Mohammed Saeed R, Mack Narine, Ramoutar Valmikie, Rampersad Jessica
Department of Clinical Medical Sciences, The University of the West Indies, St. Augustine Campus, Champs Fleurs, TTO.
Department of Medicine, San Fernando General Hospital, San Fernando, TTO.
Cureus. 2024 Jun 27;16(6):e63329. doi: 10.7759/cureus.63329. eCollection 2024 Jun.
Solitary fibrous tumors (SFT) are mesenchymal cell tumors that may arise from any site throughout the body. A small percentage of patients with SFT develop non-islet cell tumor-induced hypoglycemia (NICTH), eponymously termed Doege-Potter Syndrome (DPS). DPS is characterized by severe, refractory hypoinsulinemic hypoglycemia. Diagnosis of SFT is dependent on histologic findings and immunohistochemistry (IHC). NAB2-STAT6 gene fusions are pathognomonic for SFT but may be difficult to identify in routine cytogenetic studies. STAT6 IHC is a highly sensitive and specific surrogate for the NAB2-STAT6 gene fusion. Total resection of the tumor remains the gold-standard definitive treatment of SFT of the pleura. Palliative tumor debulking is recommended if total resection is not feasible. We here report a case of DPS in a 73-year-old female, managed with palliative care.
孤立性纤维性肿瘤（SFT）是一种间充质细胞瘤，可发生于全身任何部位。一小部分SFT患者会发生非胰岛细胞瘤所致低血糖症（NICTH），即所谓的多伊奇-波特综合征（DPS）。DPS的特征是严重的难治性低胰岛素血症性低血糖。SFT的诊断依赖于组织学检查结果和免疫组织化学（IHC）。NAB2-STAT6基因融合是SFT的特征性表现，但在常规细胞遗传学研究中可能难以识别。STAT6免疫组化是NAB2-STAT6基因融合的高度敏感和特异的替代指标。肿瘤全切除仍然是胸膜SFT的金标准确定性治疗方法。如果无法进行全切除，则建议进行姑息性肿瘤减瘤手术。我们在此报告一例73岁女性的DPS病例，采用姑息治疗。
