Iavazzo Christos, Gkegkes Ioannis D, Vrachnis Nikolaos
IASO Maternity Hospital, Athens, Greece.
1st Department of Surgery, General Hospital of Attica "KAT", Athens, Greece.
Eur J Obstet Gynecol Reprod Biol. 2014 May;176:1-4. doi: 10.1016/j.ejogrb.2014.02.013. Epub 2014 Feb 20.
Vulval sarcomas are an extremely rare type of soft tissue tumor, the principal properties of which are unpredictable symptoms and frequent recurrences. This review discusses the management options specific for cases of vulval epithelioid sarcoma. The PubMed, Scopus and Cochrane databases were systematically searched and 28 studies met the inclusion criteria for our narrative review. The mean age of the 31 included patients was 31 years (range: 17-84). Local excision (19 out of 31, 61.3%), radical vulvectomy (8 out of 31, 25.8%) and hemivulvectomy (4 out of 31, 12.9%) were the principal surgical treatments. Radiotherapy and chemotherapy were performed in 8 and 5 patients, respectively. Recurrence of the disease was present in 13 out of 31 (42%). The interval to recurrence ranged from 1 to 48 months. The main location of recurrences was the local tissues, the lymph nodes and the lung. The mean period of follow-up was 38.5 months (range: 2-146 months). Cure was considered to have taken place in 19 out of 31 (61.3%) patients; 10 out of 31 (32.6%) died, and 2 out of 31 (6.4%) at the end of follow-up were alive but not considered cured. The first and principal step for the proper treatment of vulval epithelioid sarcomas is awareness of their existence by the specialist involved. Extensive imaging is proposed for staging, while the creation of a national or international register of patients with this malignancy would enable a more consistent approach to its management.
外阴肉瘤是一种极其罕见的软组织肿瘤，其主要特点是症状不可预测且频繁复发。本综述讨论了外阴上皮样肉瘤病例的特定治疗选择。我们系统检索了PubMed、Scopus和Cochrane数据库，有28项研究符合我们叙述性综述的纳入标准。纳入的31例患者的平均年龄为31岁（范围：17 - 84岁）。主要的手术治疗方法包括局部切除（31例中的19例，61.3%）、根治性外阴切除术（31例中的8例，25.8%）和半外阴切除术（31例中的4例，12.9%）。分别有8例和5例患者接受了放疗和化疗。31例中有13例（42%）出现疾病复发。复发间隔时间为1至48个月。复发的主要部位是局部组织、淋巴结和肺部。平均随访时间为38.5个月（范围：2 - 146个月）。31例中有19例（61.3%）患者被认为已治愈；31例中有10例（32.6%）死亡，31例中有2例（6.4%）在随访结束时存活但未被认为治愈。正确治疗外阴上皮样肉瘤的首要和主要步骤是相关专科医生对其存在的认识。建议进行全面的影像学检查以进行分期，而建立这种恶性肿瘤患者的国家或国际登记册将有助于采取更一致的管理方法。
