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

BACKGROUND: Neuroendocrine tumors of the pancreas are classified according to the endocrine function as insulinomas gastrinomas somatostatinomas, or nonfunctioning tumors. However, the morphologic features are not different from each other. Therefore, we tried to compare correlations among the morphologic features, endocrine function, and the immunohistochemical reaction with specific monoclonal antibodies to the tumors. METHOD: We reviewed the medical records of seven patients with pancreatic neuroendocrine tumors retrospectively, and analysed the clinical manifestations, the methods of diagnosis, the pathological characteristics and the results of surgery. Additionally, we compared the correlation between the clinical manifestations and the expression of immunohistochemical staining by using six different kinds of monoclonal antibodies to each tumor. RESULTS: The seven pancreatic neuroendocrine tumor patients were treated by surgical excision. Four patients had benign insulinomas, two had nonfunctioning malignant tumor and one patient had a benign nonfunctioning tumor associated with stomach cancer. The pattern of immunohistochemical stain of each tumor was not correlate with the clinical manifestations. CONCLUSION: The morphologic study with H & E stain, even with immunohistochemical staining of pancreatic neuroendocrine tumor, cannot support differentiation of the functional diagnoses, such as insulinoma, gastrinoma, somatostatinoma, nonfunctional tumors and so forth.
Antibodies, Monoclonal ; Diagnosis ; Gastrinoma ; Humans ; Insulinoma ; Medical Records ; Neuroendocrine Tumors* ; Pancreas ; Retrospective Studies ; Somatostatinoma ; Stomach Neoplasms

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

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

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 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

BACKGROUND: Endocrine pancreas tumor is a rare disease which incidence is less than 2% of all pancreatic tumors. But it comprises various types of tumor and usually secretes several hormones from one type of tumor although the patient with this tumor complains of sole symptom associated with only one hormone. The mechanism and clinical significance of multiple hormone secretion in the endocrine pancreas tumom are not yet clearly defined. METHODS: We analyzed retrospectively the clinicopathologic features of 20 cases which were operated at Seoul National University Hospital during the period between February 1989 and May 1998. RESULTS: The most common tumor was insulinoma (13 cases) and the second most common tumor was nonfunctioning tumor (6 cases). There was one case of somatostatinoma. Most of the patients with insulinoma complained of neuroglycopenic symptoms. There were 9 cases (45.0%) in which the tumors secreted more than two kinds of hormones, 7 cases in insulinoma, 2 cases in nonfunctioning tumors. Whether the tumor secreted multiple hormones was detected by the method of immunohistochemical staining. Though the tumors secreted more than two kinds of hormones, the patients with the tumors complained of symptoms which were associated with the cell type most strongly stained by immunohistochemical method. Whether or not the tumors secreted multiple hormones was not associated with the pathologic features such as tumor size, histologic patterns of the tumor, status of tumor cell differentiation and malignancy. CONCLUSION: From this results, we suggest that endocrine tumors of the pancreas secreted multiple hormones not by the mechanism of dedifferentiation from already differentiated endocrine cells but by the mechanism of neogenesis of multipotent islet stem cells. Since the relationship between the function of multiple hormone secretion in the endocrine pancreas tumors and islet stem cell would be significant, further study should be needed to find out the function of stem cells and application of stem cells to clinical use.
Cell Differentiation ; Endocrine Cells ; Humans ; Incidence ; Insulinoma ; Islets of Langerhans ; Pancreas* ; Rare Diseases ; Retrospective Studies ; Seoul ; Somatostatinoma ; Stem Cells

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

We report a case of somatostatinoma, which manifested as insulinoma after liver metastasis. A 74-year-old man suffered from diabetes mellitus and jaundice. The abdominal CT scan of this patient showed a mass in the pancreas head, which obstructed biliary duct. He underwent Whipples procedure. Immunohistochemical staining of postoperative specimen disclosed that this tumor was strongly positive for somatostatin. After 4 months, follow up CT scan showed multiple metastatic lesions in the liver. We performed transarterial chemoinfusion two times, but the response was disappointing. After 2 months, he suffered from altered mentality, which was relieved by intake of sugar. Biochemical laboratory findings and immunohistochemical staining of liver biopsy disclosed that the metastatic lesion in this patient was insulinoma. We performed embolization of hepatic artery with gelform. The biochemical response was dramatic, but he died of septic shock, which was caused by gas forming liver abscess. In summary, we report a case of somatostatinoma which manifested as insulinoma after metastasis to the liver.
Aged ; Biopsy ; Diabetes Mellitus ; Follow-Up Studies ; Head ; Hepatic Artery ; Humans ; Insulinoma* ; Jaundice ; Liver Abscess ; Liver* ; Neoplasm Metastasis ; Pancreas ; Shock, Septic ; Somatostatin ; Somatostatinoma* ; Tomography, X-Ray Computed