Mao Wangjia, Sun Yuefang, Yan Shan, Gao Yuan, Bu Le, Cheng Xiaoyun
Department of Endocrinology and Metabolism, Shanghai Tenth People's Hospital, School of Medicine, Tongji University, Shanghai, China.
Department of Pathology, Shanghai Tenth People's Hospital, Tenth People's Hospital of Tongji University, 301 Yanchang Middle Road, Shanghai 200072, China.
Case Rep Endocrinol. 2024 Oct 14;2024:4584292. doi: 10.1155/2024/4584292. eCollection 2024.
Doege-Potter syndrome (DPS) is a very rare paraneoplastic condition that is marked by hypoglycemia brought on by a solitary fibrous tumor rather than an islet cell tumor. Soft tissue neoplasms termed as solitary fibrous tumors (SFTs) are rare and these tumors vary in the site of origin, from the pleural cavity, mediastinum, pericardium, retroperitoneal spaces, liver, thyroid, orbit, bladder, intestines, and soft tissues, while pelvic-derived fibrous tumors are incredibly unusual. There are currently extremely few documented cases and literature reviews both domestically and internationally. In this case study, we present an 82-year-old woman who developed DPS as a result of malignant pelvic SFTs. Her hypoglycemia was clinically healed after she underwent laparoscopic retroperitoneal tumor resection in our institution, and thereafter, her quality of life improved.
多伊格-波特综合征(DPS)是一种非常罕见的副肿瘤性疾病,其特征是由孤立性纤维瘤而非胰岛细胞瘤引起的低血糖。被称为孤立性纤维瘤(SFT)的软组织肿瘤很罕见,这些肿瘤的起源部位各不相同,包括胸腔、纵隔、心包、腹膜后间隙、肝脏、甲状腺、眼眶、膀胱、肠道和软组织,而源自盆腔的纤维瘤极其罕见。目前国内外记录在案的病例和文献综述都极少。在本病例研究中,我们报告了一名82岁女性,她因恶性盆腔SFT而患上DPS。在我们机构接受腹腔镜腹膜后肿瘤切除术后,她的低血糖在临床上得到治愈,此后,她的生活质量得到改善。