Xu Lin-Juan, Cai Jing, Huang Bang-Xing, Dong Wei-Hong
Department of Obstetrics and Gynecology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Department of Pathology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China.
World J Clin Cases. 2022 Sep 16;10(26):9454-9461. doi: 10.12998/wjcc.v10.i26.9454.
Rhabdomyosarcoma is a soft tissue tumor of primitive mesenchymal cells origin, occurring predominantly in children and adolescents, but extremely rare in adults and the data regarding its treatment are sparse. Here, we would like to share our experience in the treatment of a locally advanced primary embryonal rhabdomyosarcoma of cervix in a 39-year-old female.
The patient was admitted with symptoms of intermenstrual bleeding and postcoital bleeding for six months. Physical examination revealed a friable, polyp-like mass (5 cm × 5 cm) in her cervix protruding into the vagina, while the uterus was mobile and normal-sized. Colposcopy-directed biopsy was performed, and a pathological diagnosis of embryonal rhabdomyosarcoma was made. Magnetic resonance imaging of the pelvis showed that the cervical volume was significantly increased, with a hypointense and hyperintense soft tissue mass on the right side, invading the cervical stroma; the mass was 5 cm × 5 cm with a clear boundary and confined to the cervix; there were no obvious findings indicating tumor invasion in the vaginal wall, parametrium, or pelvic wall; no enlarged lymph nodes were observed in the pelvic cavity. Based on our findings, the tumor was classified as stage IA according to the intergroup rhabdomyosarcoma studies criteria and IB3 stage according to The International Federation of Gynecology and Obstetrics 2018. The patient underwent two courses of neoadjuvant chemotherapy and a partial remission was achieved. Subsequently, she underwent laparoscopic radical hysterectomy, bilateral salpingo-oophrectomy and pelvic lymph node dissection and there were no risk factors revealed by postoperative pathological examination. Adjuvant chemotherapy was performed after surgery. The patient was disease-free until the last follow-up, 49 mo after completing the entire treatment.
Our experience suggests that neoadjuvant vincristine, dactinomycin, and cyclophosphamide chemotherapy followed by radical surgery and adjuvant chemotherapy might be reasonable therapeutic option for bulky cervical rhabdomyosarcoma in adults without fertility desire. Since large-scale studies on such rare conditions are rather impossible, further case reports and systematic reviews could help optimize the treatment of primary, bulky cervical rhabdomyosarcoma in adults.
横纹肌肉瘤是一种起源于原始间充质细胞的软组织肿瘤,主要发生于儿童和青少年,在成人中极为罕见,且关于其治疗的数据稀少。在此,我们希望分享我们治疗一名39岁女性宫颈局部晚期原发性胚胎性横纹肌肉瘤的经验。
患者因经间期出血和性交后出血症状入院6个月。体格检查发现宫颈有一个易碎的息肉样肿物(5厘米×5厘米)突入阴道,子宫活动度良好且大小正常。进行了阴道镜引导下活检,病理诊断为胚胎性横纹肌肉瘤。盆腔磁共振成像显示宫颈体积显著增大,右侧有一个低信号和高信号的软组织肿物,侵犯宫颈间质;肿物大小为5厘米×5厘米,边界清晰,局限于宫颈;阴道壁、宫旁组织或盆腔壁未见明显肿瘤侵犯迹象;盆腔未见肿大淋巴结。根据我们的检查结果,按照横纹肌肉瘤协作组研究标准,该肿瘤分类为IA期,按照国际妇产科联盟2018年标准为IB3期。患者接受了两个疗程的新辅助化疗并获得部分缓解。随后,她接受了腹腔镜根治性子宫切除术、双侧输卵管卵巢切除术和盆腔淋巴结清扫术,术后病理检查未发现危险因素。术后进行了辅助化疗。直到最后一次随访,即完成整个治疗后49个月,患者无疾病复发。
我们的经验表明,对于无生育意愿的成年人大体积宫颈横纹肌肉瘤,新辅助长春新碱、放线菌素D和环磷酰胺化疗后行根治性手术及辅助化疗可能是合理的治疗选择。由于对这种罕见情况进行大规模研究不太可能,进一步的病例报告和系统评价有助于优化成年原发性大体积宫颈横纹肌肉瘤的治疗。