of the pancreas that occurs most frequently in young females with a mean age of 25 years  . The firThe current study reports a case of SPT with multiple liver metastases and local recurrence following distal pancreatectomy for the original lesion; the patient was treated with hepatic arterial infusion (HAI) chemotherapy, systemic chemotherapy, transarterial embolization (TAE), and surgical resection. Written informed consent was obtained from the patient.A 33-year-old female was admitted to the Department of Gastroenterological Surgery, Ishikawa Prefectural Central Hospital  in December 2006 with abdominal pain and vomiting. A cystic lesion of 10 cm in diameter was detected in the tail of the pancreas as well as multiple liver tumors using abdominal computed tomography (CT) . In JanuPhysical examination identified palpable hard masses of >10 cm in diameter around the umbilical area and right lower quadrant. Abdominal contrast-enhanced CT revealed multiple heterogeneous solid and cystic tumors in the liver, and a large tumor of 15 cm in diameter was identified in the left subphrenic area, indicating local recurrence following distal pancreatectomy . The lat2) and HAI with GEM  were initiated as described in a previous report  was a viable treatment. However, TAE was likely to cause the other tumors to rupture due to post-treatment necrosis. Therefore, TAE was performed for the unresectable right subphrenic liver tumor occupying the anteroposterior segment, and the other tumors were surgically resected once the patient’s condition stabilized post-TAE.The resected tumors were diagnosed as liver metastases and a local recurrence of SPT on postoperative pathological examination. The tumor specimen was a highly vascular lesion composed of sheets of bland cells with oval to round nuclei, moderate cytoplasm, ill-defined cell borders, and a focal pseudopapillary appearance . On immuThe postoperative course was uneventful, and the patient is currently under monthly observation at an outpatient clinic and receiving adjuvant chemotherapy with oral S-1 .SPTs of the pancreas are uncommon low-malignant epithelial tumors that are typically identified in adolescent females. Metastatic disease is rare and only occurs in around 10–15% of patients –11. PrevThe primary morphological differential diagnosis for SPT is pancreatic neuroendocrine tumor. Traditionally, negative staining for neuroendocrine markers, in particular chromogranin A, has been considered crucial to this distinction. However, a recent study demonstrated aberrant nuclear staining for β-catenin and a loss of membranous expression of E-cadherin with aberrant nuclear localization of the cytoplasmic domain in all pancreatic SPT cases analyzed . In thisAt present, no consistent clinical or histological criteria has been established to predict the biological behavior of SPT. Invasion of blood vessels, peritoneal infiltration, invasion of adjacent structures, a high degree of cellular polymorphism, and an elevated mitotic rate are characteristics proposed to be associated with metastases and recurrence. However, the absence of these features does not preclude malignant behavior. In the present case, the patient exhibited multiple liver metastases at initial presentation in the absence of malignant histological behavior.The current study demonstrates that SPT is an indolent tumor with an excellent prognosis and that surgical resection is the mainstay of treatment, even in the presence of local invasion and extrapancreatic involvement. Indeed, involvement of the surgical margins (R1) does not appear to be associated with a poor outcome. In previously reported studies, ≤20% of the cases have exhibited liver metastases at the time of resection, however, the overall five-year survival rate remains >95% . In the et al reported the advantages of neoadjuvant chemotherapy with GEM for a large SPT (The value of chemotherapy for patients with SPT remains unknown, however, a number of anecdotal studies have reported its benefit ,18–20 anarge SPT . In anotarge SPT .In the current patient, oral S-1 and HAI with GEM were administered, which resulted in the reduction of tumor size on CT and an obvious reduction of FDG uptake on FDG-PET analysis. It was originally proposed that S-1 and GEM had been invalidated as treatment options based on their ineffective use at the previous hospital, however, it is possible that a sufficient amount had not been administered due to patient complaints and side effects. In the Department of Gastroenterologic Surgery at the Graduate School of Medicine, combination treatment with oral S-1 and HAI using GEM was effective. However, there is a possibility that oral S-1 administration alone was effective in treating the local recurrence at the left diaphragm, which was not reached by the arterial infusion of GEM but nonetheless decreased slightly in size. Therefore, oral S-1 was used for postoperative adjuvant chemotherapy.Transarterial tumor embolization and transcatheter arterial chemoembolization are seldom used to treat SPT or similar pancreatic neoplasms ,22,27. AIn the current study, TAE was performed for residual liver tumors prior to surgical resection. Oral S-1 was subsequently administered as postoperative chemotherapy, and additional TAE is planned. In the future, if the central region of the liver enlarges, surgery for the residual right lobe tumor may become possible.In conclusion, this case demonstrates one method of treatment for SPT: Preoperative chemotherapy with GEM HAI in combination with oral S-1 and TAE. If complete resection can be achieved, the majority of patients with SPT have a favorable prognosis. In patients with unresectable metastases from SPT, it is crucial to perform systematic multimodal treatment, with a combination of surgery, chemotherapy and interventional radiology, to maximize treatment success.


Endoscopic retrograde cholangiopancreatography (ERCP) is one of the most frequently performed procedures for the diagnosis and treatment of biliary-pancreatic diseases. ERCP-related complications total around 2.5% to 8%, with a mortality rate ranging from 0.5% to 1%. An exceptional ERCP complication is subcapsular hepatic hematoma, and few cases are reported worldwide.We present the case of a 52-year-old woman with a history of recurring upper abdominal pain and a clinical and ultrasonographic diagnosis of obstructive jaundice due to common bile duct stones. After 2 difficult endoscopic biliary procedures, common bile duct stones clearance was obtained. Post-ERCP course was symptomatic with upper abdominal pain and anemization with hemodynamic instability.CT scan demonstrated a 15 cm × 11 cm subcapsular hepatic hematoma filled with air and liquid on the surface of the right hepatic lobe. The patient was successfully treated with the embolization of a small branch of right hepatic artery angiographically identified as the cause of bleeding.Subcapsular hepatic hematoma after ERCP is a rare complication that must be taken into account in the differential diagnosis of symptomatic cases after ERCP. Its diagnosis is based on clinical and laboratory data and especially on imaging . Treatment is often conservative but, in some cases, embolization or percutaneous drainage or surgery may be necessary. Since 2000, when subcapsular hepatic hematoma was first described,3 world literature has not reported more than 20 cases.19 In this case report, we describe: a further rare case of subcapsular hepatic hematoma manifested only with abdominal pain as first symptom, in accordance with most clinical presentations of similar cases already described; an analysis of the possible pathophysiological mechanism and a review of present research literature.Nowadays, endoscopic retrograde cholangiopancreatography (ERCP) is one of the minimally invasive most frequently performed procedures in the diagnosis and treatment of biliary-pancreatic diseases. If performed by experienced professionals, complications related to this total around 2.5% to 8%, with a mortality rate ranging from 0.5% to 1%.We introduce the case of a 52-year-old woman with a history of recurring upper abdominal pain; the last episode required hospitalization after a diagnosis of obstructive jaundice by common bile duct stones. The patient presented jaundice, increase of cholestasis indices, hyperbilirubinemia (mainly direct), ultrasonographic finding of numerous gallstones, and ectasia of the common bile duct to the distal portion, where several gallstones were observed. She had a negative personal history for noteworthy medical and surgical diseases, previous surgery, hemostasis disorders or spontaneous bleeding. Her laboratory tests showed white blood cells 4.5 × 1.000/μL (4–10 × 1.000/μL), red blood cells 4.03 × million/μL (4.3–5.5 × million/μL), hemoglobin 12.9 g/dL (12.5–15.5 g/dL), platelets 192 × 1.000/μL (150–450 × 1.000/μL), bilirubin 6.9 mg/dL (0.3–1.2 mg/dL), aspartate aminotransferase 144 U/L (2–40 U/L), alanine aminotransferase 164 U/L (4–49 U/L), prothrombin time ratio 1.02 (0.8–1.2), international normalized ratio 1.02 (0.8–1.2), and activated partial thromboplastin time ratio 1.16 (0.8–1.2). As a preliminary procedure to subsequent laparoscopic cholecystectomy, the patient underwent a biliary drainage by endoscopic biliary sphincterotomy performed on a 0.035 inch diameter, 450 cm length straight-tip guidewire (producer brand). This rather complicated procedure led to the extraction of multiple small common bile duct stones, but the clearance could not be completed because of respiratory problems in patient that allowed only the placement of a nasobiliary tube. On the first day, a trans-nasobiliary tube cholangiography was performed; as a result, in addition to the tube's endpoint, located in an intrahepatic branch of the right lobe, another 2 massive common bile duct stones were detected. On the second day, a second endoscopic operational procedure was performed and it turned out difficult too; through this procedure, a complete clearance of common bile duct was achieved. At 24 hours from this procedure, patient began complaining of intense mainly upper abdominal pain, radiating to the right shoulder region, with no peritonism or other signs or symptoms, and pain medication was not working; laboratory tests revealed 2.1 g/dL bilirubin (1.6 direct g/dL), aspartate aminotransferase 73 U/L (2–40 U/L), alanine aminotransferase 87 U/L (4–49 U/L), C-reactive protein 9.15 mg/dL (0–0.5 mg/dL), other parameters were within normal limits (including serum amylase). At 36 hours the clinical picture progressed to hemodynamic shock with hypotension, anuria, and hemoglobin levels declining from 11.7 to 8.4 g/dL (12.5–15.5 mg/L). She was treated with transfusion of packed red blood cells and plasma, as well as intravenous administration of crystalloids and antibiotics.Urgent computed tomography (CT) scan demonstrated a 15 cm × 11 cm subcapsular hepatic hematoma filled with air and liquid on the surface of the right hepatic lobe Figures . Urgent 20 By skilled experienced hands, bleeding or hematoma associated with ERCP and involving liver, spleen, intestinal wall, or abdominal cavity turn out to be an extremely rare but potentially serious event which requires prompt recognition and treatment. Subcapsular hepatic hematoma represents an exceptional event, of which world literature described 21 cases  and fever 19%). The onset of these only symptoms, in association with immediate or subsequent hypotension, should suggest the presence of subcapsular hepatic hematoma. However, in agreement with most authors, the incidence of this complication might be underestimated, as most patients might show no symptoms and instrumental post-ERCP monitoring is uncommon.9%. The o


A young male patient presented with multiple swellings on his chest and the nape of his neck. Physical examination revealed multiple small papulonodular swellings measuring 0.5 × 0.5 cm to 2 × 2 cm, that were soft without discharge with no surrounding skin changes or induration. Skin biopsy samples were diagnosed as benign adnexal neoplasm consistent with eccrine spiradenoma, trichoepithelioma, and cylindroma, i.e., Brooke–Spiegler syndrome. Having confirmed this to be a case of eccrine spiradenoma, surgical excision was performed and the raw area was covered with a split thickness skin graft taken from the right thigh and sutured over the raw area. The sternal lesion was circumferentially excised and the wound was primarily closed by Z-plasty. Surgical excision is considered the gold standard for the treatment of these cases, with low rates of recurrence. Around 50 such cases have been reported in the literature to date. Although eccrine spiradenomas are usually solitary and small, the findings in our case underscore the fact that a variety of presentations are possible. With strict clinical suspicion and histological criteria, the correct diagnosis can be achieved, especially when combined with pertinent clinical information and laboratory studies.Level of Evidence: Level V, therapeutic study. Eccrine spiradenoma is an adnexal neoplasm that continues to be designated as a tumor with eccrine differentiation, however, it is currently considered an apocrine process. The disease usually appears as solitary gray, purple, pink, red, or blue nodules on the upper half of the body. These multiple, potentially painful spiradenomas were reported for the first time by Kerstein et al .Eccrine spiradenomas are usually benign and mostly occur in patients aged 15–35 years . To dateWhile eccrine spiradenomas are indeed rare worldwide, the malignant variant is rarer still.Eccrine spiradenomas can be painful, but the rate of malignant transformation is very low. Such transformation to malignancy has been reported to develop spontaneously with a metastasis rate of 50 %, which can cause death.CYLDI gene located on chromosome 16 at position 12.1 [In the reported literature, no sex predilection exists for spiradenomas or malignant spiradenomas. Although the exact etiology of these lesions is unknown, spiradenomas appear to be caused by a defective tumor suppressor gene; in Brooke–Spiegler syndrome (BSS), there is usually a defect in the ion 12.1 .A 25-year-old patient presented with a history of insidious onset of multiple swellings over the chest and the nape of his neck since 5 years of age, which gradually progressing and was associated with an itching and burning sensation. There were no other co-morbid illnesses or significant history.Physical examination revealed multiple small papulonodular swellings measuring 0.5 × 0.5 cm to 2 × 2 cm, which had a soft consistency with no discharge and no surrounding skin changes or induration.Lesions were confined to the skin with no deeper structure involvement Fig. .Fig. 1aThe raised nodules were tender, firm, fixed to the skin, and ranged from 0.5 to 6 cm in diameter. For histological analysis, surgical excisions measuring 24 × 11 cm and 6 × 13 cm were sent for examination.The patient was turned to the prone position, and the lesions were circumferentially excised from the occipital region to the nape of the neck to the back after marking for surgical excision Fig. . The rawThe two skin biopsies were independently diagnosed as benign adnexal neoplasm consistent with eccrine spiradenoma, trichoepithelioma, and cylindroma, i.e., BSS .Multiple sections from both the tissue masses show a lesion centered in the deep dermis. The epidermis appeared unremarkable, and the multiple tumor nodules were of varying sizes situated in the dermis and not connected to the epidermis. They were oval or spherical with tightly packed cells arranged in diffuse, alveolar, or pseudo-rosette formations. The tumor nodules have two populations of cells: a large cell type with a vesicular nucleus and prominent nucleolus with scanty cytoplasm, and a small cell type with a condensed dark nucleus that is located at the periphery of the nodules. There is evidence of eosinophilic, basement membrane-like material around the lobules. However, the majority of the tumor lobules are surrounded by well-defined fibrous capsules. Some nodules show cystic changes, and mild lymphocytic infiltrate was noted throughout the tumor surrounding the nodules. There was no evidence of horn cysts and no evidence of hyaline material around the tumor nodules Figs.  and 7.FiThese are characteristic benign neoplasms that show a substantial degree of differentiation towards eccrine. They consist of two cell types: peripheral small dark cells and central large pale cells. These cells are arranged in well-defined lobules  with no connection to the overlying epidermis. These cells sometimes form primitive eccrine coils and luminal structures. The histopathologic hallmark of eccrine spiradenoma is well demarcated lobules composed of two types of cells .In the present case, special care was taken to ensure proper split thickness skin graft take. The excised area provided a well-vascularized scar-free bed on which the split thickness skin graft taken from the right thigh was sutured over the raw area. Absence of infection was ensured at the recipient site by preparing the wound bed, using quality sutures and applying regular dressings. Also, care was taken to ensure the absence of shear forces to prevent separation of the graft from the bed for graft survival. Meticulous hemostasis during operation was ensured to prevent hematoma formulation, and the operation steps were executed to give the bed a prolonged period for the normal hemostatic process to take effect. As the graft take was satisfactory, the wound healing time was normal and uneventful. The patient was followed up over a period of 6 months, and no recurrence was clinically observed. The patient was instructed to report for a follow-up after a period of another 6 months to ensure that there was no early recurrence.Eccrine spiradenoma is a benign adnexal tumor that appears as a small and bluish nodule that is typically tender on palpation. In our case, multiple tumors were present on the exterior and the interior surface of the upper body. Random multiple tumors have been reported on the chest, upper extremities of the forehead, and scalp . MoreoveCYLD gene; genetic studies have identified a single gene, CYLD1, on 16q12-q13 as being altered in BSS. The penetrance of this gene has been estimated to be between 60 and 100 %. However, mutations in the CYLD1 gene are also found in familial cylindromatosis and familial trichoepithelioma. Therefore, histopathology plays an important role in distinguishing between BSS, FC, and MFT.The literature shows that the onset of nodules may occur at birth. However, they are commonly seen in the second to third decade of life, are more common in females, and occur on the neck and the back. Affected individuals are also at a higher risk of developing benign or malignant tumors of the salivary glands. BSS is an autosomal dominant condition, caused by mutations in the 2 lasers. It has also been shown that the administration of aspirin and its derivatives can result in the rapid formation of new lesions.The various treatment modalities include excision, dermabrasion, electrodessication, cryotherapy, and radiotherapy or applying argon and COAlthough eccrine spiradenomas are usually solitary and small, the findings in our case underscore the fact that a variety of presentations are possible. This case also shows that eccrine spiradenoma is another dermatologic condition that can be distributed along Blaschko lines. The etiology of this condition, which is undoubtedly genetic, needs further investigation. However, the diagnosis is of paramount importance because of potential malignant transformation particularly in the case of multiple or symptomatic lesions. With strict clinical suspicion and histological criteria, the correct diagnosis can be achieved, especially when combined with pertinent clinical information and laboratory studies. Treatments for spiradenoma have not yet been clearly established, but surgical excision is still the gold standard with low rates of recurrence.


With the advent of MRI (Magnetic Resonance Imaging), Synovial lesions around knee are being more and more easily detected. Synovial lesions of knee present with boggy swelling, effusion, pain, and restriction of motion. Differential diagnoses of such lesions include pigmented villonodular synovitis, synovial lipoma, synovial chondromatosis, rheumatoid arthritis, synovial hemangioma, amyloid arthropathy, xanthomata and lipoma arborescens. CT and MRI often help in diagnosis of such lesions. MRI of Lipoma Arborescens has been regarded to have characteristic diagnostic appearance – it includes a synovial mass with frond-like architecture and fat signal intensity on all pulse sequences. Sometimes Lipoma Arborescens can present in conjunction with inflammatory arthritis. Synovectomy is often curative for such conditions.We report two cases where lesions diagnosed as Lipoma Arborescens on MRI subsequently revealed to be chronic inflammatory synovitis, characterized by absence of fat infiltration in histopathological examination – refuting the original diagnosis. There was infiltration of lymphocytes and neutrophils in the synovium, suggestive of chronic inflammatory arthritis. Both of these patients required management from rheumatologist, and had relief of symptoms after use of methotrexate and hydroxychloroquine. We also report a third case, where a loose body appearing as chondral fragment on arthroscopy was subsequently diagnosed as an organized hematoma on histopathological examination.Diagnostic pitfalls after MRI of the knee is not uncommon. For example - normal variant of meniscomeniscal ligaments have been reported as meniscal tears; motion artifacts have been falsely reported as meniscal injuries; and meniscofemoral ligament can appear as free osteochondral fragment. In most of these cases, a routine arthroscopy is enough to clear the confusion. However, as evident in the three cases described here - in some synovial lesions of knee joint, even after MRI and arthroscopic examination, histopathological confirmation may still be prudent. In spite of availability of advanced imaging technologies and high definition arthroscopy equipment, an arthroscopy surgeon still must not forget the value of histopathological examination in establishing the true nature of synovial lesions of the knee joint. Tools and technologies available for diagnosis and management of orthopaedic conditions have come a long way in last few decades. With advent of MRI (Magnetic Resonance Imaging), soft tissue lesions are being more and more easily detected. Magnetic Resonance Imaging (MRI) is often precedes arthroscopic procedures on the knee, and many times, management decisions are based upon MRI. Modern MRI uses superconducting magnets and radiofrequency coils. This produces a high contras image of the bone and soft tissues via manipulation of hydrogen protons  were negative. Plain x-ray image was unremarkable apart from enlarged soft tissue shadows.MRI report concluded Lipoma Arborescens of the synovium of knee joint with characteristic synovial growth pattern; and associated moderate synovial joint effusion extending in to suprapatellar bursa. There were focal benign lesions/marrow edema in the femoral intercondylar region and medial femoral condyle Fig. .Fig. 2MRAs patient was not responding to analgesics and conservative management, Arthroscopic Synovectomy was undertaken. Intra-operative images showed synovial hypertrophy with branching frond like pattern Fig. .Fig. 3ArExtensive synovectomy was done, clearing all compartments of knee from the growth. Samples of the tissue were sent to two different labs for histopathology. Histopathology reports showed fibrocollagenous tissue with dense mixed inflammatory infiltrate comprised of lymphocytes, plasma cells, neutrophils and proliferative blood vessels. For comparison, histopathology of Lipoma Arborescens normally shows synovial villiform structures with mature fat cell proliferation in the sub-synovial layer , sulfasalazine (1gm twice a day) and indomethacin (25 mg thrice a day). At 1-year follow-up, the patient is completely pain free and able to daily activities. He is still on anti-inflammatory medications.A 22-year-old male presented to us with complaint of dull aching pain in the right knee. On Clinical examination, there was effusion, associated with boggy swelling on palpation. MRI report concluded Lipoma Arborescens of the knee. Arthroscopy showed frond-like branching villous synovial growth Fig. . HistopaA 37 years old male patient came with feeling of some moving object in knee, with occasional episodic restriction in movement. It was associated with pain and swelling. X-ray showed normal alignment and joint space. MRI confirmed minimal joint effusion, with a rounded hypointese body in suprapatellar bursa (17 x 16 x 7 mm) suggestive of a focus of nodular synovitis. Arthroscopic surgery was undertaken to remove the body. On macroscopic examination, the loose body hade a white and glistening appearance, like a cartilaginous fragment Fig. . HoweverA summary of all three cases in compiled in Table Diagnostic pitfalls after MRI of knee is not uncommon. However, the literature is minimal on the situations where there was need for histopathology after knee arthroscopy, resulting in change of diagnosis and management. Sanders et al have reported normal variant oblique meniscomeniscal ligament being falsely reported as displaced flap tear of posterior horn of lateral meniscus (Sanders et al. The appearance of Lipoma Arborescens on MRI includes a synovial mass with frond-like architecture and it has been considered diagnostic (Kloen et al. In addition to the separate entities of idiopathic lipoma arborescens and inflammatory synovitis, newer literatures have started reporting about a new entity – lipoma arborescens coexisting or arising in cases of inflammatory arthritis. Yacyshyn et al have reported a case of recurrent knee effusion with positive cyclic citrullinated peptide in conjunction with lipoma abrorescens (Yacyshyn & Lambert This polyarticular clinical presentation described by Coll et al is similar to our Case 2. Furthermore, synovial fluid analysis is positive for RA Factor in our case. However, the negative histopathology report in our case refutes the diagnosis. For a case to be labeled as Lipoma arborescens, it must have fat infiltration in the synovial tissue. In both our cases, this feature was absent. Inflammatory infiltrate with lymphocytes, plasma cells, neutrophils and proliferating blood vessels confirmed chronic inflammatory synovitis. Hence it is possible for inflammatory synovitis to ‘mimic’ the appearance of lipoma arborescens on MRI, with histopathology coming in to clinch the diagnosis. In case 3, we want to highlight the fact that an organized hematoma can appear shiny glistening white, like a chondral loose body, during arthroscopic procedure Fig. . HoweverApart from the small sample size and retrospective nature, our study has one additional limitation. It can not be completely ruled out that there could be a problem with the MRI read-out by the radiologist. We have three points to discuss on this issue. First, the two cases of Lipoma Arborescens were reported at two different MRI centers. So it is less likely that the same problem with read-out happened at both the centres. Second, even if we assume that the problem lies in the read-out and not the MRI per se, then also in the end. the orthopaedic surgeon faces the same problem. If the diagnostic test is ambiguous enough to create scenarios of mis-read in any particular pathology, then it is wise for a surgeon to have a secondary safety-net in the form of histopathology. Third, we believe sending the MRI Scans for re-reporting or second opinion will itself lead to an observer bias in this case, and is not practical in routine day-to-day practice. Perhaps a better solution is to conduct a blinded and structured study, to look for inter-observer and intra-observer variability of MRI imaging modality.Before the days of MRI, diagnosis of soft tissue lesions like Lipoma arborescens used to be very difficult. Histopathology was needed for confirmation of the diagnosis. But with the advent of newer imaging techniques, battery of blood investigations and high-definition arthroscopy equipment, it is not uncommon for role of histopathology to be sidelined in some pathologies. However, it is prudent for an arthroscopy surgeon to always keep this tool handy, and use it whenever in doubt. Ultimately, knee arthroscopy is a useful tool inspect the joint and to retrieve tissue to be sent for histopathological examination, either to prove the diagnosis of lipoma arborescens or to establish the true nature of the synovial lesion.Written informed consent was obtained from the patient for the publication of this report and any accompanying image.


Herein we report a rare case of a giant suboccipital osteoma in a 55-year-old woman presenting primarily due to cosmetic issue. We discuss the management algorithm taken in the patient, highlighting excision of a potentially curable bony tumor only after ruling out its extension to the ear cavity, mastoid ear cells, transverse sinus, and the intracranial compartment.  Osteomas in the occipital and mastoid regions are exceptionally rare with only 137 cases reported in the literature . Compute2 within the right suboccipital region highly suggestive of a giant osteoma (A fifty-five-year-old woman from Chitwan, Nepal, visited our patient surgical outpatient clinic with a chief complaint of slowly progressive swelling at the back of her head. She had detected the swelling since her twenties. There was no history of trauma, redness, ear discharge, deafness, or similar swellings elsewhere in her body. It slowly progressed in size over time. Once it attained a massive size, she sought medical advice. There were no important past medical or surgical illnesses. Her bladder and bowel habits were normal. Examination revealed a bony and sessile swelling on the right suboccipital region and extending below the craniovertebral junction . The ski osteoma . Because osteoma . The les osteoma . The ble osteoma . The posOsteoma is a slow-growing benign mesenchymal osteoblastic tumor formed by mature bone tissue . OsteomaEtiology of the entity includes trauma, previous surgery, radiotherapy, chronic infection, and hormonal factors . They maThe main differential diagnosis includes osteosarcoma, osteoblastic metastasis, isolated eosinophilic granuloma, ossifying fibroma, Paget's disease, giant cell tumor, osteoid osteoma, hemangioma, calcified meningioma, and monostotic fibrous dysplasia , 18–21. Osteomas are resected only if they are symptomatic or else for cosmetic reasons. The surgical target must be outlining normal cortical bone all around the lesion. Because these lesions are limited to the external cortex, finding a plane of cleavage between the osteoma and normal bone is not difficult . If mastHistologically, osteomas are composed of well-differentiated, mature bone characterized by dense lamellae with organized Haversian canals. Histologically, there are three different subtypes: compact, spongiotic, and mixed .The prognosis of the osteoma may be considered the best in terms of cosmetic and curative aspects provided complete excision is undertaken. Malignant transformation has not been reported yet . The recIn young patients with skull osteomas, complete workup needs to be done to rule out Gardner syndrome by screening for the concurrent presence of intestinal polyps, soft tissue tumors, and dental abnormalities .Giant occipital osteomas have been rarely reported in the literature. Like giant osteomas in other locations of the skull, they can reach large volumes but are essentially benign and potentially curable by excision. Proper assessment of its extension especially when it is in the vicinity of the mastoid and the suboccipital regions is imperative to providing complete excision and limiting postoperative complications.


Intraepithelial neoplasias are preinvasive neoplastic lesions found throughout in the digestive system, and when such lesions are discovered in the gallbladder, they are referred to as intracystic papillary neoplasm (ICPN). In the gallbladder, mucinous adenocarcinoma is a rare histologic phenotype, and adenocarcinomas involving Rokitansky–Aschoff (RA) sinuses are uncommon, which were indeed found in a case reported here. A 64-year-old male presenting with upper abdominal pain demonstrated a spherical mass protruding outward from the gallbladder fundus in imaging studies. Laparoscopic cholecystectomy was performed, and the resected specimen revealed a subserosal cystic mass with a small communication with the gallbladder lumen. The cystic mass contained a gelatinous material without solid component. Histologically, the mass was consisted of subserosal cysts lined by atypical columnar mucinous epithelium with micropapillary growth and nuclear stratification. The neoplastic transformation was more pronounced toward the serosal side of the lesion where disruption of the cyst wall, intrastromal mucin lakes, and invasion of the neoplastic cells into surrounding stroma were observed. The epithelium was of intestinal lineage, which was supported by the positive immunoreactivity against CDX2 and MUC2. The cystic spaces were communicated with surrounding RA sinuses, which indicated that the tumor arose in the sinus. The pathological diagnosis was ICPN, intestinal type, with an associated mucinous adenocarcinoma arising in RA sinus. Intracystic papillary neoplasm (ICPN) of the gallbladder is a preinvasive neoplastic lesion and considered to share similar characteristics with intraductal papillary mucinous neoplasm (IPMN) and intraductal tubulopapillary neoplasm of the pancreas and intraductal papillary neoplasm of the extrahepatic bile duct. Mucinous adenocarcinoma of the gallbladder is a rare histologic phenotype, and adenocarcinomas involving Rokitansky–Aschoff sinuses (RA sinuses) are uncommon. Herein, we report a case exhibiting characteristics of the aforementioned three rare conditions, i.e., ICPN with an associated mucinous adenocarcinoma arising in RA sinus.A 64-year-old male presented with upper abdominal pain. He had suffered from similar abdominal pain for 5 months. The blood cell count and liver function tests were normal except elevated gamma-glutamyl transferase of 156 IU/L . Serum DUPAN-2 level  was modestly elevated to 296 U/ml, which was decreased to 64 U/ml after the surgery. CEA, CA 19-9, and SPan-1 levels were normal .Computer tomography demonstrated a cystic mass protruding outward from the fundus of the gallbladder. The tumor, 25 mm in diameter, showed mild contrast enhancement in the cyst wall on portal venous phase Fig. . UltrasoLaparoscopic cholecystectomy was performed with a preoperative diagnosis of cystic gallbladder tumor. A cystic mass was located at the serosal side of the gallbladder fundus. No invasion into surrounding tissues was observed Fig. . PostopePathological evaluation of the resected specimen revealed a mucin-filled subserosal cystic mass with a small communication with the gallbladder lumen at the gallbladder fundus. The cyst wall was partly thickened, which was proved to be severe fibrosis in histological evaluation. The remaining part of the gallbladder had thickened wall with denuded epithelium, consistent with chronic cholecystitis. Histologically, the tumor consisted of subserosal multilobular cysts lined by columnar mucinous epithelium Fig. . There wImmunohistochemical analysis revealed the neoplastic cells were positive for CK7, CDX2, MUC2 Fig. , and MUCThe pathological diagnosis was ICPN, intestinal type, with an associated mucinous adenocarcinoma arising in RA sinus.Intraepithelial neoplasias are preinvasive neoplastic lesions found throughout in the digestive system, and when such lesions are discovered in the gallbladder, they are referred to as ICPN , 2. ICPNMucinous adenocarcinoma is a rare histologic phenotype among gallbladder adenocarcinomas, and by conventional definition, more than 50 % of the tumor contains extracellular mucin . Dursun RA sinuses are the epithelial invaginations extending to the fibromuscular or subserosal layers through the gaps between the smooth muscle. Terada reported that RA sinuses were recognized in 351 out of 540 cholecystectomy specimens 65 %), and their epithelium occasionally showed marked proliferation 5 %, and . They poIn the present case, the papillary epithelium with abundant extracellular mucin showed various degrees of cytoarchitectural atypia, from low- to high-grade dysplasia. There were disruption of the cyst wall, intrastromal mucin lakes with floating clusters of the neoplastic cells, and stromal invasion of the neoplastic cells, indicating that the neoplasm was invasive mucinous adenocarcinoma. The epithelium was of intestinal lineage, which was supported by the positive immunoreactivity against CDX2 and MUC2. The intestinal lineage is one of the five lineages comprising ICPN: biliary, gastric foveolar, gastric pyloric, intestinal, and oncocytic and account for 8 % of the cases . CDX2 anThe unusual presentation of our case had made it difficult to diagnose preoperatively. Besides malignancy, primary hydatid cyst, lymphangioma, and mucinous cystic neoplasm (MCN) are cystic lesions of the gallbladder reported in the literature –17. MCN Interpreting the pathology report and reflecting it to the patient’s management required careful consideration. Cholecystectomy alone might not be an adequate therapy for gallbladder adenocarcinoma with subserosal invasion, since radical re-resection including hepatic resection and portal lymph node dissection resulted in a significant survival advantage . MoreoveIn summary, this report presented a rare case of ICPN with an associated mucinous adenocarcinoma arising in RA sinus of the gallbladder, and this was the first reported case with invasive carcinoma. Surgeons and surgical pathologist should be familiar with the disease which requires meticulous clinicopathological evaluation and careful patient management.Written informed consent was obtained from the patient for the publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Paragangliomas are rare tumors arising from extra-adrenal chromaffine tissues. The diagnosis of non-functional retroperitoneal paraganglioma and its surgical management can be difficult. We report a case of a retroperitoneal paragangliomaof an unusual localization that renders the surgery more challenging. A 40 year-old woman presented to our department with a four-month history of upper quadrant pain with no vomiting, no fever, nor jaundice. Physical examination was normal. Ultrasonography showed a retro duodenal homogenous mass and computed tomographyscan showed a well-circumscribed round mass of heterogeneous density, which was in close contact with the aorta and the left kidney vein. Laboratory tests were normal. The patient underwent surgical management. The surgical exploration found a retroperitoneal tumor that was encapsulated and showing intimate contact with the abdominal aorta. We performed a complete resection of the tumor. Histological examination of the surgical specimen revealed a paraganglioma. The post operative course was uneventful. Paragangliomas are rare tumors. They can be asymptomatic for a long time and thus be diagnosed at late stage. A follow-up of patients is then essential. Surgical treatment is the only radical treatment and should be performed even in paragangliomas in close contact with the great vessels. All paragangliomas are believed to be derivedfrom the neural crest; they can synthesize and store catecholamines. The diagnosis of non-functional retroperitoneal paraganglioma and its surgical management can be difficult. We report a case of a retroperitoneal paraganglioma of an unusual localization that renders the surgery more challenging.Paragangliomas are rare tumors arising from extra-adrenal chromaffine tissues, that is the paraganglia, which are A 40 year-old woman presented with a four-month history of upper quadrant pain with no vomitingnor jaundice. On examination, the patient had slight tenderness in the upper right quadrant with no palpable mass. She was afebrile with no pallor, no pedal edema nor peripheral lymphadenopathy. Ultrasonography showed a retro duodenal homogenous round mass, with no intra hepatic or common bile ducts dilatation. Abdominal computed tomography scan showed a well-circumscribed round mass of heterogeneous density that was in contact with the aorta and the left kidney vein . LaboratParagangliomas are extra adrenal tumors composed of chromaffin cells that can secrete catecholamines. Retroperitoneal paragangliomas are a rare entity. It can occur at any age, most commonly in young adult , 2. The Management of paragangliomas requires a multidisciplinary approach. Surgery is the only curative treatment provided that it is complete –10. It aParagangliomas are rare tumors, most often observed in young adults. They can be asymptomatic for a long time and thus be diagnosed at late stage. Histology is often non-contributory to determining the benign or malignant nature of the tumor. The follow-up of patients is then necessary. Surgical treatment is the only radical treatment and should be performed even in paragangliomas in close contact with the great vessels.


Background. The cerebellum as initial site of distant metastasis from differentiated thyroid carcinoma (DTC) including papillary (PTC) and follicular thyroid carcinoma (FTC) is rare manifestation. Case Presentations. Herein, we present three cases of cerebellar metastasis (CBM) of PTC. Mean age of patients was 67 years (range: 64–72), and mean duration between initial diagnosis and CBM was 49.6 months (range: 37–61). Frequent location was left cerebellar hemisphere and was associated with hydrocephalus. All patients underwent suboccipital craniectomy, and in two patients postoperative intensity modulated radiation therapy (IMRT) was given to deliver 5000 cGy in 25 fractions to residual lesions. Patient without postoperative IMRT had cerebellar recurrence along with lung and bone metastasis after 38 months. However, two patients were found alive and free of disease at the time of last follow-up. Conclusion. CBM from PTC is a rare clinical entity and is often associated with hydrocephalus. Histopathological diagnosis is important to initiate effective treatment, which relies on multidisciplinary approach to prolong the disease-free and overall survival rates. Differentiated thyroid carcinoma (DTC), especially papillary thyroid carcinoma (PTC), commonly metastasizes to regional lymph nodes, lungs, and bones. However, the brain is an uncommon site of metastasis, being found only in 0.15% to 1.3% of cases . SymptomHerein, we describe and discuss the signs and symptoms, diagnostic work-up, differential diagnosis, and management in three cases of CBM from PTC.μg) of L-thyroxine. In February 2010, she presented with three-month history of headaches, vomiting, and gait ataxia. Pertinent neurological findings were dysmetria and dysdiadochokinesia. Stimulated serum thyroglobulin level was raised (51.7 μg/L) with normal antithyroid antibodies. On I-131 whole body scintigraphy (WBS), no foci of abnormal tracer uptake were seen. Magnetic resonance imaging (MRI) of brain revealed a lobulated heterogeneous mass involving the medial left cerebellar hemisphere, measuring 4.0 × 3.5 × 2.5 cm. The mass was associated with surrounding edema, crossing the midline to the right cerebellar hemisphere and compressing the fourth ventricle  ablation of 150 millicuries (mCi) under a diagnosis of PTC, follicular variant (pT2N1aM0), in October 2005. Patient was in complete remission and was taking thyroid replacement therapy with 175 micrograms  without any recurrence in the cerebellum.A 64-year-old Saudi male had been diagnosed with PTC in July 2009 and underwent total thyroidectomy. The tumor size was 2.5 × 3 cm and histopathological variant was tall cell PTC with extrathyroid extension; however no lymphovascular space invasion or lymph node metastases were noted. Final pathological TNM classification was T3N0 M0. After RAI ablation of 100 mCi, the patient was kept on follow-up. In August 2012, he started complaints of headaches and inability to walk. Serum TG level was 3290 cephalus . WBS shocephalus , and posμg L-thyroxine. In May 2013, she presented with 4-month history of vertigo, headaches, ataxic gait, and increased tiredness. Serum TG level was moderately elevated (77 μg/L). MRI brain revealed a 4 × 4 cm heterogeneous mass in left cerebellar hemisphere associated with edema , followed by RAI ablation of 150 mCi in April 2008. Patient remained in complete remission and was on thyroid replacement therapy with 150 th edema . Patientth edema . PostopeCBM as initial manifestation of distant metastasis from PTC is rare. To date, only thirteen cases of CBM from PTC have been published  1–12]. –12. 1–12Diagnosis of CBM is challenging, as these lesions are often mistaken as chordoma, chondrosarcomas, meningioma, or schwannoma on CT and MRI imaging –7. HistoPresence of CBM is generally considered as poor prognostic factor; however, in our patients, the median survival was 37 months range: 21–60), which was found to be in agreement with literature –60, whic, 6, 7.In conclusion, cerebellar metastasis from PTC is a rare clinical entity and is often associated with hydrocephalus. Surgical resection followed by postoperative radiotherapy and RAI ablation is the treatment of choice. However, for such cases, multidisciplinary approach can prolong the disease-free and overall survival rates in PTC patients.


Metastasized pancreatic neuroendocrine tumors are extremely rare malignancies, especially in children. Therefore, therapeutic options are limited, and few standardized therapy regimens exist.We report a case of a 14-year-old white girl. In 2011 she was diagnosed with a metastasized, well-differentiated pancreatic neuroendocrine tumor with expression of synaptophysin and chromogranin A. We describe her clinical course with special attention to her individual therapeutic regimens while bringing together several disciplines of medicine.In patients such as ours, surgical intervention may be the only therapy that will lead to long-term survival. Metastasized pancreatic neuroendocrine tumors (PanNETs) are rare malignancies, especially in children and young adolescents, for which there is no standard therapeutic regimen. These lesions carry a poor prognosis. In this report, we present a case of a teenage girl (14-years old at age of first presentation) with a metastasized well-differentiated PanNET. A multidisciplinary therapeutic approach was designed by pediatric hematologists-oncologists, pediatric surgeons, visceral surgeons, pediatric radiologists and radio-oncologists. The primary tumor as well as its metastases have been kept in check to date. The patient is in good condition with little limitation of her daily activities.In 2010, a 14-year-old white girl presented to our hospital and described having symptoms of ongoing abdominal pain of approximately 3 weeks’ duration that was responsive to analgesic medication. She reported no prior history of nausea, vomiting, diarrhea, fever, sweating or weight loss. Her family history has been uneventful; both parents and a 19-year-old sister are healthy.An abdominal ultrasound taken at that time showed normal upper abdominal organs and multiple enlarged lymph nodes that had previously been thought to be a reaction to a nonspecific infection. Her pain increased 2.5 weeks later, resulting in another visit to the emergency room. An abdominal magnetic resonance imaging (MRI) scan revealed a suspicious area in the head of her pancreas, persisting enlarged lymph nodes and unclear liver lesions . The outer membrane surface of the tumor cells expressed somatostatin receptor 2 (SSR2), and the active proliferative rate of Ki67  was 20 to 25% Fig. . The res68Ga-DOTA-tyrosine octreotide (68Ga-DOTATOC) positron emission tomography (PET)-CT, thoracic X-ray, electrocardiography and echocardiography. Her blood work and urine analysis results are shown in Table Extensive further diagnostic tests were done: laboratory testing and imaging with computed tomography (CT), In proximity to the previously described lesions, a widely spreading tumor with extensive surrounding of her superior mesenteric artery and her common hepatic artery was detected on imaging studies. Compression of her splenic vein and her superior mesenteric vein, as well as obstruction of her portal vein with subsequent intestine vein insufficiency and cholestasis, was also diagnosed. These anatomic findings prompted us to refrain from a (primary) curative surgical approach because of possible life-threatening circumstances.On the basis of these diagnostic test results, an interdisciplinary decision was made to downsize the tumor with an intra-arterial 4-GBq Lu-177/4-GBq yttrium (Y)-90-DOTATOC therapy in combination with an oral radiosensitizing chemotherapy with temozolomide (5 days) and thalidomide (4 days) as an individual approach 2 weeks later. Because of an expected aplasia and the main concern of weakening her overall condition, as well as to avoid endangering the planned extended surgical procedures, cisplatin and etoposide were not used.Despite these interventions, her condition continued to worsen. Acute pancreatitis and biliary complications developed. Analgesic treatment failed to relieve her pain. As a result, surgery was indicated, despite the increased risk for potentially fatal complications.Surgery with tumor resection took place in the same month. The following steps were performed: (1) extended pancreatic head resection, duodenectomy and cholecystectomy; (2) placement of a temporary GORE-TEX mesenteric-caval shunt and a temporary arterial aortohepatic shunt; (3) distal gastric resection with reconstruction with gastrojejunostomy (end to side), pancreaticojejunostomy and hepaticojejunostomy; (4) right hemicolectomy with terminal ileostomy and Hartmann procedure; (5) extended soft tissue resection, including the whole mesenteric root; (6) resection of the portal vein with vessel interpolation by the femoral superficial vein (from left adductor channel); (7) reconstruction of the hepatic artery with end-to-end anastomosis on the celiac trunk; and (8) atypical segmental liver resection of one metastasis (segment 2/3) .The patient recovered without major complications and could finally be dismissed with home parenteral nutrition 4 weeks later.The first DOTATOC-PET scan obtained postoperatively showed two remaining small, right-sided liver metastases in segments 6 and 8.Six weeks later, a right hemihepatectomy without touching the primarily made biliodigestive anastomosis was performed as planned, followed by another DOTATOC-PET scan 1 month later. We detected that the patient was having a good response to therapy without signs of residual tumor. However, splenomegaly with high DOTATOC uptake was diagnosed. Because of the importance of the nucleotide therapy with aspired further consolidating circles and the vanished option for intra-arterial application owing to the resective surgery, splenectomy was the only opportunity to guarantee successful intravenous DOTATOC therapy. Therefore, a splenectomy was performed. In the same procedure, the ileostomy was taken down.The next intravenous Lu-177/DOTATOC therapy was done. In the same month , an oral tyrosine kinase inhibitor medication with sunitinib was initiated. A follow-up examination 2 months later with DOTATOC-PET and MRI scans was unremarkable. The patient recovered in a rehabilitation unit without being dependent on parenteral nutrition any longer.Unfortunately, the next follow up DOTATOC-PET scan taken 1 year later showed increased uptake in her para-aortal lymph nodes and left inguinal soft tissue, so metastases of the primary tumor were expected Fig. . SurgicaAfterward, the patient additionally received intravenous Y-90-DOTATOC therapy. Despite those interventions, the next DOTATOC-PET scan still showed suspicious para-aortic lymph nodes. Repeat resection of the para-aortic lymph nodes was performed, followed by intravenous Ac-225-DOTATOC therapy.Owing to the stable oncologic situation and regarding the cumulative dose of radioactivity, with its increasing risk of chronic nephrotoxicity and bone marrow toxicity, no further nuclear medical therapy was planned as of this writing. Meanwhile, somatostatin (SST) analogue therapy with Somatuline Autogel® 60mg , a long-lasting lanreotide option (injected subcutaneously once per month), as a non-radioactive blockade of tumor receptors was started to achieve remission.The follow-up screening with DOTATOC-PET/CT 1.5 years after her first diagnosis indicated nonspecific nuclide uptake in her right breast and in one left-sided inguinal lymph node, as well as new high nuclide uptake in the right temporobasal part of her brain. Subsequent MRI of her brain confirmed a noticeable lesion raising strong suspicion of cranial metastases. Those lesions were irradiated with the patient in stereotactic positioning, and a good response was observed on the next MRI. In another surgical intervention, the left-sided inguinal lymph node was removed, which was pathologically confirmed as a metastasis of the primary tumor.The patient reports that she is unaffected and repeat imaging is scheduled at short intervals to ensure regular follow-up.The case presentation is summarized in Fig. . They occur in 1 to 2 of 100,000 cases per year, accounting for less than 2% of all digestive malignant tumors and less than 1% of endocrine tumors. PanNETs represent approximately 3% of primary pancreatic neoplasms. Although they may manifest at any age, they most often occur in the fourth to sixth decades of life [PanNETs are rare pancreatic neoplasms compared with their more common exocrine counterparts. Arising from the pancreatic islets of Langerhans, they are also called islet cell tumorsClassically, PanNETs are divided into two groups: functioning pancreatic neuroendocrine tumors (F-PanNETs) and non-functioning pancreatic neuroendocrine tumors (NF-PanNETs). F-PanNETs are hormonally active and may lead to earlier diagnosis because of their hormone-related symptoms. Common types are insulinoma and gastrinoma, and rare types are VIPoma , glucagonoma or somatostatinoma. For example, insulinomas often present with hypoglycemic symptoms and low blood glucose levels and are reversible after glucose intake; gastrinomas present with diarrhea, hypergastrinemia, gastric acid hypersecretion and peptic ulcer diathesis, known as Zollinger–Ellison syndrome .Although NF-PanNETs secrete a number of substances such as chromogranin, synaptophysin, neuron-specific enolase, pancreatic polypeptide and ghrelin, in contrast to F-PanNETs they do not present clinically with hormone-related symptoms. As a result, they often present later in the course of the disease with symptoms such as abdominal pain (35 to 75%), weight loss 20 to 35%) and anorexia and nausea 45%). Less frequent signs include obstructive jaundice (17 to 50%), intra-abdominal hemorrhage (4 to 20%) or a palpable mass (7 to 40%). Symptoms may also be attributable to metastatic disease, which occurs in, for example, the liver or bones (between 32 and 73% of cases are metastatic at diagnosis). NF-PanNETs may occur in association with the multiple endocrine neoplasia type 1 syndrome, von Hippel–Lindau syndrome, neurofibromatosis type 1 and tuberous sclerosis [0 to 35% 5%. Less 68Ga-DOTATOC-PET/CT with a higher spatial resolution than scintigraphy, fine-needle aspiration cytology and/or biopsy, and laboratory tests with CgA [There are different possibilities for a diagnostic strategy: abdominal ultrasound, endoscopic ultrasound, abdominal CT, abdominal MRI, SST receptor scintigraphy, with CgA , 6.The 2010 World Health Organization classification system distinguishes between poorly differentiated neuroendocrine carcinomas (NECs) versus well-differentiated neuroendocrine tumors (NETs). All NECs are graded G3 with a Ki67 index greater than 20%. NETs are divided into G2 grade with a Ki67 index of 3 to 20% and G1 grade with a Ki67 index of 2% or less. Most pancreatic non-functioning (NF) neuroendocrine neoplasms are well differentiated ; NF-NECs are uncommon [In the present case, our patient has been diagnosed with a well-differentiated NF-NEC, despite a Ki67 index of 20 to 25%, with a TNM classification of ypT3, ypN1(8/31), ypM1(liver) according to European Neuroendocrine Tumor Society consensus guidelines. Poorly differentiated pancreatic neuroendocrine neoplasms are characterized by their aggressive tumor biology, absence of SST receptors and poor prognosis.Clinical management involves a multidisciplinary approach, but surgery remains the only curative therapy for early-stage disease. It represents the treatment of choice in cases of localized PanNETs, including typical and atypical resections. In particular, radical resection of pancreatic tumors is associated with favorable long-term outcomes in children and adolescents . PalliatFurther treatment options are SST analogues  for subgroups of patients with slowly progressive, low proliferative PanNETs; systemic chemotherapy  in all PanNETs and NECs; peptide radionuclide receptor therapy  for patients with NETs with the presence of SST receptors; hepatic artery embolization or chemoembolization in patients with liver metastases who are not candidates for surgical resection; radiofrequency ablation in patients with unresectable metastases more than 5 to 7cm in diameter; radiotherapy using high-energy isotope treatment for patients with inoperable disease; immunotherapy , which may be used to offer hormone-related symptom control to patients in whom SST analogue therapy has failed; and molecular targeted therapy  for individual attempts and future options , 3, 8.In our patient, a primary curative surgical approach could not be used, owing to the anatomic findings of the spreading tumor. As the initial PRRT with Y-90-DOTATOC) combined with chemotherapy  failed [0-DOTATOCThe median overall survival for patients with NF-PanNETs is reportedly approximately 38 months. Patients with distant metastases have a median survival of 23 months, as compared with 70 to 124 months for those with localized disease –12. Our PanNETs are rare tumors that often invade adjacent organs and anatomic structures, causing symptoms. Because of its rarity, randomized controlled studies have not been done and current treatment recommendations are based primarily on case series and individual treatment approaches. So far, total resection of the tumor is the treatment of choice and seems to be associated with the best prognosis, particularly when accompanied by further different therapies as mentioned above. Unresectable or partially resectable tumors and recurrent tumors can be treated with local and/or systemic radiation (PRRT) and/or adjuvant chemotherapy to downsize the tumor. Even then, surgery seems to be an important intervention option, which gives the patient relief from symptoms.Written informed consent was obtained from the patient’s legal guardian (and herself) 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.


In the last decade, more medical articles about this subject have been publishedthan in the last 50 yearsThe purpose of this report is to relate a patient with morbid obesity that underwentbariatric surgery and after that developed the Cushing's syndrome caused by anadrenocortical carcinoma.A 54 years old patient was admitted to the endocrinology unit in February 2012. She hada 3-month history of acne, hirsutism, muscle weakness in her lower limbs, increasinglevels of hypertension and diabetes mellitus imbalance. The physical exam revealed arounded face, clavicular fatness, a fatty hump, BP= 200X100 mmHg, BMI=32.4 kg/m². Shehad a gastric bypass due to morbid obesity in 2009, with BMI=52,47 kg/m². Hormonal datawere consistent with CS- ACTH independent .Abdominal computed tomography showed an expanding lesion in the left adrenal area of13.0X9.0 cm, with irregular contour lines and heterogeneous caption of contrast .She was submitted to a left adrenalectomy and partial nephrectomy in April 2012. Heranatomopathological exam result was 630 g, size 21X10X 5.0 cm .; TNM tumor stage was 3-0-0. The pathologic findings were: 15 mitosis in 50 high power fields and the nuclearpleomorphisms was present; it showed the presence of confluent necrosis and capsularinvasion; Ki-67 index was 20% positive according to the immunohistochemical criteriaThere was regression of clinical and laboratorial alterations related to the Cushing'ssyndrome. She had a follow-up as an outpatient and used mitotane 2 g per day. Afterseven months, abdominal and thoracic tomography showed recurrence of the tumor andmetastasis in the lungs .As there was no satisfactory response from mitotane, cisplatin and etoposide, herclinical situation worsened and she died 13 months after having had an adrenalectomy.The patient's daughter has agreed in writing to allow the publication of this study.,,Brazil is the country with the second highest numbers of bariatric surgeries and it hasthe highest number of bariatric surgeons in the world


Background. Spontaneous orbital haemorrhage is a very rare condition and vision-threatening event. It may occur due to trauma, orbital surgery/injections, orbital vascular anomalies, and a variety of systemic predisposing factors. Signs of retrobulbar hemorrhage include proptosis, ophthalmoplegia, increased intraocular pressure, loss of pupillary reflexes, and optic disc or retinal pallor. Both Computed Tomography scan and Magnetic Resonance Imaging may be performed in the diagnosis. Case Report. A 31-year-old woman was referred to our hospital with a complaint of headache and blurred vision following a strong sneeze. Ophthalmological examination revealed mild Relative Afferent Pupillary Defect in left eye. Computed Tomography revealed left hyperdense retrobulbar mass and displaced optic nerve. T1 weighted hypointense, T2 weighted hyperintense and non-enhanced round shape, sharply demarcated lesion measuring 18 × 15 × 14 × mm in diameter compatible with haematoma was detected by MRI. Surgically Caldwell-Luc procedure was performed. Histological examination confirmed haematoma. Follow-up Magnetic Resonance Imaging revealed a small reduction in the size of lesion but not complete resolution. The patient's complaint was regressed. She is now free of symptoms and is still under surveillance. To our knowledge, this is the first reported case of retrobulbar haematoma caused by sneeze. Spontaneous orbital haemorrhage is a very rare condition and a vision-threatening event. It may occur due to trauma, orbital surgery/injections, orbital vascular anomalies, and a variety of systemic predisposing factors such as coagulopathy, anticoagulant medications , dyscrasia , septicemia, and uncontrolled hypertension . ValsalvA 31-year-old woman was referred to our hospital with a complaint of headache and blurred vision following a strong sneeze. She also had nausea and vomiting. Her past medical history was unremarkable. She has no history of alcohol and tobacco consumption. Ophthalmological examination revealed mild Relative Afferent Pupillary Defect (RAPD) in left eye. All blood tests including blood count, electrolytes, coagulation screen, and thyroid function tests were within normal limits. CT revealed left hyperdense retrobulbar mass (70 HU) which displaced optic nerve superomedially Figures . For betSpontaneous retrobulbar haematoma is rare. The largest report of nontraumatic orbital hemorrhage describes 115 cases over a 24-year period and revealed that orbital vascular malformations  were present in 90%, had additional or other predisposing factors in 11% , and had no predisposing factor in 5% [The most common symptoms of retrobulbar hemorrhage include pain, pressure, and loss of vision. Other common symptoms include diplopia, nausea, and vomiting , 5. The RAPD is an extremely significant clinical finding. Optic nerve lesions and severe retinal disease are most common factors RAPD . We deteValsalva maneuver has been reported to be the cause of nontraumatic orbital hemorrhage in patient with no known underlying vascular malformation . In thesCT or MRI is necessary for information about normal orbital structures and pathological lesions. Both of them confirm the diagnosis and exclude other potential causes. Due to the emergent nature of retrobulbar hemorrhage in cases associated with trauma, CT is preferred because of its fast and better visualization of the bony anatomy. The other conditions including vascular anomalies, MRI provide better information . We initThe surgical approach was limited to simple drainage of the collection. If the patient has diffuse bleeding causing compressive optic neuropathy or encysted collection of blood with compressive optic neuropathy, urgent surgical intervention was performed. We performed surgical treatment cause of compressive optic neuropathy.In the literature, we find that sneeze leads to orbital haematoma. To our knowledge, this is the first reported case of retrobulbar haematoma caused by sneeze. That is why we wanted to share it with our colleagues.


Mediastinal localization of hydatidosis is very rare even in endemic areas. The diagnosis is based on typical clinical and radiological criteria.We report a case of a mediastinal location of hydatidosis in a 60-year-old Arab man admitted for chest pain. The chest radiograph showed a rounded and homogeneous opacity. Computed tomography showed a right mediastinal cyst, without other thoracic or abdominal sites. Through a posterolateral thoracotomy, we found a cystic mass in the posterior mediastinum. The patient received a cystectomy with medical treatment based on albendazole. He improved a few weeks later.Mediastinal cysts remain rare, even in endemic countries, which makes initial diagnosis difficult. Our observation shows the importance of keeping this diagnosis in mind when a patient presents with signs of mediastinal compression. Taenia Echinococcus granulosus[Hydatidosis, or hydatid cyst (HC), represents an epidemic disease that particularly affects North Africa and South America where areas of traditional breeding predominate. The pathogenesis is due to the accidental infestation of a human by a dog’s dejections which contained the ranulosus. It afferanulosus. We repoA 60-year-old Arab man, who had pulmonary HC surgery 20 years ago, attended a first consultation 1 month after the onset of symptoms: chest pain with a dry cough associated with inspiratory dyspnea, all operating in a context of apyrexia and conservation of general status. A physical examination on admission was normal. A chest radiography showed a rounded and homogeneous opacity.A computed tomography (CT) showed, at the middle mediastinum in laterovertebral view, behind the right main bronchus, a liquid formation with well limited contours and regular thin wall containing within cubicles separated by septa. After injection of contrast material, the wall and septa were discreetly enhanced Figures  and 2. WHe underwent a posterolateral thoracotomy through the right-side sixth intercostal space, showing a posterior mediastinal cystic mass that occupied the upper and anterior mediastinum. After a needle aspiration of the cyst, several vesicles were removed Figures  and 4. EE. granulosus. Humans can be accidentally infested by ingesting food contaminated with parasite eggs or by direct contact with a sick dog.Hydatid disease is a parasitic disease secondary to the development of the larval form of On entering the intestines of humans, the parasite gains further entry to locate itself in the liver or lung, and its passage into the systemic circulation is responsible for its location in diverse locations . These tThe pathogenesis of mediastinal localization of HC remains controversial . Some fiHC of the mediastinum is often revealed by chest pain and signs of mediastinal compression , which can be discovered incidentally or by a complication (rupture in the heart or in a large vessel) ,5.Imaging plays a vital role in the diagnosis and staging of lesions. Chest radiography oriented the diagnosis by showing a mediastinal water tone, often rounded or oval. Thoracic ultrasound allows confirmation of the diagnosis when the lesion is accessible. Thoracic ultrasound also reveals the fluid character of the opacity and, in many cases, the proligerous membrane, pathognomonic of HC ,6. It alMagnetic resonance imaging (MRI) is indicated in cases of intolerance to iodine or pregnant women. These different ways of cross-sectional imaging  allow, in most cases, HC mediastinal cystic masses to be differentiated from other mediastinal cystic masses, such as enteric cyst, cystic lymphangioma, pleuropericardial cyst and bronchogenic cyst. The existence of locations in diaphragmatic hydatid advocates the hydatid nature of the mediastinal liquid mass. However, in case of doubt, it is only in the intraoperative period that the diagnosis of mediastinal HC is supported . HydatidSurgical treatment of mediastinal HC is essential. It consists of a cystectomy associated with total or partial pericystectomy. The surgical approach is according to cases, a posterolateral thoracotomy, anterolateral thoracotomy or median sternotomy ,4,7. TheThe mediastinum is an atypical location of hydatid cyst, rare even in endemic countries. Diagnosis is based on radiography, biology (hydatid serology) and histology study. This case report indicates that the etiology of hydatidosis should be kept in mind when a patient presents with signs of mediastinal compression.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.The authors declare that they have no competing interests.YM was the surgeon responsible and is the first author, OA was the second surgeon, and was a major contributor in writing the manuscript. MK was the surgeon aid. YB analyzed the patient data regarding the serology. NI recruited the patient. YR was the anesthesist reanimator. BF contributed to the writing and corrections of the manuscript. All the authors read and approved the final manuscript.