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首页> 外文期刊>Experimental and therapeutic medicine >Treatment protocols for growth hormone-secreting pituitary adenomas combined with craniofacial fibrous dysplasia: A case report of atypical McCune-Albright syndrome
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Treatment protocols for growth hormone-secreting pituitary adenomas combined with craniofacial fibrous dysplasia: A case report of atypical McCune-Albright syndrome

机译：生长激素分泌型垂体腺瘤合并颅面纤维异常增生的治疗方案：非典型麦康-奥尔布赖特综合征的一例报道

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摘要

McCune-Albright syndrome (MAS) is a rare, post-zygotic (non-germline) disorder, characterized by hypersecretory endocrinopathies, fibrous dysplasia of the bone and café-au-lait macules. The most common endocrine dysfunction is gonadal hyperfunction; thus, hypersecretion of growth hormones (GHs) as a manifestation of endocrine hyperfunction in MAS is rarely reported. MAS affects both genders, although the majority of cases have been reported in young females. Atypical presentations of MAS, with only one or two of the classic symptoms, have been previously described, but remain particularly challenging due to the lack of a diagnostic phenotype. In patients with atypical MAS, analysis of mutations in the gene of the α-subunit of the stimulatory G-protein is limited; thus, diagnosis is based on clinical judgment. In the present study, a male with polyostotic fibrous dysplasia and GH-secreting pituitary adenomas, diagnosed with atypical MAS, was reported. The pituitary adenoma was effectively treated with radiotherapy and the patient underwent surgery for the polyostotic fibrous dysplasia, with marked improvements observed in appearance.
机译：McCune-Albright综合征（MAS）是一种罕见的合子后（非生殖细胞）疾病，其特征是分泌过多的内分泌病变，骨骼的纤维异常增生和café-la-lait的黄斑。最常见的内分泌功能障碍是性腺功能亢进。因此，很少报道生长激素（GHs）分泌过多是MAS内分泌功能亢进的表现。尽管大多数病例都报告在年轻女性中，但MAS会影响这两种性别。先前已描述了仅具有一种或两种典型症状的MAS的非典型表现，但由于缺乏诊断表型，仍然具有特别的挑战性。对于非典型MAS患者，刺激性G蛋白α亚基基因突变的分析是有限的。因此，诊断是基于临床判断。在本研究中，报告了一名男性，患有多发性纤维化异型增生和分泌GH的垂体腺瘤，被诊断为非典型MAS。垂体腺瘤已通过放射治疗得到了有效治疗，患者接受了多骨性纤维化异常增生手术，外观明显改善。

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