The Spectrum of Thyroid Gland Pathology in Carney Complex: The Importance of Follicular Carcinoma.
The Spectrum of Thyroid Gland Pathology in Carney Complex: The Importance of Follicular Carcinoma.
复制标题
DOI：
10.1097/pas.0000000000000975
复制
发表时间：
2018-05
期刊：
影响因子：
--
通讯作者：
Stratakis CA
中科院分区：
文献类型：
--
作者：
Carney JA;Lyssikatos C;Seethala RR;Lakatos P;Perez-Atayde A;Lahner H;Stratakis CA
1985年，Carney综合征（CNC）的最初描述包括粘液瘤、斑点状皮肤色素沉着和内分泌过度活跃（肾上腺、垂体和睾丸）。1997年，3例CNC患者发现甲状腺肿瘤，并且该综合征的腺体受累明显。在此，我们描述了26例CNC和甲状腺疾病患者的临床、病理和随访结果。患者主要是中年妇女，无症状的甲状腺肿块。甲状腺功能亢进4例，其中2例为甲状腺滤泡增生，2例为甲状腺毒性腺瘤。病理结果包括16例良性病变（滤泡性增生、结节性增生和滤泡性腺瘤）和10例癌（滤泡性或乳头状）。滤泡状癌具有多灶性、双侧性和淋巴结转移的特点。肿瘤直径大于或等于3 cm的4例患者中有3例死亡。1例患者有不寻常的多灶性显微镜下滤泡增生。发现和治疗甲状腺肿瘤的患者与数控需要长期随访的患者的综合征。
The initial description of Carney complex (CNC) in 1985 included myxomas, spotty skin pigmentation, and endocrine overactivity (of the adrenal, the pituitary, and the testis). In 1997, thyroid neoplasms were found in 3 patients with CNC and involvement of the gland in the syndrome was apparent. Herein, we describe the clinical, pathologic, and follow-up findings in 26 patients with CNC and a disorder of the thyroid gland. The patients were predominantly middle-aged women with an asymptomatic thyroid mass. Four patients had hyperthyroidism, which was caused by follicular hyperplasia in 2 patients and by toxic adenoma in 2 others. Pathologic findings included benign lesions (follicular hyperplasia, nodular hyperplasia, and follicular adenoma) in 16 patients and carcinomas (follicular or papillary) in 10 patients. The follicular carcinomas had unusual features, multifocality, bilaterality, and lymph node metastasis. The tumor was fatal in 3 of 4 patients with a tumor 3 cm or greater in diameter. One patient had an unusual multifocal microscopic follicular hyperplasia. Detection and treatment of the thyroid neoplasms in patients with CNC requires long-term follow-up of patients with the syndrome.