Doege-Potter Syndrome and Hypoglycemia associated with Solitary Fibrous Tumor of the Pleura: Two Case Reports.
Doege-Potter Syndrome and Hypoglycemia associated with Solitary Fibrous Tumor of the Pleura: Two Case Reports.
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DOI：
10.1177/1179548420964759
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发表时间：
2020
期刊：
影响因子：
--
通讯作者：
Sua LF
中科院分区：
文献类型：
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作者：
Fernández-Trujillo L;Bolaños JE;Álvarez C;Giraldo J;Velásquez M;Zúñiga-Restrepo V;Pérez B;Sua LF
胸膜孤立性纤维瘤（SFTP）是罕见的间叶性肿瘤，起源于胸膜间叶细胞生长，往往是单个肿瘤，通常有惰性病程并表现出非特异性症状。 SFTP 通常可以通过胸部偶然发现单个肿块来诊断，并应通过活检和免疫组织化学进行确认。少数病例可能出现与胰岛素样生长因子 2 (IGF-2) 产生相关的 Doege-Potter 综合征（DPS，难治性低血糖发作）。 SFTP 和 DPS 都是罕见事件，全球报告的病例数不到 2000 例。治愈性治疗是肿瘤切除术。下面介绍两例由SFTP引起的DPS患者。
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.