A 27-year-old male presented with intestinal obstruction due to a jejuno-jejunal intussusception, 23 years after a childhood nasopharyngeal carcinoma. He was successfully treated for the latter by radiotherapy but sub...A 27-year-old male presented with intestinal obstruction due to a jejuno-jejunal intussusception, 23 years after a childhood nasopharyngeal carcinoma. He was successfully treated for the latter by radiotherapy but subsequently required a mandibuloplasty for presumably facial hypoplasia sequel to the radiotherapy. The present intussusception was resected with wide margins revealing a partly haemorrhagic, polypoid and sessile jejunal tumour measuring 7.7 × 3.5 × 2.6 cm. Microscopy with extensive immunohistochemical studies revealed a relatively rare and highly malignant epitheloid leiomyosarcoma arising from the muscularis propria with extension to the mucosa and serosa. No metastases were found and post-operative follow-up has so far been uneventful. The present case of a malignant tumour presenting as an intussusception in a young adult, was unrelated to a childhood malignant tumour in the same patient. The former was successfully treated by a wide excision in support of the advocated surgical approach to adult intussusception.展开更多
Gangliocytic paraganglioma is a rare neuroendocrine tumor predominantly arising in the second part of the duodenum with rare local recurrence or metastasis to regional lymph nodes.A 92-year-old female presented with o...Gangliocytic paraganglioma is a rare neuroendocrine tumor predominantly arising in the second part of the duodenum with rare local recurrence or metastasis to regional lymph nodes.A 92-year-old female presented with obstructive jaundice.On exam she had pale conjunctiva and icteric sclera.Abdominal examination revealed tenderness in the upper abdomen.Laboratory data was consistent with obstructive jaundice.Computed tomography of the abdomen revealed a dilated gall bladder and a common bile duct(CBD)with no evidence of liver lesions or pancreatic head mass.Endoscopic ultrasonography revealed a 1 cm isoechoic submucosal nodule at the periampullary area,dilated CBD(9 mm),a prominent pancreatic duct(4.1 mm)and a hydropic gall bladder with no stones.Endoscopic retrograde cholangiopancreaticography was performed to relieve obstruction and showed a 1 cm periampullary mass which underwent an en-bloc snare resection.Histopathology analyses with immunohistochemical stains were positive for cytokeratin,synaptophysin,S-100 protein,neuron specific enolase and negative for actin and desmin consistent with periampullary gangliocytic paraganglioma.Periampullary gangliocytic paraganglioma is a rare benign tumor of the small bowel.Common presentation includes abdominal pain and obstructive jaundice which should be included in differential diagnosis of obstructive jaundice.Endoscopic resection is a curative therapy in the absence of local invasion or distant metastasis.展开更多
Background: Epitheloid trophoblast tumor (ETT) is a tenuous type of gestational trophoblastic disease. Although clinically ETT behave in variable fashion, it is considered as a potentially malignant tumor, fatal malig...Background: Epitheloid trophoblast tumor (ETT) is a tenuous type of gestational trophoblastic disease. Although clinically ETT behave in variable fashion, it is considered as a potentially malignant tumor, fatal malignancy occurs in 10% of the cases. Case and Conclusion: A 41 years old patient, who previously underwent Tompkins operation because of congenital uterine septum was admitted to our clinic due to irregular bleeding. Despite of serum hCG levels under the upper limit of normal, histopathology analysis of D&C obtained endometrial samples confirmed the presence of ETT by immunostainings. Henceforth we performed prompt abdominal hysterectomy and preserved the normal structured ovaries. According to the müllerianosis theory of endometriosis aberrant differentiation or migration could cause spreading of cells throughout the fetal organogenesis of the uterus. These cells in an apoptosis week environment might correlate with the initiation of ETT. As a conclusion we suggest further studies to assess the link between ectopic cells and ETT, to gain a better understanding in the pathomechanism of the tumor.展开更多
Sarcoidosis is a multisystem disease characterized by the presence of non-caseating granulomas in affected organs.Pulmonary involvement is the most common site of disease activity.However,hepatic involvement is also c...Sarcoidosis is a multisystem disease characterized by the presence of non-caseating granulomas in affected organs.Pulmonary involvement is the most common site of disease activity.However,hepatic involvement is also common in sarcoidosis,occurring in up to 70% of patients.Most patients with liver involvement are asymptomatic.Therefore,the majority of cases are discovered incidentally,frequently by the finding of elevated liver enzymes.Pain in the right upper quadrant of the abdomen,fatigue,pruritus,and jaundice may be associated with liver involvement.Portal hypertension and cirrhosis are complications linked to long-standing hepatic sarcoidosis.Liver biopsy is usually required to confirm the diagnosis.It is important to differentiate hepatic sarcoidosis from other autoimmune and granulomatous liver diseases.Not all cases of hepatic sarcoidosis require treatment.For symptomatic patients,the first line treatment includes corticosteroids or ursodeoxycholic acid.Various immunosuppressant agents can be used as second line agents.Rarely,severe cases require liver transplantation.展开更多
文摘A 27-year-old male presented with intestinal obstruction due to a jejuno-jejunal intussusception, 23 years after a childhood nasopharyngeal carcinoma. He was successfully treated for the latter by radiotherapy but subsequently required a mandibuloplasty for presumably facial hypoplasia sequel to the radiotherapy. The present intussusception was resected with wide margins revealing a partly haemorrhagic, polypoid and sessile jejunal tumour measuring 7.7 × 3.5 × 2.6 cm. Microscopy with extensive immunohistochemical studies revealed a relatively rare and highly malignant epitheloid leiomyosarcoma arising from the muscularis propria with extension to the mucosa and serosa. No metastases were found and post-operative follow-up has so far been uneventful. The present case of a malignant tumour presenting as an intussusception in a young adult, was unrelated to a childhood malignant tumour in the same patient. The former was successfully treated by a wide excision in support of the advocated surgical approach to adult intussusception.
文摘Gangliocytic paraganglioma is a rare neuroendocrine tumor predominantly arising in the second part of the duodenum with rare local recurrence or metastasis to regional lymph nodes.A 92-year-old female presented with obstructive jaundice.On exam she had pale conjunctiva and icteric sclera.Abdominal examination revealed tenderness in the upper abdomen.Laboratory data was consistent with obstructive jaundice.Computed tomography of the abdomen revealed a dilated gall bladder and a common bile duct(CBD)with no evidence of liver lesions or pancreatic head mass.Endoscopic ultrasonography revealed a 1 cm isoechoic submucosal nodule at the periampullary area,dilated CBD(9 mm),a prominent pancreatic duct(4.1 mm)and a hydropic gall bladder with no stones.Endoscopic retrograde cholangiopancreaticography was performed to relieve obstruction and showed a 1 cm periampullary mass which underwent an en-bloc snare resection.Histopathology analyses with immunohistochemical stains were positive for cytokeratin,synaptophysin,S-100 protein,neuron specific enolase and negative for actin and desmin consistent with periampullary gangliocytic paraganglioma.Periampullary gangliocytic paraganglioma is a rare benign tumor of the small bowel.Common presentation includes abdominal pain and obstructive jaundice which should be included in differential diagnosis of obstructive jaundice.Endoscopic resection is a curative therapy in the absence of local invasion or distant metastasis.
文摘Background: Epitheloid trophoblast tumor (ETT) is a tenuous type of gestational trophoblastic disease. Although clinically ETT behave in variable fashion, it is considered as a potentially malignant tumor, fatal malignancy occurs in 10% of the cases. Case and Conclusion: A 41 years old patient, who previously underwent Tompkins operation because of congenital uterine septum was admitted to our clinic due to irregular bleeding. Despite of serum hCG levels under the upper limit of normal, histopathology analysis of D&C obtained endometrial samples confirmed the presence of ETT by immunostainings. Henceforth we performed prompt abdominal hysterectomy and preserved the normal structured ovaries. According to the müllerianosis theory of endometriosis aberrant differentiation or migration could cause spreading of cells throughout the fetal organogenesis of the uterus. These cells in an apoptosis week environment might correlate with the initiation of ETT. As a conclusion we suggest further studies to assess the link between ectopic cells and ETT, to gain a better understanding in the pathomechanism of the tumor.
文摘Sarcoidosis is a multisystem disease characterized by the presence of non-caseating granulomas in affected organs.Pulmonary involvement is the most common site of disease activity.However,hepatic involvement is also common in sarcoidosis,occurring in up to 70% of patients.Most patients with liver involvement are asymptomatic.Therefore,the majority of cases are discovered incidentally,frequently by the finding of elevated liver enzymes.Pain in the right upper quadrant of the abdomen,fatigue,pruritus,and jaundice may be associated with liver involvement.Portal hypertension and cirrhosis are complications linked to long-standing hepatic sarcoidosis.Liver biopsy is usually required to confirm the diagnosis.It is important to differentiate hepatic sarcoidosis from other autoimmune and granulomatous liver diseases.Not all cases of hepatic sarcoidosis require treatment.For symptomatic patients,the first line treatment includes corticosteroids or ursodeoxycholic acid.Various immunosuppressant agents can be used as second line agents.Rarely,severe cases require liver transplantation.
