Doege-Potter syndrome presenting with hypoinsulinemic hypoglycemia in a patient with a malignant extrapleural solitary fibrous tumor: a case report.
Doege-Potter syndrome presenting with hypoinsulinemic hypoglycemia in a patient with a malignant extrapleural solitary fibrous tumor: a case report.
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DOI：
10.1186/1752-1947-7-11
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发表时间：
2013-01-09
影响因子：
1
通讯作者：
Weiss, Geoffrey R
中科院分区：
文献类型：
--
作者：
Schutt, Robert C;Gordon, Trish A;Weiss, Geoffrey R
未贴标签：简介：Doege-Potter综合征是一种副肿瘤综合征，其特征是继发于孤立性纤维性肿瘤的非胰岛细胞肿瘤性低血糖。这种肿瘤通过分泌胰岛素样生长因子II的激素原形式引起低血糖。我们描述的诊断和管理Doege-Potter综合征和使用经动脉化疗栓塞的恶性胸膜外孤立性纤维tumor.CASE介绍：我们的病人是一个64岁的白人妇女谁最初提出了尿失禁，并被发现有一个14.5*9.0*9.0cm腹膜后孤立性纤维肿瘤压缩她的膀胱。她的肿瘤手术切除，但复发与多个肝转移灶。尽管全身化疗和多柔比星经动脉化疗栓塞治疗，肝转移仍进展。她的病程因复发性空腹低血糖而变得复杂，这种低血糖很可能继发于Doege-Potter综合征。她的低血糖症是通过皮质类固醇治疗和频繁的计划营养摄入来控制的。肝脏孤立性纤维瘤的罕见性和随后的对照试验的缺乏使得这份报告具有重要意义，因为它描述了Doege-Potter综合征的诊断方法，描述了我们使用阿霉素经动脉化疗栓塞的经验，并提出了在不适合手术切除的广泛疾病的情况下肿瘤相关的低血糖症的管理选择。
UNLABELLED:INTRODUCTION: Doege-Potter syndrome is a paraneoplastic syndrome characterized by non-islet cell tumor hypoglycemia secondary to a solitary fibrous tumor. This tumor causes hypoglycemia by the secretion of a prohormone form of insulin-like growth factor II. We describe the diagnosis and management of Doege-Potter syndrome and the use of transarterial chemoembolization in a patient with a malignant extrapleural solitary fibrous tumor.CASE PRESENTATION: Our patient was a 64-year-old Caucasian woman who initially presented with urinary incontinence and was found to have a 14.5*9.0*9.0cm retroperitoneal solitary fibrous tumor compressing her bladder. Her tumor was surgically resected but recurred with multiple hepatic metastatic lesions. The hepatic metastases progressed despite systemic chemotherapy and treatment with doxorubicin transarterial chemoembolization. Her course was complicated by the development of recurrent fasting hypoglycemia, most likely secondary to Doege-Potter syndrome. Her hypoglycemia was managed with corticosteroid therapy and frequent scheduled nutrient intake overnight.CONCLUSIONS: The rarity of hepatic solitary fibrous tumors and consequent lack of controlled trials make this report significant in that it describes the diagnostic approach to Doege-Potter syndrome, describes our experience with the use of doxorubicin transarterial chemoembolization, and presents management options for tumor-associated hypoglycemia in the case of extensive disease not amenable to surgical resection.