Multicentric insulinoma associated with multiple endocrine neoplasia type 1: One case report

Background: Pancreatic neuroendocrine tumors may be associated with multiple endocrine neoplasia type 1 (MEN1) in 15-25% of cases, and up to 10% develop insulinomas, which are the most frequent cause of persistent hyperinsulinemic hypoglycemia in adults. The objective is to show a clinical case that presents this association. Clinical case: 36-year-old patient with seizures associated with fasting hypoglycemia of 36 mg/dL. The patient presented high levels of insulin, peptide C, proinsulin, hypercalcemia, hypophosphatemia and parathyhroid hormone. Ultrasound sonography test showed hypoechoic lesion in the pancreatic tail of 2.3 cm. Tomography showed a 10 mm hypodense lesion at pancreatic head. Scintigram showed hypercaptating parathyroid tissue. Magnetic resonance did not show evidence of pituitary adenoma. We carried out a tumor enucleation of the pancreatic head and distal partial pancreatectomy with histopathological diagnosis of well-differentiated low-grade multifocal neuroendocrine tumor (five tumors); two tumors were immunoreactive to insulin. After surgery, glucose level was normalized (105 mg/dL). Patient underwent parathyroidectomy with a report of adenoma of the parathyroid gland.

Conclusion: The multicentricity of pancreatic neuroendocrine tumors related to parathyroid neoplasia suggests the diagnosis of MEN1. Genetic molecular studies should be performed to confirm this and provide the patient with a correct diagnosis, targeted treatment and adequate genetic counseling.