Somatostatinoma duodenal / Duodenal somastotinoma

Alrededor del 70%de los tumores endocrinos bien diferenciados asientan en el tracto gastrointestinal. Los tumores duodenales bien diferenciados constituyen solo el 2,6 % de todos los tumores neuroendocrinos (NET).El somatostatinoma es un tumor raro que se localiza en páncreas o duodeno con una incidencia de 1:40 millones.La neurofibromatosis tipo I es una enfermedad autosómica dominante, la mutación en el gen supresor de tumores NF1 favorece la aparición de neoplasias en estos pacientes.

About 70% of well-differentiated endocrine tumors arise from the gastrointestinal tract. Duodenal well-differentiated tumors account for only 2.6% of all neuroendocrine tumors. Somatostatinomas are rare neuroendocrine tumors (NETs) with an incidence of 1 in 40 million. These unusual tumors arise predominantly in the pancreas and peripancreatic duodenum. Neurofibromatosis type I is an autosomal dominant disease, the mutation in the tumor suppressor gene NF1 favors the appearance of neoplasms in these patients.

Combined presence of multiple gastrointestinal stromal tumors along with duodenal submucosal somatostatinoma in a patient with neurofibromatosis type 1.

Neurofibromatosis type‑1 (NF‑1) is an autosomal dominant disorder, with increased risk of developing benign and malignant tumors of the gastrointestinal tract (GIT). However, the synchronous presence of multiple GIT stromal tumors and duodenal submucosal somatostatinoma, like in this 50‑year‑old female NF‑1 patient, is very rare. She presented with hematemesis, malena, along with multiple neurofibromas all over the body. Thorough radiological and peroperative work‑up revealed multiple ulcerated submucosal and serosal nodules in the proximal small intestine. Histological work‑up revealed diagnosis of a duodenal submucosal somatostatinoma with multifocal serosal gastrointestinal stromal tumors. This case is being reported to highlight the rare coincidence of multiple GIT tumors in an NF‑1 patient.

Gastric Somatostatinoma: An Extremely Rare Cause of Upper Gastrointestinal Bleeding

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.

Duodenal Somatostatinoma: A Case Report and Review

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.

Non-Functioning Somatostatinoma of the Duodenum in Patient with Von Recklinghausen's Disease

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.

Non-Functioning Somatostatinoma of the Duodenum in Patient with Von Recklinghausen's Disease

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.

Somatostatinoma de duodeno: relato de caso e revisão da literatura / Duodenal somatostatinoma: case report and literature review

Introdução: Somatostatinomas são tumores carcinóides produtores de somatostatina, localizados principalmente na cabeça do pâncreas e duodeno. A importância desse relato deve-se à raridade deste subtipo histológico. Relato de Caso: Mulher, 68 anos, portadora de diabetes mellitus há dois anos, com queixa de dor abdominal há cinco meses. Ao exame físico notava-se massa em epigástrio e hipocôndrio direito, fixa e dolorosa à palpação. A ultra-sonografia e a tomografia computadorizada de abdome evidenciaram, respectivamente, colelitíase e tumor sólido em topografia de cabeça de pâncreas, medindo cerca de 12 x 8 cm. A paciente foi submetida a procedimento cirúrgico, no qual foi encontrado um grande tumor envolvendo o duodeno e o pâncreas, optando-se pela gastroduodenopancreatectomia cefálica e colecistectomia. Os exames anátomo-patológico e imuno-histoquímico da peça operatória revelararam tratar-se de um somatostatinoma de duodeno (estadiamento: T4N0Mx). A paciente apresentou boa evolução no pós-operatório, e com 18 meses da cirurgia encontra-se sem sinais de recidiva tumoral. Discussão: Os somatostatinomas são tumores raros, geralmente malignos, mais freqüentemente encontrados no pâncreas, sendo que apenas 2,5% deles são funcionantes. O diagnóstico é realizado por exame anátomo-patológico e imuno-histoquímico e o tratamento de escolha é a ressecção cirúrgica. Quando a cirurgia curativa não pode ser realizada, alguns pacientes podem se beneficiar de procedimentos paliativos e/ou quimioterapia. O seguimento deve ser realizado a cada seis meses com tomografia abdominal e a dosagem sérica de somatostatina pode ser um método complementar. A sobrevivência em cinco anos após a retirada completa do tumor e na ausência de linfonodos comprometidos ou metástases chega a 100%.

Introduction: Somatostatinomas are rare somatostatin-producing carcinoid tumors, mainly found in the duodenum and pancreatic head. The importance of this case report lies in the rarity of such histological subtype. Case Report: Sixty-eight years old female patient with a two-year diabetes history and five-month abdominal pain complaint. Physical examination revealed a tumor in the epigastrium and right hypochondrium, immovable and painful upon palpation. Ultrasound and abdominal computerized tomography showed, respectively, calculous chronic cholecystitis and solid tumour, measuring approximately 12 x 8 cm, located in pancreatic head topography. The patient was submitted to surgery in which a large tumor was found evolving duodenum and pancreas. Cephalic gastroduodenopancreatectomy and cholecistectomy were performed. The anatomopathological and immunohistochemical study of the surgical piece revealed a duodenal somatostatinoma (stage: T4N0Mx). The patient afforded good postoperative evaluation and after 18 months from surgery, presented no signs of tumor recurrence. Discussion: Somatostatinomas are rare, usually malignant tumors, more frequently found in the pancreas, and only 2.5% are functional. Diagnosis is established by anatomopathological and immunohistochemical exams and the chosen treatment is surgical resection. When curative surgery cannot be performed, some patients may benefit from palliative surgery and/or chemotherapy. Follow-up must take place every six months with abdominal tomography and serum somatostatin dosage could be a complementary method. The five-year survival rate is 100% upon complete tumor resection with the absence of affected lymph nodes or metastasis.

A Case of Pancreatic Somatostatinoma / 대한소화기학회지

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.

Somatostatinoma of the Ampulla of Vater

Somatostatinoma is a rare form of neuroendocrine tumor that was first described in 1977. Most tumors have involved the pancreas, and gastrointestinal tract involvement is rare. Somatostatinomas of the ampulla of Vater are extremely rare and present distinct clinical and pathologic differences. Pancreatic somatostatinoma has been associated with a clinical syndrome of dyspepsia, mild diabetes, cholelithiasis, steatorrhea, and hypochlorhydria, but duodenal somatostatinoma, in general, has been clinically silent. A further contrast is that duodenal carcinoid tumors, mainly gastrinoma, tended to be benign, whereas ampullary carcinoid tumors, mainly somatostatinoma, exhibited malignant behavior. Therefore, definite diagnosis is important for treatment and prognosis, and is performed by image study, immunohistochemistry and electron microscopic examination. We report a case of somatostatinoma of the ampulla of Vater in a 51-year-old male. He complained of generalized abdominal pain for a few days. Gastrofiberscopically, a 1.2 cm sized bulging mass was observed on the ampulla of Vater. Radiologically, on abdomen CT, a protruding enhancing mass was revealed in the duodenum. In octreoscan, there was an abnormal focus off increased radiouptake in the infrahepatic area. He underwent a pancreatoduodenectomy. Grossly, the mass was an intraluminary protruding polypoid submucosal mass with focal ulceration in the ampulla of Vater. Histologically, it showed well-differentiated nonpleomorphic tubular cell nest and psammoma bodies. Immunohistochemically, the tumor cells showed a neuroendocrine nature with synaptophysin immunostain and intense staining only for somatostatin.

A Case of Somatostatinoma which Manifested as Insulinoma when Metastasized to the Liver / 대한내분비학회지

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.

Somatostatin Secreting Islet Cell Adenoma: A case report

Somatostatin secreting islet cell adenoma(somatostatinoma) of the pancreas is a rare entity. Less than 30 cases of pancreatic somatostatinoma could be searched in the world literature. We present a case of somatostatinoma of the pancreas in a 64-year-old woman. This patient had suffered from diabetes, cholelithiasis and intermittent diarrhea. Laboratory examination revealed steatorrhea, blood somatostatin level of 30 pg/ml, and fasting blood glucose level of 116 mg/dl. Subtotal pancreatectomy was done after radiological demonstrations of a round tumor mass in the pancreas. It was a 2 cm-sized well demarcated yellowish brown round solid mass located in the proximal pancreas. A retention cyst was seen just distal to the tumor. Histologically, tumor masses consisted of polygonal cells with distinct cell border, having granular eosinophilic cytoplasm and small bland looking nuclei. The tumor cells were arranged in small solid islands and trabeculae, separated by fibrovascular stroma. Immunohistochemical stain for somatostatin was positive in the cytoplasm of the individual tumor cell, and ultrastructurally variable sized membrane bound electron dense granules of 200 nm in average diameter were found in tumor cells.

Duodenal Somatostatinoma: A case report

Somatostatinoma is rare endocrine tumor that was first described in 1977 by Ganda et al. and Larsson et al. simultaneously. It seems nonfunctioning at clinical level. But it may present with diabetes, diarrhea, cholelithiasis, steatorrhea, indigestion, hypochlorhydria, and anemia. In contrast with pancreatic somatostatinoma, duodenal somatostatinoma, in general, is clinically silent. Duodenal endocrine tumors show similar histologic pattern. Therefore, the definite diagnosis is performed by immunohistochemistry and electron microscopic examination. We have experienced a case of somatostatinoma of duodenum in a 62-year-old male. He has complained generalized pruritus for one year and jaundice for 2 weeks. Grossly, the mass was a intraluminary protruding, polypoid lesion with focal mucosal erosion at immediately distal to Ampulla of Vater. Histologically, it showed tall, cylindrical cells with distinct cell membranes, having granular cytoplasm and small innocent looking nuclei. No mitosis was seen. The tumor cells were arraged in small solid groups and trabeculae, separated by fibrovascular stroma. Immunohistochemically, the tumor cells were strongly positive with somatostatin and negative with several other hormonal and neuroendocrine markers. Ultrastructurally, the cytoplasm contains numerous, homogeneous low electron dense secretory granules, which are essentially similar to those seen in normal delta cells.