Yang Jie, Yang Jiaxin, Yu Mei, Yuan Zhen, Cao Dongyan, Keng Shen
Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Science, Beijing, China.
Int J Gynecol Cancer. 2017 Feb;27(2):311-314. doi: 10.1097/IGC.0000000000000872.
Rhabdomyosarcoma (RMS) is a rare tumor in girls. We intend to analyze the clinical feature, therapeutic effect, and prognosis of pediatric and adolescent RMS of the female genitourinary tract.
Pediatric or adolescent patients with female genital tract RMS in 1 tertiary hospital from 1995 to 2015 were retrospectively reviewed. The medical records were collected, including general information, tumor primary site, stage, histology, treatments, adverse effects, and survival outcomes.
There were 8 girls with genital tract tumor in 403 patients with RMS during 20 years in 1 hospital: 5 originated in the vagina, 2 in the cervix, and 1 in the pelvis. Except for the patient with pelvic RMS who was diagnosed after acute abdominal pain, all the other patients presented with polypoid masses protruding from the vagina accompanied by mucosanguineous discharge. Mean age of patients at diagnosis was 5.4 years (8 months-14 years). Patients were categorized by using clinical group and TNM classification of malignant tumours staging system, 7 with stage I and 1 with stage II; none of them were classified to a higher stage. Histologically, all RMS were embryonal tumors with 3 botryoid variants. Patients were treated with surgery and adjuvant chemotherapy during which 6 patients developed fourth degree myelosuppression. All patients achieved complete remission. Two patients with vaginal RMS and the patient with pelvic RMS experienced tumor relapse and 2 patients died.
Rhabdomyosarcoma is a rare malignant sarcoma with a higher incidence in children and adolescents. Tumor sites are strongly associated with age at diagnosis. After combined therapy with local excision and chemotherapy, most patients can achieve a good prognosis. The prognosis is highly correlated with tumor site and histologic type.
横纹肌肉瘤（RMS）在女孩中是一种罕见肿瘤。我们旨在分析女性泌尿生殖道小儿及青少年RMS的临床特征、治疗效果及预后。
回顾性分析1995年至2015年期间1家三级医院收治的女性生殖道RMS小儿或青少年患者。收集病历资料，包括一般信息、肿瘤原发部位、分期、组织学类型、治疗方法、不良反应及生存结果。
20年间，1家医院的403例RMS患者中有8例女孩患有生殖道肿瘤：5例起源于阴道，2例起源于宫颈，1例起源于盆腔。除盆腔RMS患者因急性腹痛后确诊外，其他患者均表现为从阴道突出的息肉样肿物并伴有黏液血性分泌物。患者诊断时的平均年龄为5.4岁（8个月至14岁）。采用临床分组及恶性肿瘤TNM分期系统对患者进行分类，7例为Ⅰ期，1例为Ⅱ期；均未分类至更高分期。组织学上，所有RMS均为胚胎性肿瘤，有3例葡萄状变型。患者接受手术及辅助化疗，其中6例患者发生Ⅳ度骨髓抑制。所有患者均达到完全缓解。2例阴道RMS患者及盆腔RMS患者出现肿瘤复发，2例患者死亡。
横纹肌肉瘤是一种罕见的恶性肉瘤，在儿童和青少年中发病率较高。肿瘤部位与诊断时的年龄密切相关。经局部切除及化疗联合治疗后，多数患者可获得良好预后。预后与肿瘤部位及组织学类型高度相关。
