Scientific Papers
Surgical Strategy for Insulinomas in Multiple Endocrine Neoplasia Type I

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Abstract

Background: Hyperinsulinemia in multiple endocrine neoplasia type I (MEN-I) is a rare but potentially curable condition that presents difficulties not encountered in sporadic cases.

Methods: The present report documents our surgical approach to 3 MEN-I patients with hyperinsulinemia.

Results: Primary hyperparathyroidism was manifested in all 3 patients at the time of presentation. Distal subtotal pancreatectomy with enucleation of tumor at the head of pancreas detected intraoperatively resulted in immediate cure of 2 patients. Persistent disease occurred in 1 patient after enucleation of tumor at the head of the pancreas guided by preoperative imaging elsewhere. The patient was subsequently cured by distal subtotal pancreatectomy. Pathology revealed multiple tumors (4 to 14) in all patients.

Conclusions: A different surgical strategy with an aim of distal subtotal pancreatectomy and enucleation of any tumor identified in the head of pancreas is the treatment of choice for hyperinsulinemia in MEN-I patients.

Section snippets

Patients and Methods

From 1975 to 1996, 35 patients with histologically confirmed functioning insulinoma were surgically treated in our institution. Three patients (8.6%) were diagnosed to have MEN-I because of the presence of concomitant endocrinopathy and/or positive family history. There were 2 men and 1 woman with ages of 23, 29, and 41 years, respectively. Their clinical presentation, preoperative localization studies, surgical treatment, pathology of resected specimen, and postoperative outcome are reported.

Results

All patients presented with neuroglycopenic symptoms from 1 to 3 months. One patient had a family history of primary hyperparathyroidism, and another patient had a history of pituitary tumor before presentation. Hypoglycemia with inappropriately elevated insulin level were documented during overnight fasting or a 72-hour supervised fasting hypoglycemic test. Hypercalcemia with elevated parathyroid hormone was found at the time of presentation, and the diagnosis of MEN-I was suspected in all

Comments

Whenever insulinomas are multiple, MEN-I should be suspected. Approximately 4% to 10% of patients with hyperinsulinism will have MEN-I,1, 2, 3and 10% to 15% of MEN-I patients will acquire insulinomas as the only functional component of the pancreatic disease.3, 4, 5In contrast to sporadic disease, in which about 90% of patients will have a solitary insulinoma, about 90% of patients with MEN-I have multiple insulinomas.[5]In a review of 60 patients reported in the English literature, 76% of

References (11)

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