Department of Radiology, Emergency County Hospital, Sibiu, Romania;
Rom J Morphol Embryol. 2023 Jan-Mar;64(1):83-88. doi: 10.47162/RJME.64.1.10.
Uterine embryonal rhabdomyosarcoma (uERMS) in adult women is a very rare malignant entity. The study aim was to report a case of adult uERMS and to discuss the implications of histopathological diagnosis on the treatment and prognosis.
We present here the clinicopathological features of a uERMS case in an adult woman. The study has been approved by the institutional Ethics Committee and an informed consent has been obtained (IJB∕CE3005). A 45-year-old woman presented to her gynecologist with intermenstrual bleedings and polypoid cervical mass (initially interpreted as benign polyp). A second biopsy was sent to our Department of Pathology at the Jules Bordet Institute, Brussels, Belgium for revision and was reinterpreted as botryoid-type uERMS. The patient underwent a total hysterectomy. The final pathology confirms a 3 cm cervical ERMS, and a simple surveillance was decided by our multidisciplinary team. Six months later, pelvic magnetic resonance imaging control showed a recurrence in the right pelvic lymph nodes. Multi-drug chemotherapy and radiotherapy were done before surgical resection. Pathological examination of the resected pelvic mass confirmed uERMS recurrence of 60 mm, with large zones of necrosis and the presence of cartilaginous structures. The patient is free of disease 60 months after diagnosis.
Adult uERMS is rare and the pathological examination is the main element for diagnosis and treatment. It is often confused with other benign entities, at least at the time of diagnosis. ERMS should be included in the differential diagnosis of cervical and uterine polyp of adult women. Long-term survival is possible with a multimodal therapy approach.
成人女性的子宫胚胎性横纹肌肉瘤（uERMS）是一种非常罕见的恶性肿瘤。本研究旨在报告一例成人 uERMS 病例，并讨论组织病理学诊断对治疗和预后的影响。
我们在此介绍一例成年女性 uERMS 的临床病理特征。本研究已获得机构伦理委员会的批准，并获得了知情同意（IJB∕CE3005）。一名 45 岁女性因月经间期出血和宫颈息肉样肿块（最初解释为良性息肉）就诊于妇科医生。第二次活检被送到我们位于比利时布鲁塞尔的 Jules Bordet 研究所的病理科进行复查，并重新解释为葡萄状型 uERMS。患者接受了全子宫切除术。最终的病理证实为 3cm 宫颈 ERMS，我们的多学科团队决定进行简单的监测。6 个月后，盆腔磁共振成像检查显示右侧盆腔淋巴结复发。在进行手术切除之前，患者接受了多药化疗和放疗。对切除的盆腔肿块的病理检查证实 uERMS 复发，大小为 60mm，伴有大片坏死区和软骨结构。患者在诊断后 60 个月无疾病。
成人 uERMS 罕见，病理检查是诊断和治疗的主要依据。它常与其他良性实体混淆，至少在诊断时是这样。ERMS 应纳入成年女性宫颈和子宫息肉的鉴别诊断。采用多模式治疗方法可实现长期生存。
