Ibrahim Uroosa, Saqib Amina, Mohammad Farhan, Ding Juan, Salman Blerina, Collado Fady K, Dhar Meekoo
Department of Hematology and Oncology, Staten Island University Hospital.
Pulmonary/Critical Care, Staten Island University Hospital.
Cureus. 2017 Nov 21;9(11):e1864. doi: 10.7759/cureus.1864.
Embryonal rhabdomyosarcoma (RMS) is a rare type of sarcoma, primarily seen in the pediatric and adolescent population. Three subtypes of embryonal RMS are described, with the botryoid type being the most common. The incidence of this disease in adult females is 0.4% to 1% with the affected age group being patients in the third to fourth decade of life. It is exceedingly rare in patients above 40 years of age. We describe the case of a 48-year-old female, gravida 9 para 5, who presented with abnormal vaginal bleeding and an exophytic mass on examination. Given her lack of requirement of maintaining parity, she underwent radical surgery. The tumor was 8 cm in the largest dimension with a high histologic grade and some cartilaginous differentiation. Immunohistochemical stains were positive for vimentin, CD99, myogenin, and MyoD1 consistent with a diagnosis of embryonal rhabdomyosarcoma, botryoid subtype. Based on high survival rates when treated with aggressive adjuvant chemotherapy, a decision was made to treat the patient with the ARST0331 regimen. We discuss the diagnostic pathologic features of the disease, the epidemiology, and the most common presentation along with prognostic factors, treatment strategies, and outcomes.
胚胎性横纹肌肉瘤（RMS）是一种罕见的肉瘤类型，主要见于儿童和青少年人群。胚胎性RMS有三种亚型，葡萄状型最为常见。成年女性中这种疾病的发病率为0.4%至1%，受影响的年龄组为30至40岁的患者。在40岁以上的患者中极为罕见。我们描述了一例48岁女性，孕9产5，因异常阴道出血就诊，检查发现有外生性肿物。鉴于她没有保留生育功能的需求，她接受了根治性手术。肿瘤最大直径为8 cm，组织学分级高，有一些软骨分化。免疫组化染色波形蛋白、CD99、肌细胞生成素和肌分化抗原1阳性，符合胚胎性横纹肌肉瘤葡萄状亚型的诊断。基于积极辅助化疗治疗时的高生存率，决定采用ARST0331方案治疗该患者。我们讨论了该疾病的诊断病理特征、流行病学、最常见的表现以及预后因素、治疗策略和结果。
