Doege–Potter Syndrome and Hypoglycemia associated with Solitary Fibrous Tumor of the Pleura: Two Case Reports

Abstract

Solitary fibrous tumors of the pleura (SFTP) are rare mesenchymal neoplasms that originate from mesenchymal growth in the pleura, tend to be single tumors, usually have an indolent course and show nonspecific symptoms. SFTP can be often diagnosed from an incidental finding of a single mass in the thorax and should be confirmed by biopsy and immunohistochemistry. A minority of cases may present Doege–Potter syndrome (DPS, episodes of refractory hypoglycemia) associated with production of insulin-like growth factor 2 (IGF-2). Both SFTP and DPS are rare occurrences with less than 2000 cases reported worldwide. The curative treatment is tumor resection. Two cases of patients with DPS caused by SFTP are presented below.

Doege-Potter Syndrome: A Rare Paraneoplastic Syndrome of Solid Fibrous Tumors Reported in Indonesia

“…Fewer than 2,000 cases reported worldwide, SFT is a rare case, with its origins mostly from visceral pleura. 2,9 Recognized first in 1870, the incidence of the case is usually 60-70 years old with a rate of 2.8 of 10,000 people, and it is composed of about 5% of total pleural tumor developments. SFT is usually a benign tumor with an asymptomatic respiratory manifestation like dyspnea or chest pain, it rarely comes with paraneoplastic syndrome.…”

“…Solitary fibrous tumors (SFT), which originate from mesenchymal cells, are rare groups of tumors and often manifest as benign, asymptomatic, and intrathoracic. 1,2 Paraneoplastic syndrome, like Doege-Potter syndrome (DPS), is occasionally associated with these tumors. 3 Karl Doege and Roy Potter first found and described the presence of this syndrome as a non-betapancreatic cell tumor.…”

“…5 DPS presents hyperinsulinemic hypoglycemia from the ectopic secretion of insulin-like growth factor II (IGF-II) from the non-islet cell from a solitary fibrous tumor. 2 SFT is a large, slow-growing tumor that origins from mesenchymal and causes DPS in about 2-4% of cases. Tumoral overproduction of incompletely processed IGF-II is the most common cause of hypoglycemia in this type of tumor.…”

Doege–Potter syndrome due to endothoracic solitary hypoglycemic fibrous tumor

“…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.…”

Doege-Potter syndrome due to endothoracic solitary hypoglycemic fibrous tumor

“…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.…”