Insulinomatosis: A case report of a multifocal insulin-secreting entity

Insulinomatosis is an exceedingly rare cause of endogenous hyperinsulinemic hypoglycemia, characterized by the presence of multiple insulin-producing neoplasms within the pancreas associated with clinical symptoms of hypoglycemia. We report a case of insulinomatosis in a woman in her sixties with a lifelong history of hypoglycemia and a family history of relatives with symptoms concerning for hypoglycemia. Laboratory evaluation was consistent with hyperinsulinemic hypoglycemia, and further evaluation with various imaging modalities supported the diagnosis of a multifocal neuroendocrine tumor in the pancreas. A complete pancreatectomy was performed, and pathological analysis confirmed a diagnosis of insulinomatosis. This case highlights the need for correlation between biochemical, imaging, histopathologic, and genetic evaluation in diagnosing insulinomatosis. Furthermore, this patient’s case underscores the gaps in understanding the familial and genetic basis of insulinomatosis, which may help inform decision-making in the management of this exceptionally rare disease.