Surgical management of hyperinsulinism in the multiple endocrine neoplasia, type 1 syndrome
D. A. Rasbach, J. A. van Heerden, R. L. Telander, C. S. Grant and J. A. Carney
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.
