Doege-potter syndrome: a report of a histologically benign but clinically malignant case

Abstract

BackgroundSolitary fibrous tumors of the pleura (SFTPs) are relatively rare tumors that originate from mesenchymal cells of submesothelial tissue of the pleura. Most patients with SFTPs are asymptomatic; however, pleuritic chest pain, cough, and dyspnea can develop. If hypoglycemia is associated with a solitary fibrous tumor, it is referred to as the Doege-Potter syndrome.Case presentationA 70-year-old man had visited our hospital with a chief complaint of dyspnea, and he was diagnosed as having a solitary fibrous tumor. A few years later, he developed hypoglycemia, and he underwent excision of the mass.ConclusionOccasionally, SFTPs induce several paraneoplastic events, such as hypertrophic osteoarthropathy. We described here a patient with an SFTP with Doege-Potter syndrome who was successfully treated with complete resection. Although lesions can be histologically benign, they can clinically present with malignant features.

Pleural Solitary Fibrous Tumors—A Retrospective Study on 45 Patients

“…NITCH is encountered in approximately 4-5% of PSFT cases [34], and can be present in solitary pleural fibroma as well as in pelvic ones [35,36]. In our study group, out of 45 cases, Doege-Potter syndrome was present in two patients with malignant PSFT (4.44%).…”

“…NICTH syndrome disappears with the tumor resection, a few days after the surgical procedure (3-4 days) [35]. In the cases of tumors that cannot be surgically removed, presenting with hypoglycaemia, some authors suggest alternative treatments such as a combination of two agents (Temozolomide and Bevacizumab), or radio embolization with yttrium 90 (Y 90 )-labelled glass microsphere [36,37].…”

Doege-Potter Syndrome: A Presumptive Case of Metastatic Hemangiopericytoma with Persistent Hypoglycemia in a 27-Year-Old Male

“…7 Our patient's medical history, presentation, histologic and diagnostic findings of metastatic hemangio-ideal benefit of IGF-II, these findings consolidated the presumptive diagnosis of hypoglycemia secondary to hemangiopericytoma or solitary fibrous tumor. 1 This is a pattern seen in a rare disease entity called Doege-Potter Syndrome.…”

“…This condition is called Doege-Potter syndrome (DPS), which accounts for less than 5% of cases. 1 DPS is a rare paraneoplastic condition characterized by hypoinsulinemic hypoglycemia secondary to ectopic secretion of the prohormone of IGF-II. 2 Only 45 cases of Doege-Potter syndrome have been reported worldwide.…”

Solitary fibrous tumor of the pleura with Doege–Potter syndrome: Second recurrence in a 93-year-old female

“…Paraneoplastic syndrome, such as hypoglycemia or hypertrophic pulmonary osteoarthropathy, may occur in 3%–4% of SFT. 1,4 Associated refractory hypoglycemia is reported as Doege–Potter syndrome and is caused by the inappropriate secretion of a high-molecular-weight form of IGF-II. This molecule activates insulin receptors, inhibiting hepatic gluconeogenesis and increasing glucose uptake, which results in hypoglycemia.…”