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Doege-Potter syndrome due to a solitary fibrous tumor of the pleura: a case report

Authors :
Juan Estrada-Maya
Juan Sebastián Montejo
Katerin Dayana Báez López
Juan Carlos Garzón
Source :
Journal of Medical Case Reports, Vol 18, Iss 1, Pp 1-7 (2024)
Publication Year :
2024
Publisher :
BMC, 2024.

Abstract

Abstract Background Doege-Potter syndrome is a rare paraneoplastic phenomenon associated with solitary fibrous tumors of the pleura (SFTPs). It is characterized by the presence of severe, sustained, and treatment-refractory hypoglycemia. Hypoglycaemia, which may be the sole symptom at disease onset, is mediated by the secretion of high-molecular-weight insulin-like growth factor (IGF-2). Most tumors exhibit benign behavior, with a 100% survival rate at 5 years. However, 10% of these tumors may display aggressive behavior with local or metastatic recurrence. We present a clinical case of a patient with a benign solitary fibrous tumor of the pleura who presented with symptomatic hypoglycemia and required pulmonary and pleural surgical resection to control the paraneoplastic phenomenon. Case presentation A Hispanic 46-year-old man presented with a 15-day history of transient alterations in consciousness worsened by fasting. The relevant medical history included obstructive sleep apnea treated with continuous positive air pressure (CPAP) and previous smoking. In-hospital studies revealed noninsulinemic hypoglycemia and a benign SFTP. Complete surgical resection was performed while the patient received dextrose fluids and corticosteroids perioperatively for hypoglycemia. Subsequently, the hypoglycemia resolved, and the patient was followed-up without disease recurrence. Conclusion Doege-Potter syndrome is challenging to recognize. However, effective treatment can be achieved with a high survival rate. Raising awareness among healthcare professionals about the recognition of this paraneoplasic syndrome patients will improve diagnostic suspicion, biochemical confirmation, the development of diagnostic and therapeutic guidelines, and the creation of predictive indices for aggressive presentations requiring closer monitoring.

Details

Language :
English
ISSN :
17521947
Volume :
18
Issue :
1
Database :
Directory of Open Access Journals
Journal :
Journal of Medical Case Reports
Publication Type :
Academic Journal
Accession number :
edsdoj.9d216699352e4c90bfc368d0454462ec
Document Type :
article
Full Text :
https://doi.org/10.1186/s13256-024-04658-1