A 73-year-old male patient presented with upper gastrointestinal bleeding. The reason was an arterial hemorrhage from a duodenal tumor that could only be stopped by an angiographic intervention. In the further staging, there was evidence for a neuroendocrine carcinoma of the pancreatic head with infiltration of the duodenum and hepatic metastases. Due to good differentiation (G1) a systemic biotherapy with octreotide LAR was induced. After recurrent bleeding with arrosion of a branch of the superior mesenteric artery, a duodenum-preserving pancreatic head resection was performed. Afterwards, the systemic therapy was changed to a palliative chemotherapy with streptozotocin and 5-fluorouracil due to local progression of the disease and a Ki-67 index of 4% in the primary tumor.
In about 0.7% of patients with neuroendocrine tumors, the lesion is located in the pancreas. At this site these entities are very heterogeneous. The majority are nonfunctional tumors without secretion of bioactive substances and the associated symptoms. About 60% of the patients present with advanced metastasized disease. The therapy depends on the local spread and histological grading as well as symptoms of the patient. The only curative option represents surgical resection. However, even in a palliative situation, there can be benefit for the patient in case of a tumor mass reduction of > 90%. Alternative therapies in the palliative situation are somatostatin analogs, a classic systemic chemotherapy, or locoregional interventional procedures.
[Fulminant duodenal bleeding as first manifestation of a neuroendocrine carcinoma of the pancreatic head]