Division of Endocrinology, Diabetes & Metabolism, University of Tennessee Health Science Center, Memphis, Tennessee.
Memphis Pathology Group, Memphis, Tennessee.
Am J Med Sci. 2018 Aug;356(2):181-184. doi: 10.1016/j.amjms.2018.01.008. Epub 2018 Jan 31.
Nonislet cell tumor hypoglycemia is rare. We highlight the diagnosis and treatment of recurrent severe hypoglycemia in a 49-year-old woman with malignant solitary fibrous tumor of the pleura (Doege-Potter syndrome). The clinical, laboratory and radiologic findings of the case are presented and a brief literature review is provided. Of note, imaging studies showed a large mass in the right hemithorax and pathology and immunehistochemical stains confirmed a malignant solitary fibrous tumor of the pleura. She was a poor surgical candidate owing to a large tumor burden. She was treated with a combination of temozolomide and bevacizumab to which she responded with resolution of hypoglycemia. The treatment of choice for hypoglycemia in patients with the Doege-Potter syndrome is surgical excision. We here report that a combination of temozolomide and bevacizumab may be a viable option in patients with inoperable disease.
非胰岛细胞瘤性低血糖症较为罕见。我们重点介绍了一例 49 岁女性患者的诊断和治疗经过,该患者患有恶性胸膜孤立性纤维瘤(Doege-Potter 综合征),并反复发作严重低血糖。本文呈现了该病例的临床、实验室和影像学检查结果,并进行了简要的文献复习。值得注意的是,影像学检查显示右胸腔有一个大肿块,且病理和免疫组化染色证实为恶性胸膜孤立性纤维瘤。由于肿瘤负荷大,该患者不适合手术。她接受了替莫唑胺和贝伐珠单抗联合治疗,低血糖得到缓解。Doege-Potter 综合征患者低血糖的治疗选择是手术切除。我们在此报告,替莫唑胺和贝伐珠单抗联合治疗可能是无法手术患者的一种可行选择。