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多发性骨纤维结构不良伴巨人症和巨大骨盆肿瘤：McCune-Albright 综合征的罕见病例。

Polyostotic fibrous dysplasia with gigantism and huge pelvic tumor: a rare case of McCune-Albright syndrome.

机构信息

Department of Bone and Joint Surgery, Ehime University Graduate School of Medicine, Shitsukawa, Toon, Ehime 791-0204, Japan.

出版信息

Int J Clin Oncol. 2011 Jun;16(3):270-4. doi: 10.1007/s10147-010-0127-9. Epub 2010 Sep 29.

DOI:10.1007/s10147-010-0127-9
PMID:20878436
Abstract

We report a rare case of polyostotic fibrous dysplasia on endocrine hyperfunction with elevated human growth hormone and normal serum level of prolactin. There were some differential points of gender, gigantism, endocrine function, and GNAS gene from McCune-Albright syndrome. Malignant transformation was suspected in the pelvic tumor from imaging because rapid growth of the tumor by imaging was observed; however, no malignant change occurred in this case.

摘要

我们报告了一例罕见的多骨性纤维结构不良伴内分泌功能亢进，表现为人增长激素升高而催乳素血清水平正常。该病与 McCune-Albright 综合征在性别、巨人症、内分泌功能和 GNAS 基因等方面存在一些不同点。由于影像学检查发现肿瘤快速生长，怀疑盆腔肿瘤发生恶性转化；然而，本例并未发生恶性变化。

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引用本文的文献

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本文引用的文献

1
Activating Gsalpha mutations: analysis of 113 patients with signs of McCune-Albright syndrome--a European Collaborative Study.激活型Gsα突变：113例McCune-Albright综合征体征患者的分析——一项欧洲协作研究
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THE MALIGNANT TRANSFORMATION OF FIBROUS DYSPLASIA.骨纤维异常增殖症的恶性转化
Am J Med Sci. 1964 Jan;247:1-20. doi: 10.1097/00000441-196401000-00001.
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Thyroid carcinoma in the McCune-Albright syndrome: contributory role of activating Gs alpha mutations.
McCune-Albright综合征中的甲状腺癌：激活型Gsα突变的作用
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Mechanisms of bisphosphonate effects on osteoclasts, tumor cell growth, and metastasis.双膦酸盐对破骨细胞、肿瘤细胞生长及转移的作用机制。
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Searching for Arg201 mutations in the GNAS1 gene in Italian patients with McCune-Albright syndrome.在意大利患有麦库恩-奥尔布赖特综合征的患者中寻找GNAS1基因的Arg201突变。
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Dedifferentiated chondrosarcoma in Albright syndrome. A case report and review of the literature.奥尔布赖特综合征中的去分化软骨肉瘤。病例报告及文献复习。
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Fibrous dysplasia of the spine with sarcomatous transformation: a case report and review of the literature.脊柱骨纤维发育不良伴肉瘤样变：一例报告并文献复习
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CT of the "Tegernsee Giant": juvenile gigantism and polyostotic fibrous dysplasia.“特格恩湖巨人”的CT检查：青少年巨人症和多骨纤维发育不良。
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