When an Unexpected Diagnosis Occurs: a Vaginal Premenopausal Sarcoma

Abstract Vaginal cancer is a rare entity. The evidence on its management resides mostly in clinical cases or small case series. Of the histological types, the most frequent is the squamous cell carcinoma, followed by adenocarcinoma. But what to do when identifying an even more infrequent sarcoma in a premenopausal woman? In this study, we describe the case of a 53-year-old woman presenting with metrorrhagia for two months, who was evaluated after an intense episode. A necrotic and ulcerative vaginal swelling was documented and then submitted to biopsy, which revealed a vaginal sarcoma. The patient was referred to radiation therapy with 50 Gy (aiming to control the symptoms and to cause tumor reduction for posterior pelvic exenteration with intraoperative radiotherapy) and developed an extra-pelvic metastization at the end of the treatment, which caused a fast negative outcome. Despite the initial poor prognosis, a chemo-irradiation or primary surgery regimen might have achieved (although with greater side effects) a better survival. This case-report entails a discussion about the strategies to manage vaginal sarcoma in advanced stage and in premenopausal women.

摘要：阴道癌是一种罕见疾病，其诊疗相关证据大多来自临床病例报告或小型病例系列研究。在其组织学亚型中，最常见的为鳞状细胞癌（squamous cell carcinoma），其次为腺癌（adenocarcinoma）。但当在绝经前女性体内检出更为罕见的阴道肉瘤时，应采取何种诊疗策略？本研究报告1例53岁女性病例：该患者因经间期出血（metrorrhagia）持续2个月，于一次急性发作后就诊；检查发现阴道存在坏死溃疡性肿物，活检病理证实为阴道肉瘤。患者接受了50 Gy（戈瑞）的放射治疗，旨在控制症状、缩小肿瘤，以择期行联合术中放疗（intraoperative radiotherapy）的盆腔廓清术（pelvic exenteration）；但治疗结束时患者出现盆腔外转移，病情快速恶化。尽管初始预后不佳，但采用放化疗联合治疗或原发性手术方案（尽管不良反应更为显著）或可获得更佳的生存获益。本病例报告旨在探讨晚期阴道肉瘤及绝经前女性阴道肉瘤的诊疗策略。