Doege-Potter Syndrome; A Case of Solitary Fibrous Pleura Tumor Associated with Severe Hypoglycemia: A Case Report in Internal Medicine

Endocr Metab Immune Disord Drug Targets. 2023;23(12):1562-1569. doi: 10.2174/1871530323666230623112047.

Abstract

Background: Doege-Potter syndrome is a rare paraneoplastic entity that is often diagnosed incidentally during the work-up of hypoglycemia of unclear etiology. It is characterized by a non-islet cell tumor hypoglycemia mostly associated with solitary fibrous tumors. These uncommon tumors have been reported in <5% of solitary fibrous tumors. Although not unique in its kind, this case is extremely important as this syndrome often conceals unrecognized tumors that can be surgically resolved.

Case presentation: We present the case of a 59-year-old non-diabetic man with a 2-month history of severe and recurrent fasting hypoglycaemia presenting with severe dyspnea and sweating. Further workup revealed low insulin, C-peptide, and IGF-1 levels and a large right in-trathoracic solitary fibrous tumor. Unfortunately, bioassays for IGF-2 were unavailable at our hos-pital. Nevertheless, as hypoglycemia completely resolved after resection of the mass, Doege-Potter syndrome was highly suspected.

Conclusion: Doege-Potter syndrome is a complication of rare tumors. If hy-poglycemia is unexplained, this syndrome should always be suspected, and the presence of un-known masses should be investigated.

Keywords: Hypoglycemia; IGF-2; STAT6; doege-potter syndrome; non-islet cell tumor hypoglycemia; solitary fibrous tumor.

Publication types

  • Case Reports

MeSH terms

  • Humans
  • Hypoglycemia* / diagnosis
  • Hypoglycemia* / etiology
  • Male
  • Middle Aged
  • Pleura / pathology
  • Solitary Fibrous Tumor, Pleural* / diagnosis
  • Solitary Fibrous Tumor, Pleural* / diagnostic imaging
  • Syndrome

Supplementary concepts

  • Hereditary renal agenesis
  • Renal Adysplasia