儿童肿瘤相关性早熟30例病因及临床特征  

Etiology and clinical characteristics of 30 children with tumor-associated precocity

作　　者：刘敏[1] 倪桂臣[1] 

机构地区：[1]首都医科大学附属北京儿童医院内分泌遗传代谢中心,儿科学国家重点学科,100045

出　　处：《中华实用儿科临床杂志》2013年第20期1548-1551,共4页Chinese Journal of Applied Clinical Pediatrics

摘　　要：目的分析儿童肿瘤相关性早熟的病因和临床特征。方法选取2006年1月至2012年3月首都医科大学附属北京儿童医院外科收治的30例肿瘤相关性早熟患儿为研究对象，对其病因、临床特征和治疗情况等进行回顾性分析。结果本研究对象包括男14例，女16例；年龄（3．74±2．44）岁，其中0-〈5岁22例（73．3％）。其病因构成：下丘脑错构瘤8例；下丘脑生殖细胞瘤2例；蛛网膜囊肿1例；肾上腺皮质肿瘤7例（腺瘤1例，皮质癌6例）；卵巢囊肿5例；卵巢肿瘤2例（内胚窦瘤、性索问质肿瘤各1例）；MuCune—Albright综合征2例；纵隔畸胎瘤、阴茎原始神经外胚层肿瘤、睾丸问质细胞增生伴瘤结节各1例。中枢性性早熟1l例（36．7％），病因以下丘脑错构瘤（8例）最多见，其中4例下丘脑错构瘤患儿伴有痴笑癫痫，应用促性腺激素释放激素类似物治疗可有效控制患儿的性早熟症状。外周性性早熟19例（63．3％），病因男童以肾上腺皮质肿瘤、女童以卵巢囊肿居多。除1例肾上腺皮质癌患者以面部痤疮伴多毛起病、1例卵巢囊肿患者以间断腹痛起病，其余男童均以外生殖器发育、女童均以乳房发育或阴道出血为首发症状。结论肿瘤是儿童性早熟的重要病因之一。在性早熟诊治过程中，除应重视性早熟患者垂体、性腺以及肾上腺的影像学检查外，还需注意一些少见部位发生的肿瘤。卵巢囊肿伴性早熟的患者应注意与MuCune—Albright综合征鉴别。Objective To analyze the etiology and clinical characteristics of children with tumor-associated precocity. Methods Thirty children with tumor-associated precocity hospitalized in Department of Surgery of Beijing Children＇s Hospital Affiliated to Capital Medical University from Jan. 2006 to Mar. 2012 were selected as research sub- jects. The causes, clinical characteristics and treatment situation of the patients were retrospectively studied. Results The group of patients included 14 boys and 16 girls, with average age of （3.74 _＋ 2.44 ）years. Twenty-two patients （73.3%） were younger than 5 years old, and their etiological distributions listed as follows ：8 cases were hypothalamic hamartoma（HH） ,2 cases were hypothalamic germinoma, 1 case was arachnoid cyst,7 cases were adrenocortical tumor （ in which 1 case was adenoma and 6 cases were adenocacinoma respectively） ,5 cases were ovarian cyst,2 cases were ovarian tumor （ in which 1 case was endodermal sinus tumor and 1 case was sex cord-stromal tumor respectively） ,2 ca- ses were MuCune-Albright syndrome, and 1 case was mediastinal teratoma, 1 case was penis primitive neuroectoderm tumor, and 1 case was Leydig cell proliferation accompanied with neoplasma. Eleven patients （36.7%） suffered central precocious puberty,with HH（ n = 8） being the most common causes. Four patients with HH presented with gelastic epi- lepsy. Precocious puberty caused by HH patients could be safely controlled by gonadotropin-releasing hormone agonists. Nineteen patients （63.3 % ） suffered peripheral precocious puberty, with adrenocortical tumor being the most common cause for the boys and ovarian cyst being the most common cause for the girls： Besides that, the onset symptom of a pa- tient with adenocacinoma was facial acne accompanied with hypertrichiasis and another patient with ovarian tumor had intermittent abdominal pain, and the onset symptoms of all the boys were external genital development and those of the girls were mammary development or co

关 键 词：性早熟 下丘脑错构瘤 肾上腺皮质肿瘤 卵巢囊肿 MuCune—Albright综合征 畸胎瘤 

分 类 号：R725.8[医药卫生—儿科]