Multiple Endocrine Neoplasia type 1 is an autonomic dominant disease with a high degree of penetrance. It is characterized by combinations of over 20 different endocrine and nonendocrine
tumors. A 25-year-old woman was referred for 1 year-evolution
amenorrhea and bilateral
galactorrhea. She also had fasting hypoglycaemia and
hypercalcemia, and she was diagnosed of
Multiple Endocrine Neoplasia type 1A. Resection of three parathyroid glands was performed showing
hyperplasia of principal cells. Post-
parathyroidectomy serum levels of
calcium and intact PTH were normal but 3 years later serum
calcium levels rose again. A
99mTc-sestamibi scan showed increased uptake in the low right area compatible with
adenoma. After biochemical test showing probable
insulinoma,
somatostatin receptor scintigraphy showed a focal captation in head and body of pancreas. MRI found two nodules in the same localization. An
antral gastrectomy, total
pancreatoduodenectomy, colecistectomy and
truncal vagotomy was performed and histopathologic examination revealed a combination of
neuroendocrine tumors:
gastrinomas, somastotinomas,
glucagonomas and
insulinomas. After surgery she started with tingling in fingers, toes and lips, and
calcium levels was 5.9 mg/dl and PTH intact 3 pg/ml. A new 99m Tc-sestamibi scan showed no captation and cervical ultrasonography was normal. Now, 2 years later, she continues with normal
calcium and i-PTH levels. This report represents an unusual case of MEN 1A with association of
insulinomas,
gastrinomas glucagonomas and
somatostatinomas in the same patient.