Abstract
Multiple endocrine neoplasia type 1 (MEN1) is a rare syndrome of autosomal dominant inheritance defined by co-occurrence of two or more tumors originating from the parathyroid gland, pancreatic islet cells, and/or anterior pituitary. Insulinoma which has an incidence of 0.4% is a rare pancreatic neuroendocrine tumor. Malignant insulinoma is extremely rare, while primary hyperparathyroidism is a common occurrence in MEN1. We present a case of MEN1 syndrome with 2.6 cm insulinoma in the pancreatic head and parathyroid adenoma in a 56-year-old female who presented with symptoms suggestive of hypoglycemia like multiple episodes of loss of consciousness for four years. Classical pancreaticoduodenectomy was carried out, and the postoperative period was uneventful. Later, subtotal parathyroidectomy was performed, which showed parathyroid adenoma. Patients presenting with features of hypoglycemia should be vigilantly assessed for the presence of a sinister pathology.
Highlights
Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized primarily by two or three principal MEN1-related endocrine tumors originating from parathyroid glands, endocrine gastroenteropancreatic (GEP) tract, and anterior pituitary [1]
Primary hyperparathyroidism is found in 90% of patients with MEN1 syndrome [1], whereas only 5–10% insulinomas present as a constituent of MEN1 [2]
Surgical resection is the mainstay of treatment of insulinoma [4] and parathyroid adenoma [1]
Summary
Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized primarily by two or three principal MEN1-related endocrine tumors originating from parathyroid glands, endocrine gastroenteropancreatic (GEP) tract, and anterior pituitary [1]. Primary hyperparathyroidism is found in 90% of patients with MEN1 syndrome [1], whereas only 5–10% insulinomas present as a constituent of MEN1 [2]. Most of the insulinomas are benign; very rarely, they are malignant [3]. Whipple’s triad along with supervised fasting serum insulin, C-peptide, and proinsulin thresholds/cutoffs along with imaging studies are vital in diagnosis and localization of mass [4]. Surgical resection is the mainstay of treatment of insulinoma [4] and parathyroid adenoma [1]
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