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

Bibliographic Details
Title: 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)
Publisher Information: BMC, 2024.
Publication Year: 2024
Collection: LCC:Medicine
Subject Terms: Solitary fibrous tumor, Hypoglycemia, Insulin-like growth factor II, Doege-Potter syndrome, Paraneoplastic syndrome, Medicine
More Details: 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.
Document Type: article
File Description: electronic resource
Language: English
ISSN: 1752-1947
Relation: https://doaj.org/toc/1752-1947
DOI: 10.1186/s13256-024-04658-1
Access URL: https://doaj.org/article/9d216699352e4c90bfc368d0454462ec
Accession Number: edsdoj.9d216699352e4c90bfc368d0454462ec
Database: Directory of Open Access Journals
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More Details
ISSN:17521947
DOI:10.1186/s13256-024-04658-1
Published in:Journal of Medical Case Reports
Language:English