Department of Obstetrics and Gynecology, Baylor College of Medicine, USA.
Gynecol Oncol. 2012 Sep;126(3):351-6. doi: 10.1016/j.ygyno.2012.05.008. Epub 2012 May 15.
Cervical rhabdomyosarcoma is extremely rare, and there is a paucity of literature on the subject. The purpose of this study was to describe the clinical and pathologic features of cervical rhabdomyosarcoma.
We retrospectively reviewed all patients with cervical rhabdomyosarcoma who presented to our institution from 1980 to 2010. We reviewed pathologic, demographic, and clinical information.
During the study period, 11 females presented with cervical rhabdomyosarcoma. The median age at presentation was 18.4 years, and 6 patients were <19 years old at diagnosis. Vaginal bleeding was the most common presenting symptom, and a vaginal mass was often a co-presenting symptom. Eight patients (73%) presented with stage IB disease, and 8 (73%) presented with the embryonal (botryoid) histologic subtype. Nine patients (82%) received multimodal therapy consisting of surgery with chemotherapy, radiation therapy, or both. All patients were without evidence of disease after completion of primary therapy, but 3 patients experienced local recurrence. At a median follow-up of 23 months, 6 patients (55%) were without evidence of disease, 1 (9%) was alive with disease, 1 (9%) had died of disease, and 3 (27%) had died of other causes. Three patients (27%) had other primary malignancies in addition to rhabdomyosarcoma-1 had a Sertoli-Leydig tumor, 1 had a Sertoli-Leydig tumor and a pinealoblastoma, and 1 had thyroid cancer and a parotid adenocarcinoma.
With multimodal therapy, cervical rhabdomyosarcoma appears to be associated with a good prognosis. Favorable prognostic factors such as early stage at diagnosis and a favorable histologic subtype may contribute to the excellent observed survival.
宫颈横纹肌肉瘤极为罕见,相关文献也很少。本研究旨在描述宫颈横纹肌肉瘤的临床和病理特征。
我们回顾性分析了 1980 年至 2010 年期间我院收治的所有宫颈横纹肌肉瘤患者的临床和病理资料。我们分析了病理、人口统计学和临床资料。
研究期间,共有 11 例女性患有宫颈横纹肌肉瘤。中位发病年龄为 18.4 岁,6 例患者在诊断时年龄<19 岁。阴道出血是最常见的首发症状,常伴有阴道肿块。8 例(73%)患者为 IB 期,8 例(73%)为胚胎型(葡萄状)组织学亚型。9 例(82%)患者接受了多模式治疗,包括手术联合化疗、放疗或两者联合。所有患者在完成初始治疗后均无疾病证据,但 3 例患者出现局部复发。中位随访 23 个月时,6 例(55%)患者无疾病证据,1 例(9%)患者疾病存活,1 例(9%)患者死于疾病,3 例(27%)患者死于其他原因。除横纹肌肉瘤外,还有 3 例患者(27%)患有其他原发性恶性肿瘤——1 例为 Sertoli-Leydig 细胞瘤,1 例为 Sertoli-Leydig 细胞瘤伴松果体细胞瘤,1 例为甲状腺癌伴腮腺腺癌。
采用多模式治疗后,宫颈横纹肌肉瘤似乎预后良好。早期诊断和有利的组织学亚型等有利预后因素可能是观察到的良好生存的原因。