Sardinha Marina Gomes Pereira, Ramajo Fábio Morozetti, Ponce Cesar Cilento, Marques Camila Franzin, Bittencourt Carolina Marques Fontes, Caldano Fernando Gardin, Moço José Matheus Frizzo Lopes, Yano Otávio de Lacquila, Reis Pedro Marques da Rosa, Malaguti Vinicius Silva, de Sousa Catarina Tealdi Reno Gandra, Nogueira Roberto Cesar
Centro Universitário Lusíada. Santos, SP, Brazil.
Hospital Ana Costa, Department of Ginecology. Santos, SP, Brazil.
Autops Case Rep. 2019 Jul 12;9(3):e2019104. doi: 10.4322/acr.2019.104. eCollection 2019 Jul-Sep.
Rhabdomyosarcoma (RMS) is a rare solid tumor in childhood and adolescence. The higher incidence is predominant during the first two decades of life. According to the Intergroup RMS Study Group, the embryonal RMS (ERMS), botryoidal variant, constitutes a histological subtype characterized as a "grape-like" lesion of 2.0 cm to 9.5 cm. The treatment involves chemotherapy, surgery, and/or radiotherapy. We present the case of a 14-year-old female patient diagnosed with ERMS, botryoidal variant, which originated in the uterine cervix with vaginal externalization. The initial therapeutic approach comprised an initial prolapsed mass excision followed by Wertheim-Meigs surgery due to the tumor extension. No consensual protocol to ERMS treatment is found in the medical literature; however, a combined approach seems to offer a better result. The postoperative time period was uneventful and the patient followed an adjuvant therapy with vincristine, d-actinomycin, and cyclophosphamide. A comprehensive evaluation of the therapeutic options preserving the reproductive function-unfortunately not always possible-is part of a multi-disciplined care team concerning the pediatric patients.
横纹肌肉瘤(RMS)是儿童和青少年时期罕见的实体瘤。在生命的前二十年中发病率较高。根据横纹肌肉瘤国际协作组研究小组的定义,葡萄状胚胎性横纹肌肉瘤(ERMS)是一种组织学亚型,其特征为大小在2.0厘米至9.5厘米之间的“葡萄样”病变。治疗方法包括化疗、手术和/或放疗。我们报告了一例14岁女性患者,诊断为葡萄状胚胎性横纹肌肉瘤,起源于宫颈并伴有阴道外露。初始治疗方法包括首先切除脱垂肿块,随后由于肿瘤扩散进行了Wertheim-Meigs手术。医学文献中未发现针对胚胎性横纹肌肉瘤治疗的共识方案;然而,综合治疗方法似乎能取得更好的效果。术后恢复顺利,患者接受了长春新碱、放线菌素D和环磷酰胺的辅助治疗。对于儿科患者,保留生殖功能的治疗选择的综合评估——遗憾的是并非总是可行——是多学科护理团队工作的一部分。