Somatostatinoma

Somatostatinomas are rare, indolent pancreatoduodenal neuroendocrine tumours (NETs) with an incidence of 1 in 40 million. The majority are non-functioning and are detected either incidentally or by local mass effect symptoms, but active tumours can produce a spectrum of symptoms called ‘somatostatinoma syndrome’. Specific preoperative diagnosis for a somatostatinomas is difficult and may only reveal a NET; immunohistochemical staining for somatostatin is diagnostic and may confirm diagnosis postoperatively. Operative resection provides the mainstay of treatment, even in the presence of metastasis, with somatostatin analogues and chemotherapy considered as second-line treatments.