Doege-Potter syndrome in a patient with a giant abdominal solitary fibrous tumor: a case report and review of the literature

Acta Clin Belg. 2023 Aug;78(4):358-364. doi: 10.1080/17843286.2023.2165652. Epub 2023 Jan 15.

Abstract

A 63-year-old man with spells of reduced consciousness in the morning and a giant abdominal mass presented to our institution for a second opinion. Investigation revealed non-diabetic hypoinsulinemic hypoglycemic events. Removal of the abdominal mass solved the hypoglycemia. Anatomopathological examination confirmed a solitary fibrous tumor (SFT). Doege-Potter syndrome was diagnosed. Doege-Potter syndrome is a potentially life-threatening rare paraneoplastic syndrome characterized by recurrent hypoinsulinemic hypoglycemia due to the overproduction of a prohormone form of insulin-like growth factor-II (pro-IGF-II) from a solitary fibrous tumor. First, we describe the clinical, laboratory and radiologic findings of the case. Second, a brief literature review on Doege-Potter syndrome is provided.

Keywords: Doege-Potter syndrome; Hypoglycemia; IGF-II; non-islet cell hypoglycemia (NITCH); solitary fibrous tumor (SFT).

Publication types

  • Review
  • Case Reports

MeSH terms

  • Humans
  • Hypoglycemia* / diagnosis
  • Hypoglycemia* / etiology
  • Kidney
  • Male
  • Middle Aged
  • Solitary Fibrous Tumors* / complications
  • Solitary Fibrous Tumors* / surgery

Supplementary concepts

  • Hereditary renal agenesis
  • Renal Adysplasia