title: Surgical management of hyperinsulinism in the multiple endocrine neoplasia, type 1 syndrome.
abstract: We retrospectively reviewed the records of 12 patients from one institution (from 1970 through 1983) who had hyperinsulinism as a part of the multiple endocrine neoplasia, type 1 syndrome. All of the patients underwent surgical exploration: ten had subtotal pancreatectomies, one had enucleation, and one underwent a total pancreatectomy. There was no operative mortality. Preoperative and intraoperative localization studies (angiography and ultrasonography) tended to underestimate the number of pancreatic tumors. Ten patients had multiple B-cell adenomas (mean, 7.4), while two patients had single tumors. This study confirms that hyperinsulinism, when it occurs as part of the multiple endocrine neoplasia, type 1 syndrome, is usually due to multiple islet cell tumors, and that the islet cell tissue is probably diffusely dysplastic. Subtotal (85%) pancreatectomy is suggested as the procedure of choice for this subgroup of patients with hyperinsulinism.
authors.forename: D A
authors.initials: DA
authors.lastname: Rasbach
authors.forename: J A
authors.initials: JA
authors.lastname: van Heerden
authors.forename: R L
authors.initials: RL
authors.lastname: Telander
authors.forename: C S
authors.initials: CS
authors.lastname: Grant
authors.forename: J A
authors.initials: JA
authors.lastname: Carney
mesh_terms: D000236:Adenoma / Q000150:complications / Q000601:surgery; D007516:Adenoma, Islet Cell / Q000150:complications / Q000601:surgery; D000293:Adolescent; D000328:Adult; D002277:Carcinoma / Q000150:complications / Q000601:surgery; D002648:Child; D005260:Female; D006801:Humans; D006946:Hyperinsulinism / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D009377:Multiple Endocrine Neoplasia / Q000150:complications / Q000601:surgery*; D010190:Pancreatic Neoplasms / Q000150:complications / Q000601:surgery*; D011183:Postoperative Complications; D012189:Retrospective Studies