Solitary fibrous tumor of the pleura as a rare cause of severe hypoglycemia: Doege-Potter syndrome
Authors:
P. Pavlíková 1; L. Sochorová 1; O. Snížková 2; V. Malý 1; I. Staněk 1
Authors place of work:
Oddělení hrudní chirurgie, Centrum vysoce specializované pneumoonkochirurgické péče, Masarykova nemocnice v Ústí nad Labem
1; Patologické oddělení, Masarykova nemocnice v Ústí nad Labem
2
Published in the journal:
Rozhl. Chir., 2020, roč. 99, č. 2, s. 95-98.
Category:
Case Report
doi:
https://doi.org/10.33699/PIS.2020.99.2.95–98
Summary
Introduction: Doege-Potter syndrome is a rare syndrome characterized by hypo-insulinemic hypoglycemia. It is caused by excessive ectopic secretion of insulin-like growth factor II from a solitary fibrous tumors of intrapleural or extrapleural origin. Laboratory tests reveal low levels of C-peptide and insulin, on the contrary insulin-like growth factor II level is elevated, which is characteristic for Doege-Potter syndrome. Majority of solitary fibrous tumors present no symptomatology, recurrent hypoglycemia is relatively rare, but it may be the only clinical manifestation. The therapy is surgical, consisting of radical en-bloc tumor resection.
Case report: Authors present a case report of a patient with recurrent hypoglycemia caused solely by solitary fibrous tumor. Hypoglycemia resolved immediately after surgical resection and there were no recurrences.
Conclusion: Doege-Potter syndrome should be considered as the differential diagnosis in a patient with suspicion on thoracic malignancy if accompanied by features suggestive of hypoglycemia. Prolonged follow up is strongly advised because of the risk of disease recurrence, even in patients with benign solitary fibrous tumors of the pleura (SFTP).
Keywords:
solitary fibrous tumor – pleural tumor – hypoglycemia – DoegePotter syndrome
Zdroje
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