Title:Doege-Potter Syndrome; A Case of Solitary Fibrous Pleura Tumor Associated
with Severe Hypoglycemia: A Case Report in Internal Medicine
Volume: 23
Issue: 12
Author(s): Viviana Castaldo, Daniela Domenici*, Mauro Valentino Biscosi, Paolo Ubiali, Cesare Miranda, Giorgio Zanette, Cinzia Mazzon and Maurizio Tonizzo
Affiliation:
- Department of Endocrinology and
Metabolic Diseases, Hospital Santa Maria degli Angeli, Pordenone, Italy
Keywords:
Hypoglycemia, doege-potter syndrome, non-islet cell tumor hypoglycemia, solitary fibrous tumor, IGF-2, STAT6.
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.
