Cojocaru Elena, Palahepitiva Gamage Githmi, Butler John, Barton Desmond P, Thway Khin, Fisher Cyril, Messiou Christina, Miah Aisha B, Zaidi Shane, Gennatas Spyridon, Benson Charlotte, Huang Paul, Jones Robin L
Sarcoma Unit, The Royal Marsden Hospital, 203 Fulham Rd, London SW3 6JJ, UK.
Royal College of Surgeons in Ireland, Ireland.
Gynecol Oncol Rep. 2021 Mar 2;36:100737. doi: 10.1016/j.gore.2021.100737. eCollection 2021 May.
Ovarian sarcomas account for 1% of all ovarian malignancies and amongst these, primary ovarian leiomyosarcoma is the rarest subtype. Primary ovarian leiomyosarcoma has a very poor prognosis, with less than 20% of patients being alive at 5 years. Only a few cases have been published in the literature and there is very limited knowledge on the clinical behaviour and optimal management of these tumours. We have performed a retrospective analysis of a prospectively maintained database to identify all primary ovarian leiomyosarcoma diagnosed and treated at the Royal Marsden NHS Foundation Trust between 1998 and 2020. Sixteen patients were identified from our database and fifteen were eligible for the analysis. Twelve patients presented with localized disease and underwent initial surgery and three patients had metastatic disease at presentation. Recurrence-free survival post-surgery was 16 months. Eight patients received first-line chemotherapy and four patients received second-line chemotherapy. Two patients had indolent metastatic disease and benefited from local therapies only. The median overall survival in the metastatic setting in our cohort was 51 months, which is consistent with previously published cases. Primary ovarian leiomyosarcoma is an extremely rare malignancy with a poor prognosis. This study is the largest case series of primary ovarian leiomyosarcoma published to date, providing clinically important information regarding survival and metastatic rate as well as treatment outcomes in the metastatic setting.
卵巢肉瘤占所有卵巢恶性肿瘤的1%，其中原发性卵巢平滑肌肉瘤是最罕见的亚型。原发性卵巢平滑肌肉瘤预后极差，5年生存率不到20%。文献中仅报道了少数病例，对于这些肿瘤的临床行为和最佳治疗方案的了解非常有限。我们对一个前瞻性维护的数据库进行了回顾性分析，以确定1998年至2020年期间在皇家马斯登国民保健服务信托基金会诊断和治疗的所有原发性卵巢平滑肌肉瘤病例。从我们的数据库中识别出16例患者，其中15例符合分析条件。12例患者表现为局限性疾病，接受了初始手术，3例患者初诊时已有转移性疾病。术后无复发生存期为16个月。8例患者接受了一线化疗，4例患者接受了二线化疗。2例患者有惰性转移性疾病，仅从局部治疗中获益。我们队列中转移性疾病患者的中位总生存期为51个月，这与之前发表的病例一致。原发性卵巢平滑肌肉瘤是一种极其罕见的恶性肿瘤，预后较差。本研究是迄今为止发表的最大规模的原发性卵巢平滑肌肉瘤病例系列，提供了有关生存率、转移率以及转移性疾病治疗结果的重要临床信息。
