Doege-Potter syndrome: a rare presentation of a solitary fibrous tumor of the pleura

Abstract

Solitary fibrous tumors of the pleura usually have a benign behavior, although some may present malignant characteristics -recurrences, metastases, and paraneoplastic syndromes. When a solitary fibrous tumor is responsible for the development of hypoglycemia, we have a case of Doege-Potter syndrome. We describe a case of a male patient that presented recurring and severe hypoglycemia, being also diagnosed with an extensive intrathoracic mass, which was proven to be a solitary fibrous tumor of the pleura. The treatment, through the maintenance of proper serum levels of glucose (administration of solutions with glucose and steroids), followed by the surgical resection of the tumor, promoted the complete resolution of the hypoglycemia episodes. The early recognition of this condition and the proper treatment is essential for a good clinical outcome. It is important to underscore that the multidisciplinary evaluation (oncologists, radiologists, surgeons, and pathologists) of rare tumors is recommended to achieve better therapeutic results.

Doege–Potter syndrome due to endothoracic solitary hypoglycemic fibrous tumor

“…Given this unusual performance of these markers, The Signal transducer and activator of transcription 6 (STAT6) has turned out to be more specific, being positive in >95% of the cases, recently reported as part of the pool of tests to address these cases. 8 This clinical case reported Doege-Potter syndrome secondary to the SFT tumor. However, the main paraneoplastic symptom of SFT is osteoarthropathy, hypoglycemia is less than 5%, which is a rare complication.…”

“…Given this unusual performance of these markers, The Signal transducer and activator of transcription 6 (STAT6) has turned out to be more specific, being positive in >95% of the cases, recently reported as part of the pool of tests to address these cases. 8 …”

“…The most frequent age of SFT is older adults. Several studies have reported SFT in patients aged 67 and 83 years, 6 , 9 , 14 although there are case series in patients aged 48 years, 15 52 years, 8 55 years, 7 and 58 years. 3 In this study, an elderly patient (74‐year‐old) with Doege–Potter syndrome secondary to benign SFT was reported for the first time in Peru, which is different from previous reports.…”

“…In this study, we reported negative for actin, S100, and EMA, which is different from previous reports that showed positive for smooth muscle actin, EMA, S100, and keratin. Given this unusual performance of these markers, The Signal transducer and activator of transcription 6 (STAT6) has turned out to be more specific, being positive in >95% of the cases, recently reported as part of the pool of tests to address these cases 8 …”

“…Finally, there is a growing interest in SFT and Doege–Potter syndrome. In Latin America, several cases have been reported in the Argentinian, 17,18 Peruvian, 7 Chilean, 19 Brazilian, 8 Colombian, 5,14,20,21 and Bolivian 22 population, which demonstrates that even being a rare cause of the paraneoplastic syndrome is of great interest in clinical practice.…”