Answer: Somatostatinoma

Histology: The lesion was resected revealing a well-differentiated neoplasm that formed nests, cords and some lumina. The nuclei are round and uniform, and have a “salt and pepper” chromatin pattern. Psammoma bodies are present.

Discussion: Patients with von Recklinghausen’s neurofibromatosis have an increased risk of developing a wide variety of neoplsms, including somatostatinomas of the duodenum/ampulla of Vater. Well-differentiated endocrine neoplasms of the duodenum may produce a variety of hormones, but those that are psammomatous tend to be somatostatinomas. The diagnosis of a pure somatostatinoma in the duodenum should therefore alert one to the possibility of coexistent von Recklinghausen’s neurofibromatosis (see reference).

Reference(s):
– Y. Dayal, et al., Duodenal carcinoids in patients with and without neurofibromatosis. A comparative study. Am J Surg Pathol. 1986 May;10(5):348-57.