A 49-year-old woman presented with chronic abdominal discomfort, significant weight loss, and chronic intermittent diarrhea. She suddenly developed massive upper gastrointestinal bleeding and was referred for further treatment. Endoscopy indicated a large mass in the upper gastric body with antral and duodenal bulb involvement. Endosonography showed a large well-defined isoechoic gastric subepithelial mass with multiple intra-abdominal and peripancreatic lymphadenopathy, suspected to be malignant on the basis of fine needle aspiration cytology. The tumor was surgically removed, and histopathology showed typical characteristics of a neuroendocrine tumor. On the basis of immunohistochemical staining, somatostatinoma, a rare neuroendocrine tumor, was diagnosed. Gastrointestinal bleeding is a rare presentation and the stomach is an uncommon tumor location.
Biopsy, Fine-Needle ; Carbamates ; Diarrhea ; Endoscopy ; Endosonography ; Female ; Hemorrhage ; Humans ; Lymphatic Diseases ; Middle Aged ; Neuroendocrine Tumors ; Organometallic Compounds ; Somatostatinoma ; Stomach ; Weight Loss

Somatostatinomas are rare functioning carcinoid tumors that usually arise in the pancreas and duodenum. They are seldom associated with typical clinical symptoms; their diagnosis is confirmed only by histological and immunohistochemical studies and the presence of specific hormones. Two distinct clinicopathological forms of somatostatinoma exist: duodenal and pancreatic somatostatinomas. Clinically, compared to pancreatic somatostatinomas, duodenal somatostatinomas are more often associated with nonspecific symptoms and neurofibromatosis, but less often with somatostatinoma syndrome or metastasis. Histologically, duodenal somatostatinomas frequently have psammoma bodies in the tumor cells. We report a case of duodenal somatostatinoma in 58-year-old man with vague epigastric pain and nausea. He did not have diabetes, steatorrhea, or cholelithiasis. Abdominal computed tomography showed a 25-mm mass in the duodenum and 25-mm nodule in the liver. Endoscopic retrograde cholangiopancreatography showed a duodenal submucosal tumor. Although the endoscopic biopsies were free of malignancy, the patient subsequently underwent Whipple's operation for the duodenal mass. Examination revealed as a somatostatinoma using a special stain for somatostatin.
Cholangiopancreatography, Endoscopic Retrograde ; Duodenal Neoplasms/diagnosis/*pathology/surgery ; Humans ; Male ; Middle Aged ; Somatostatinoma/diagnosis/*pathology/surgery

Pancreatic endocrine tumors (PET) are rare neoplasms of the pancreas accounting for less than 5% of all primary pancreatic malignancies. Insulinomas, gastrinomas, glucagonomas and somatostatinomas is included in PET. PETs are usually classified into functioning and non-functioning tumors and presents with a range of benignity or malignancy. It is very important to accurate diagnose the PET location and to predict the benignity or malignancy of PET in terms of the treatment strategy, because PET have higher respectability, better response to chemotherapy and better prognosis compared to that of pancreatic adenocarcinoma. The utility and reliability of different imaging modalities depends on the characteristics of PETs, specifically their size. Functioning PET tend to be small (less than 2 cm), well circumscribed, homogeneous, and usually shown as strong enhancement on contrast enhanced CT or MR imaging. Non-functioning PET tend to be larger (4~10 cm), heterogeneous, and may contain the cystic areas of degeneration and necrosis. In this article, we present the various imaging findings of PET according to recent WHO classification.
Accounting ; Adenocarcinoma ; Gastrinoma ; Glucagonoma ; Insulinoma ; Magnetic Resonance Imaging ; Necrosis ; Pancreas ; Prognosis ; Somatostatinoma

Blood Glucose ; analysis ; Humans ; Hypoglycemia ; diagnosis ; etiology ; pathology ; Male ; Somatostatinoma ; complications ; diagnosis ; pathology ; Young Adult

Pancreatic endocrine tumors (PET) are rare neoplasms of the pancreas and account for less than 5% of all primary pancreatic malignancies. Included in this group are insulinomas, gastrinomas, glucagonomas and somatostatinomas. Collectively, these neoplasms are classified as functional PETs. When a PET is not associated with a clinical syndrome due to hormone oversecretion, it is referred to as a non-functional PET. Non-functionalPETs are pancreatic tumors with endocrine differentiation but lack a clinical syndrome of hormone hypersecretion. Although a pancreatic carcinoma shows aggressive biological behavior, a cutaneous metastasis from a pancreas carcinoma is rare. We report a case of a case of a cutaneous metastasis from an endocrine pancreatic carcinoma in a 50-year-old female that clinically manifested as a painful firm nodule on the back.
Female ; Gastrinoma ; Glucagonoma ; Humans ; Insulinoma ; Middle Aged ; Neoplasm Metastasis ; Pancreas ; Pancreatic Neoplasms ; Somatostatinoma

Somatostatinoma is a rare endocrine tumor that comprises around 1% of all gastroenteropancreatic endocrine neoplasm. The estimated annual incidence is 1 in 40 million. This tumor may be associated with von Recklinghausen's disease. We present here a rare case of a 51-year-old female patient with a duodenal nonfunctioning somatostatinoma combined with von Recklinghausen's disease. Whipple' procedure was performed. The postoperative course was uneventful and the histopathologic findings were consistent with malignant nonfunctioning somatostatinoma with lymph node metastases. The patient is alive, healthy and without tumor recurrence 10 months after surgery.
Duodenum ; Female ; Humans ; Incidence ; Lymph Nodes ; Middle Aged ; Neoplasm Metastasis ; Neuroendocrine Tumors ; Neurofibromatosis 1 ; Recurrence ; Somatostatinoma

Somatostatinoma is a rare endocrine tumor that comprises around 1% of all gastroenteropancreatic endocrine neoplasm. The estimated annual incidence is 1 in 40 million. This tumor may be associated with von Recklinghausen's disease. We present here a rare case of a 51-year-old female patient with a duodenal nonfunctioning somatostatinoma combined with von Recklinghausen's disease. Whipple' procedure was performed. The postoperative course was uneventful and the histopathologic findings were consistent with malignant nonfunctioning somatostatinoma with lymph node metastases. The patient is alive, healthy and without tumor recurrence 10 months after surgery.
Duodenum ; Female ; Humans ; Incidence ; Lymph Nodes ; Middle Aged ; Neoplasm Metastasis ; Neuroendocrine Tumors ; Neurofibromatosis 1 ; Recurrence ; Somatostatinoma

Adult ; Chromogranin A ; metabolism ; Female ; Humans ; Immunohistochemistry ; Pancreatic Neoplasms ; diagnosis ; metabolism ; therapy ; Phosphopyruvate Hydratase ; metabolism ; Somatostatin ; metabolism ; Somatostatinoma ; diagnosis ; metabolism ; therapy ; Synaptophysin ; metabolism ; Tomography, X-Ray Computed

Tumors of the minor papilla are very rare and these tumors have generally been known as neuroendocrine tumors such as carcinoid tumor and somatostatinoma. As these are mostly submucosal tumors, their diagnosis is difficult by just performing endoscopic forceps biopsy, but diagnosis is possible by surgery or endoscopic resection. EUS and ERCP is an essential tool for the diagnosis of these tumors, and abdominal CT or MRI is also useful because there is the possibility of malignant tumors such as carcinoid tumor. For our present two cases, screening endoscopy revealed the polypoid lesion of the minor papilla. EUS disclosed that the submucosal tumor was limited to the submucosal layer and no abnormality was found from the abdominal CT and ERCP. Herein, we performed endoscopic resection for making the diagnosis and treatment. Histologically, these 2 tumors were diagnosed as ectopic pancreas and gangliocytic paraganglioma, respectively.
Biopsy ; Carcinoid Tumor ; Cholangiopancreatography, Endoscopic Retrograde ; Diagnosis ; Endoscopy ; Magnetic Resonance Imaging ; Mass Screening ; Neuroendocrine Tumors ; Pancreas ; Paraganglioma ; Somatostatinoma ; Surgical Instruments ; Tomography, X-Ray Computed

Somatostatinoma is a rare neoplasm usually arising from the pancreas and duodenum which typically presents with indolent, nonspecific symptoms in the absence of systemic neuroendocrine manifestations that characterize somatostatinoma syndrome. It accounts for less than 1% of all gastrointestinal endocrine tumors with an annual incidence of 1 per 40 million. It is often associated with regional and/or portal metastasis at the time of diagnosis, and complete tumor resection is possible only in 60% to 70% of cases. We experienced a case of pancreatic somatostatinoma recently. A 51-year-old woman presented with right upper quadrant abdominal pain and loose stool for one month. A hypermetabolic lesion in the pancreatic head was detected on positron emission tomography-CT (PET-CT) scan. The tumor was resected by pylorus preserving pancreaticoduodenectomy. Immunohistochemical staining of the tumor tissue exhibited diffuse positivity for somatostatin, but was negative for insulin and glucagon. Herein, we report a case of pancreatic somatostatinoma diagnosed postoperatively.
Cholangiopancreatography, Endoscopic Retrograde ; Female ; Humans ; Middle Aged ; Pancreatic Neoplasms/*diagnosis/pathology ; Positron-Emission Tomography ; Somatostatinoma/*diagnosis/pathology ; Tomography, X-Ray Computed