The syndrome of refractory watery diarrhea and hypokalemia in patients with a non-insulin—secreting islet cell tumor: A further case study and review of the literature

Abstract

Another case is reported of the syndrome of severe refractory diarrhea and hypokalemia, followed by peptic ulceration, in a patient with non-insulin-secreting islet cell adenomas and islet cell hyperplasia. Operative removal of part of the pancreas failed to arrest the accompanying excessive secretion of gastric juice, and the patient died. The reported cases of this syndrome are reviewed. It is pointed out that patients with non-insulin-secreting islet cell adenomas may present in three ways: (1) with a long history of diarrhea but without evidence of peptic ulceration; (2) with a long history of diarrhea, with later onset of symptoms and signs of peptic ulceration; and (3) with recurrent peptic ulceration but without any history of antecedent diarrhea. It is emphasized that there are multiple adenomas in the pancreas in about two-thirds of the reported cases; a diffuse hyperplasia and hypertrophy of the islets of Langerhans may well be the main lesion in all these cases. Since these islet cell adenomas apparently secrete a humoral substance which stimulates gastric secretion and accounts for the severe diarrhea and other systemic effects, surgical removal may be expected to alleviate the signs and symptoms, as happened in two recently reported cases.