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伴有胰岛素样生长因子-2过表达的大的孤立性纤维瘤

Large solitary fibrous tumor with overexpression of insulin-like growth factor-2.

作者信息

Okabe Ryo, Sonobe Makoto, Bando Toru, Date Hiroshi

机构信息

Department of Thoracic Surgery, Kyoto University Hospital, Shogoin-Kawara-cho 54, Sakyo-ku, Kyoto 606-8507, Japan.

出版信息

Interact Cardiovasc Thorac Surg. 2010 Nov;11(5):688-90. doi: 10.1510/icvts.2010.240770. Epub 2010 Aug 13.

Abstract

We present the case of a 66-year-old woman in whom a large solitary fibrous tumor (SFT) in the right thoracic cavity caused intermittent symptoms of hypoglycemia. A diagnosis was made of non-islet cell tumor hypoglycemia on the basis of the presence of hypoglycemia requiring continuous glucose infusion, elevated serum insulin-like growth factor-2 (IGF-2), and a large well-defined tumor in the right thoracic cavity. The patient underwent complete resection of the tumor. Histological examination revealed spindle tumor cells with a hemangiopericytoma-like vascular pattern. Mitotic figures and necrotic areas were rare, and cellular atypia and nuclear pleomorphism were mild. Under immunohistochemical examination, the tumor cells were positive for CD34. Overexpression of IGF-2 mRNA in the tumor was detected by reverse-transcription polymerase-chain reaction. The diagnosis of SFT with IGF-2 production was confirmed. Immediately after surgery, her serum glucose level was normalized (without the need for glucose infusion) and serum IGF-2 level was decreased. Two years after surgery, the patient remains alive and well, with no signs of recurrence or hypoglycemia.

摘要

我们报告一例66岁女性患者,其右胸腔内的一个巨大孤立性纤维瘤(SFT)导致间歇性低血糖症状。基于存在需要持续输注葡萄糖的低血糖、血清胰岛素样生长因子-2(IGF-2)升高以及右胸腔内一个边界清晰的大肿瘤,诊断为非胰岛细胞瘤低血糖症。患者接受了肿瘤的完整切除。组织学检查显示梭形肿瘤细胞,具有类似血管外皮细胞瘤的血管模式。有丝分裂象和坏死区域少见,细胞异型性和核多形性轻度。免疫组织化学检查显示肿瘤细胞CD34阳性。通过逆转录聚合酶链反应检测到肿瘤中IGF-2 mRNA的过表达。确诊为产生IGF-2的SFT。术后即刻,她的血清葡萄糖水平恢复正常(无需输注葡萄糖),血清IGF-2水平下降。术后两年,患者存活且状况良好,无复发或低血糖迹象。

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