Duodenal somatostatinoma: a case report and review of the literature

Abstract

IntroductionAbout 70% of well-differentiated endocrine tumors arise from the gastrointestinal tract. Duodenal well-differentiated endocrine tumors account for only 2.6% of all neuroendocrine tumors. Following the first two case reports of somatostatin-secreting tumors in 1977, fewer than 200 cases of somatostatinoma have been reported. These tumors of the duodenum are usually silent and asymptomatic, but can cause gastrointestinal symptoms. Depending on the localization of the tumor, multiple surgical procedures can be performed, ranging from local resection to pancreaticoduodenectomy.Case presentationHere, we report a case of a submucosal duodenal mass in a 42-year-old Turkish White man presenting with nausea, vomiting, fatigue and abdominal pain. The treatment decision of pancreaticoduodenectomy made preoperatively was later altered to intraoperative removal via local resection with sphincteroplasty.ConclusionTumors of the periampullary region are considered highly malignant, and the Whipple operation is usually the only procedural treatment. In the current case, we decided not to perform pancreaticoduodenectomy but to excise the mass intraoperatively, and consequently avoided unnecessary resection of the pancreas and anastomosis to undilated hepatic and pancreatic ducts. This protective strategy prevented duodenum- and pancreas-related morbidity.

Duodenal somatostatinoma presenting as obstructive jaundice with the coexistence of a gastrointestinal stromal tumour in neurofibromatosis type 1: a case with review of the literature

“…Whipple’s operation with regional lymphadenectomy should be considered for more aggressive or larger tumours. Radiation and chemotherapy are also treatment options, but most metastatic NETs are unresponsive to chemotherapy 33–37. For tumours <1 cm, there is no consensus regarding management, although endoscopic excision is considered a reasonable approach because of the low incidence of metastasis and lymph node involvement.…”

Somatostatinoma of the Ampulla: An Incidental Postoperative Finding Following Colorectal Cancer Resection

“…Due to the rarity of the disease, long-term studies demonstrating efficacy of these treatment modalities is lacking. 3, 11 …”

Somatostatinoma: Beyond neurofibromatosis type 1 (Review)

“…FLORICA SANDRU 1,2 , MARA CARSOTE 3,4 , ANA VALEA 5,6 , SIMONA ELENA ALBU 7,8 , RĂZVAN-COSMIN PETCA 9 and MIHAI CRISTIAN DUMITRASCU 7,8 duodenum source; overall with an incidence of 1/40 million persons (1). Clinical presentation includes diabetes mellitus, cholelithiasis, steatorrhea, abdominal pain, vague symptoms, and some describe the specific triad as: Glucose metabolism anomalies, steatorrhea and aclorhydria (2,3).…”

“…However, the neoplasia may develop completely asymptomatic or it may be detected as an incidental finding during an imaging technique of an endocrine/non-endocrine organ or during a surgical procedure for abdominal pain of unknown cause or other non-related synchronous conditions (2,6,7). The tumour may be sporadic or associated to different genetic backgrounds as neurofibromatosis type 1 for duodenal localisation (8). Blood somatostatin assays as well as others less specific neuroendocrine markers such as chromogranin A or neuron specific enolase are useful for initial diagnosis but also as prognostic parameters during follow-up (9).…”