الملخص
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.
الموضوعات
Male , Humans , Middle Aged , Neuroendocrine Tumors/diagnosis , Neuroendocrine Tumors/surgery , Somatostatinoma/diagnosis , Somatostatinoma/surgery , Neurofibromatosis 1الملخص
Pancreatic neuroendocrine tumors are infrequent and slow-growing neoplasms. They are classified basedon their clinical presentation as functioning and nonfunctioning tumors. The most common functionaltumors are the insulinoma and gastrinoma. They can be sporadic or be part of hereditary forms as MEN-1.The diagnosis is based on the detection of the specific clinical syndrome in association of high levels of the substance secreted by the tumor and conventional imaging studies or others such as stimulation tests, somatostatin receptor scintigraphy and endoscopic ultrasound. In general, these tumors have a better prognosis than the pancreatic adenocarcinoma and they can show metastasis to the liver and infrequently, in bones. The treatment can be managed medically diminishing the inappropriate secretion of the substances by the tumor using, for example, the somatostatin analogs. Surgery should be always considered, especially in case of insulinomas, small non-functioning tumors, and small gastrinomas that can be managed with surgery enucleation. More advanced resective surgery, such as Whipple resection, are not routinely recommended and they should be limited to selected patients. In advanced tumors, there are other treatment alternatives, for example, hepatic resection, radiofrequency, chemotherapy and new agents such as sunitinib and everolimus.
Los tumores neuroendocrinos pancreáticos son infrecuentes y de crecimiento lento. Se clasifican en tumores funcionantes o no funcionantes (TNEP-NF), de acuerdo a la presentación clínica. Los tumores funcionantes más frecuentes son los insulinomas y los gastrinomas. Pueden ocurrir en forma esporádica o asociados a síndromes hereditarios como la NEM- 1, entre otros. El diagnóstico se basa en la detección del síndrome clínico específico asociado a la demostración de niveles elevados de la sustancia secretada y exámenes imagenológicos convencionales u otros más específicos como de estimulación, cintigrafía de receptores de somatostatina y endosonografía. En general, tienen mejor pronóstico que los adenocarcinomas pancreáticos y pueden dar metástasis hepáticas y con menor frecuencia, óseas. El tratamiento puede ser médico disminuyendo la secreción inapropiada de las sustancias producidas por el tumor como los análogos de somatostatina. La cirugía siempre debe ser considerada, especialmente en caso de insulinomas, pequeños TNEP-NF, y gastrinomas pequeños, que pueden ser tratados con enucleación quirúrgica. Las cirugías resectivas más avanzadas, como la operación de Whipple no están recomendadas en forma rutinaria y sólo deben ser utilizadas en pacientes seleccionados. En casos de tumores avanzados, existen alternativas de tratamiento, como la resección hepática, radiofrecuencia, quimioterapia, y terapia con nuevos agentes en estudio como el sunitinib y everolimus.
الموضوعات
Humans , Pancreatic Neoplasms/diagnosis , Pancreatic Neoplasms/therapy , Neuroendocrine Tumors/diagnosis , Neuroendocrine Tumors/therapy , Gastrinoma/diagnosis , Gastrinoma/therapy , Glucagonoma/diagnosis , Glucagonoma/therapy , Insulinoma/diagnosis , Insulinoma/therapy , Somatostatinoma/diagnosis , Somatostatinoma/therapy , Vipomaالملخص
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.
الموضوعات
Humans , Male , Middle Aged , Cholangiopancreatography, Endoscopic Retrograde , Duodenal Neoplasms/diagnosis , Somatostatinoma/diagnosisالملخص
Descrito por primera vez por Frantz en 19591,2 lostumores sólidos pseudopapilares (TSS) son neoplasiasde origen epitelial, de etiología incierta que afectangeneralmente a mujeres jóvenes.2 Dentro la sinonimiapara referir dicha lesión se los conoce también comoneoplasia papilar epitelial, tumor acinar sólido y quístico,neoplasia papilar quística, carcinoma papilar quístico, tumor papilar de bajo grado y finalmente tumor de Frantzpor su primera descripción. Si bien es cierto que tienenun potencial maligno, este es solo del 1 a 2 3 y no fuesino hasta 1996 cuando la OMS4 lo reconoce con suactual nombre, tumor sólido pseudopapilar.Debido a que la neoplasia es muy rara, el objetivode este reporte de casos es presentar el caso clínicoy discutir sobre algunos hechos de su diagnóstico y manejo quirúrgico.
الموضوعات
Humans , Female , Adult , Pancreatic Neoplasms/therapy , Somatostatinoma/diagnosis , Adenolymphoma/surgery , Adenolymphoma/etiology , Cholangiography/methodsالموضوعات
Humans , Female , Middle Aged , Pancreas/abnormalities , Pancreatic Ducts/pathology , Pancreatic Neoplasms/diagnosis , Somatostatinoma/diagnosis , Fatal Outcome , Pancreatic Ducts/surgery , Pancreatic Neoplasms/pathology , Pancreatic Neoplasms/surgery , Somatostatinoma/pathology , Somatostatinoma/surgeryالملخص
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.