• Saudi Med J · Mar 2021

    Case Reports

    Insulinoma in tuberous sclerosis: An entity not to be missed.

    • Mohammed S Al Qahtani, Shoukat A Bojal, Abdullah A Alqarzaie, and Abdulaziz A Alqahtani.
    • From the Department of Surgery (Al Qahtani M), Multi Organ Transplant Center; from the Department of Surgery (Bojal), King Fahad Specialist Hospital; and from the College of Medicine (Alqarzaie, Alqahtani A) Imam Abdulrahman Bin Faisal University, Dammam, Kingdom of Saudi Arabia.
    • Saudi Med J. 2021 Mar 1; 42 (3): 332-337.

    AbstractPancreatic neuroendocrine tumors are rare with an incident rate of 5 cases per million individuals. Tuberous sclerosis complex is an autosomal dominant disease. This disease involves multisystem and occurs in one out of every 6,000-10,000 individuals. In this study, we describe a 47-year-old male known tuberous sclerosis patient with an insulinoma. The tumor was incidentally detected in follow-up imaging for a previous ampulla of Vater tubular adenoma. However, the patient reported symptoms of hypoglycemia. The insulinoma was enucleated successfully. Histopathology revealed a well-differentiated, grade one neuroendocrine tumor measuring around 2 cm in diameter. Seven cases were reported in the literature of tuberous sclerosis-associated insulinoma. The 7 reported cases had different hypoglycemia related symptoms. The reported tumors varied in size and location on the pancreas. This paper details the eighth case worldwide where an insulinoma occurred in a tuberous sclerosis patient.Copyright: © Saudi Medical Journal.

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