Doege-Potter Syndrome with a Benign Solitary Fibrous Tumor: A Case Report and Literature Review

Abstract

Doege-Potter syndrome is a rare paraneoplastic syndrome that is often diagnosed incidentally during the workup of hypoglycemia of unclear etiology. It is characterized by a non-islet cell tumor hypoglycemia secondary to excessive production of partially processed IGF-II hormone from a solitary fibrous tumor (SFT). Often these tumors are intrathoracic, benign, and asymptomatic. Occasionally they present as a paraneoplastic event; hypertrophic osteoarthropathy in Pierre-Marie-Bamberger syndrome and hypoglycemia in Doege-Potter syndrome. The NAB2-STAT6 gene fusion is the hallmark of the SFT. Complete surgical resection of the tumor often results in resolution of symptoms and cure in most cases. Here we present the case of an 83-year-old non-diabetic female with recurrent syncopal events who was diagnosed with the Doege-Potter syndrome secondary to a SFT of pleura. Her tumor was positive for NAB2-STAT6 gene fusion on RT-PCR. Following the resection of the giant tumor mass, she became symptom-free within 24 h, and has remained asymptomatic at 4 months follow-up.

Doege-Potter syndrome due to endothoracic solitary hypoglycemic fibrous tumor

“…This hormone is responsible for glycemic control, and in Doege-Potter syndrome it is common for insulin levels to be decreased. As in previous studies, 5,6 our patient had low levels of basal insulin (0.04 U / I) and C-peptide (0.01 ng / dL).…”

“…It has been described that the most frequent age of SFT is older adults. Several studies have reported SFT in patients aged 67 and 83 years, 5,9,11 although there are case series in patients aged 48 years, 12 52 years, 8 55 years, 6 and 58 years. 3 In this study, an elderly patient (74 years old) with Doege-Potter syndrome secondary to benign SFT was reported for the first time in Peru, which is different from previous reports.…”

Doege-Potter syndrome due to endothoracic solitary hypoglycemic fibrous tumor

“…4 The SFT associated with Doege-Potter syndrome reported in this 74-year-old patient has demonstrated positivity for BCL2 +, focal CD34 +, and Focal CD99 +. Mohammed et al, 5 have reported a large number of lesions that are positive for CD34 + and CD99 +, as well as other case reports, have shown immunoreactivity of these cellular markers. 6,7 However, the authors emphasize that these immunohistochemical markers do not have high specificity and may be present in many soft tissue neoplasms.…”

“…Previous studies have described complete resection as an effective treatment for this disease, as seen in some cases, including the one we reported in this article. 4,5,9 In addition, other surgical techniques with a favourable prognosis have been reported as successful. 3,13 With the discovery of the NAB2-STAT6 gene fusion as the characteristic molecular driver of SFT, it is possible that embolization therapy and radiation will be effective.…”

“…Based on this, individualized therapies aimed at inhibiting the descending pathways of NAB2-STAT6 could be considered for the treatment of this rare group of neoplasms. 5 Finally, there is a growing interest in SFT and Doege-Potter syndrome. In Latin America, several cases have been reported in the Argentine, 14,15 Peruvian, 6 Chilean, 16 Brazilian, 8 Colombian, 7,11,17,18 and Bolivian 19 population, showing that even being a rare cause of the paraneoplastic syndrome is of great interest in clinical practice.…”