Fernández-Ruiz Marta, Pantoja-Garrido Manuel, Frías-Sánchez Zoraida, Rodríguez-Jiménez Inmaculada, Aguilar-Martín María Del Valle
Servicio de Ginecología del Hospital general Santa María del Puerto (Cádiz), España.
Unidad de Gestión Clínica de Obstetricia y Ginecología del Hospital Universitario Virgen Macarena de Sevilla (España).
Rev Colomb Obstet Ginecol. 2019 Dec;70(4):266-276. doi: 10.18597/rcog.3328.
To present the case of a squamous carcinoma in the neovagina of a patient with Mayer-Rokitansky-Küster-Hauser syndrome and review of the literature related to treatment and prognosis of vaginal neoplasms or HPV infection in transgender women or with MRKH syndrome.
A 56-year-old woman consulted to the Hospital Universitario of Sevilla (Spain). During the clinical examination, a exophytic tumor at the bottom of the vagina was found and the biopsy reported squamous carcinoma and positive nucleic acid amplification test for human papilloma (HPV) type 16. A literature search of case reports, case series and observational studies published from 2000 to October 2019 in English and Spanish was performed in Medline via PubMed, with the follow- ing terms: "congenital abnormalities"; "Mullerian aplasia"; "neovagina"; "Vaginal neoplasms"; "Squamous carcinoma"; "HPV infection" was performed.
14 studies were finally included; seven corresponded to squamous cell carcinoma, three to adenocarcinoma and four reported HPV infection only. All of the squamous cell carcinomas were at advanced stages due to local or lymphatic compro- mise and received radiotherapy with concomitant chemotherapy or radical surgery. The prognosis was bad in three of the cases. Patients with adenocarcinoma also presented with advanced lesions due to local extensión and received surgical treatment and two cases received concomitant chemotherapy. Only one patient was followed-up for five years or more. HPV infection is common in women who underwent neovagina reconstruction.
Patients with neovagina are susceptible to develope squamous carcinomas or adenocarcinomas depending if skin or intestinal tissue grafts are used. According to local compromise at the time of diagnosis, radical or combined treatments are required. Which screening strategies for HPV, squamous cell carcinomas and adenocarcinoma is to be investigated.
介绍1例患有迈耶-罗基坦斯基-库斯特-豪泽综合征（Mayer-Rokitansky-Küster-Hauser syndrome，MRKH综合征）患者新阴道发生鳞状细胞癌的病例，并综述与变性女性或患有MRKH综合征的女性阴道肿瘤或人乳头瘤病毒（HPV）感染的治疗及预后相关的文献。
一名56岁女性前往西班牙塞维利亚大学医院就诊。临床检查时，在阴道底部发现一个外生性肿瘤，活检报告为鳞状细胞癌，人乳头瘤病毒16型核酸扩增试验呈阳性。通过PubMed在Medline上检索了2000年至2019年10月以英文和西班牙文发表的病例报告、病例系列和观察性研究，检索词如下：“先天性异常”；“苗勒管发育不全”；“新阴道”；“阴道肿瘤”；“鳞状细胞癌”；“HPV感染”。
最终纳入14项研究；7项为鳞状细胞癌，3项为腺癌，4项仅报告了HPV感染。所有鳞状细胞癌均因局部或淋巴受累而处于晚期，接受了放疗联合化疗或根治性手术。其中3例预后不良。腺癌患者也因局部扩散而出现晚期病变，接受了手术治疗，2例接受了联合化疗。只有1例患者随访了5年或更长时间。HPV感染在接受新阴道重建的女性中很常见。
根据使用的是皮肤还是肠道组织移植，新阴道患者易发生鳞状细胞癌或腺癌。根据诊断时的局部受累情况，需要采取根治性或联合治疗。HPV、鳞状细胞癌和腺癌的筛查策略有待研究。
