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Comprehensive management of vulvovaginal cancers. ​

文献信息 ​

DOI10.3322/caac.70014
PMID40377134
期刊CA: a cancer journal for clinicians
影响因子232.4
JCR 分区Q1
发表年份2025
被引次数2
关键词外阴阴道癌，外阴阴道黑色素瘤，外阴阴道肉瘤
文献类型Journal Article, Review
ISSN0007-9235
页码410-435
期号75(5)
作者Angélica Nogueira-Rodrigues, Maaike H M Oonk, Domenica Lorusso, Brian Slomovitz, Mario M Leitão, Glauco Baiocchi

一句话小结 ​

外阴和阴道癌是一种罕见且主要影响老年女性的恶性肿瘤，尤其与人乳头瘤病毒（HPV）感染相关，外阴癌的复发率较高且系统治疗选择有限。研究强调了改善治疗效果、参与临床试验的重要性以及HPV疫苗接种对降低发病率的潜在影响，突显了对这一领域持续关注的必要性。

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外阴阴道癌 · 外阴阴道黑色素瘤 · 外阴阴道肉瘤

摘要 ​

外阴和阴道癌是一种罕见的恶性肿瘤，外阴癌的发病率为每10万名女性中有2.7例，主要影响60岁以上的女性，尽管在年轻人群中也观察到发病率上升。约90%的外阴癌为鳞状细胞癌，且通常与人乳头瘤病毒（HPV）感染有关。阴道癌占所有女性癌症的比例不足1%，同样呈现出与HPV相关的趋势。本综述阐述了外阴和阴道癌的病因、组织病理学及治疗策略，包括癌症和外阴阴道黑色素瘤及肉瘤。外阴癌的主要治疗方式为手术干预，涉及肿瘤切除及腹股沟-股骨淋巴结分期。对于局部晚期外阴癌，建议在需要进行切除性手术时进行化学放疗。诊断后2年内的复发率范围为12%至37%。不幸的是，针对复发或转移性疾病的系统治疗十分有限，5年生存率约为20%。目前的证据主要来源于回顾性研究或小规模的二期临床试验，或者是根据宫颈癌的治疗进行推断。强烈建议参与临床试验，并及时获得最佳支持性护理，以减轻局部进展对生活质量的影响。此外，治疗对身体形象和性方面的心理社会影响也需要仔细考虑。未来的HPV疫苗接种倡议可能会降低癌症发病率，尽管这种疫苗接种的显著效果需要数十年才能显现，这突显了提高外阴和阴道癌治疗效果和减少发病率的迫切需要。

英文摘要 ​

Vulvar and vaginal cancers represent rare malignancies, with an incidence of 2.7 per 100,000 women for vulvar cancer, predominantly affecting women older than 60 years, although rising rates are observed in younger demographics. Approximately 90% of vulvar cancers are squamous cell carcinoma and frequently are associated with human papillomavirus (HPV) infection. Vaginal cancer, constituting less than 1% of all female cancers, similarly exhibit HPV-related trends. This review delineates the etiology, histopathology, and treatment strategies for carcinomas and vulvovaginal melanomas and sarcomas. Surgical intervention remains the primary treatment modality for vulvar cancer, involving tumor resection and inguinofemoral lymph node staging. For locally advanced vulvar carcinoma, chemoradiation is advised when exenterative surgery would be indicated. Recurrence rates within 2 years after diagnosis range from 12% to 37%. Unfortunately, systemic treatments for recurrent or metastatic disease are limited, with 5-year survival rates at approximately 20%. Current evidence primarily derives from retrospective studies or small phase 2 trials or otherwise is extrapolated from the treatment of cervical cancer. Enrollment in clinical trials is strongly advocated, along with prompt access to best supportive care to mitigate the effect of locoregional progression on quality of life. Moreover, the psychosocial implications of treatment on body image and sexuality necessitate careful consideration. Future HPV vaccination initiatives may reduce cancer incidence, although significant effects of such vaccination will manifest over decades, underscoring the urgent need to enhance treatment efficacy and minimize morbidity in vulvar and vaginal cancers.

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主要研究问题 ​

  1. 如何评估HPV疫苗接种对减少外阴和阴道癌发病率的长期影响？
  2. 在外阴癌的治疗中，手术与化放疗的结合使用对患者生存率的影响如何？
  3. 对于复发或转移性外阴癌，现有的系统治疗方法有哪些具体的局限性？
  4. 在外阴癌患者中，如何有效管理治疗带来的身体形象和性健康问题？
  5. 针对年轻女性的外阴癌病例，是否需要制定不同的筛查和治疗策略？

核心洞察 ​

研究背景和目的 ​

阴道和外阴癌是一类罕见的恶性肿瘤，外阴癌的发病率为每10万女性中约2.7例，主要影响60岁以上女性，但在年轻人群中的发病率也在上升。约90%的外阴癌为鳞状细胞癌，且常与人乳头瘤病毒（HPV）感染相关。该文献旨在概述外阴和阴道癌的病因、组织病理学及治疗策略。

主要方法/材料/实验设计 ​

该文献通过回顾性分析现有文献，汇总了外阴和阴道癌的临床管理策略，强调了手术、放疗和化疗的作用。

Mermaid diagram

关键结果和发现 ​

  1. 病因：外阴癌的主要病因包括HPV感染、吸烟和免疫抑制。约18%-52%的外阴癌与HPV相关，HPV阳性病例预后较好。
  2. 组织病理学：外阴癌以鳞状细胞癌为主，阴道癌同样以鳞状细胞癌为主，且存在多种亚型。
  3. 治疗策略：
    • 手术：手术切除是外阴癌的主要治疗方法，通常包括肿瘤切除和淋巴结分期。对于局部晚期患者，建议化放疗。
    • 放疗：用于术后或晚期患者，以降低复发风险。
    • 化疗：复发或转移性疾病的治疗选择有限，五年生存率约为20%。
  4. 临床试验：建议患者参与临床试验，以获取更好的支持性护理。

主要结论/意义/创新性 ​

外阴和阴道癌的管理面临多重挑战，包括治疗的高发病率和复发率。尽管手术和化放疗是主要治疗手段，但对于转移或复发性疾病的治疗仍需进一步研究。HPV疫苗接种的推广可能有助于降低癌症发生率，强调了提升治疗效果和降低并发症的重要性。

研究局限性和未来方向 ​

  • 局限性：目前的数据主要来源于回顾性研究或小规模的临床试验，缺乏大规模前瞻性研究。
  • 未来方向：需要开展更多针对外阴和阴道癌的临床试验，以探索新的治疗策略。同时，需加强对患者的心理支持和身体形象的关注，提升生活质量。

通过对文献的全面分析，该综述为外阴和阴道癌的管理提供了重要的见解和未来研究的方向。

参考文献 ​

  1. How important is the pathological margin distance in vulvar cancer? - G Baiocchi;H Mantoan;L de Brot;L Badiglian-Filho;L Y Kumagai;C C Faloppa;A A B A da Costa - European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology (2015)
  2. Sentinel node biopsy for early-stage melanoma: accuracy and morbidity in MSLT-I, an international multicenter trial. - Donald L Morton;Alistair J Cochran;John F Thompson;Robert Elashoff;Richard Essner;Edwin C Glass;Nicola Mozzillo;Omgo E Nieweg;Daniel F Roses;Harald J Hoekstra;Constantine P Karakousis;Douglas S Reintgen;Brendon J Coventry;He-jing Wang; - Annals of surgery (2005)
  3. Vulvar melanoma: a multivariable analysis of 644 patients. - Valerie E Sugiyama;John K Chan;Jacob Y Shin;Jonathan S Berek;Kathryn Osann;Daniel S Kapp - Obstetrics and gynecology (2007)
  4. Margin status revisited in vulvar squamous cell carcinoma. - N C Te Grootenhuis;A W Pouwer;G H de Bock;H Hollema;J Bulten;A G J van der Zee;J A de Hullu;M H M Oonk - Gynecologic oncology (2019)
  5. Worldwide human papillomavirus genotype attribution in over 2000 cases of intraepithelial and invasive lesions of the vulva. - Silvia de Sanjosé;Laia Alemany;Jaume Ordi;Sara Tous;Maria Alejo;Susan M Bigby;Elmar Armin Joura;Paula Maldonado;Jan Laco;Ignacio G Bravo;August Vidal;Núria Guimerà;Paul Cross;Gerard V Wain;Karl Ulrich Petry;Luciano Mariani;Christine Bergeron;Václav Mandys;Adela Rosa Sica;Ana Félix;Alp Usubutun;Muhieddine Seoud;Gustavo Hernández-Suárez;Andrzej Marcin Nowakowski;Godfrey Wilson;Veronique Dalstein;Monika Hampl;Elena Sachiko Kasamatsu;Luis Estuardo Lombardi;Leopoldo Tinoco;Isabel Alvarado-Cabrero;Myriam Perrotta;Neerja Bhatla;Theodoros Agorastos;Charles F Lynch;Marc T Goodman;Hai-Rim Shin;Halina Viarheichyk;Robert Jach;M O L Eugenia Cruz;Julio Velasco;Carla Molina;Jacob Bornstein;Annabelle Ferrera;Efren Javier Domingo;Cheng-Yang Chou;Adekunbiola F Banjo;Xavier Castellsagué;Michael Pawlita;Belén Lloveras;Wim G V Quint;Nubia Muñoz;F Xavier Bosch; - European journal of cancer (Oxford, England : 1990) (2013)
  6. Therapeutic cancer vaccines: advancements, challenges, and prospects. - Ting Fan;Mingna Zhang;Jingxian Yang;Zhounan Zhu;Wanlu Cao;Chunyan Dong - Signal transduction and targeted therapy (2023)
  7. Vulvar Adenocarcinoma of Intestinal Type: A Case Report of an Uncommon Entity. - Olivier Moscoso;Armando Reques;Adela Saco;Josep Castellví;Natalia R Gómez-Hidalgo;Santiago Ramón Y Cajal;Ángel García - International journal of gynecological pathology : official journal of the International Society of Gynecological Pathologists (2022)
  8. KIT pathway alterations in mucosal melanomas of the vulva and other sites. - Katarina Omholt;Eva Grafström;Lena Kanter-Lewensohn;Johan Hansson;Boel K Ragnarsson-Olding - Clinical cancer research : an official journal of the American Association for Cancer Research (2011)
  9. The importance of the groin node status for the survival of T1 and T2 vulval carcinoma patients. - M P Burger;H Hollema;A G Emanuels;M Krans;E Pras;J Bouma - Gynecologic oncology (1995)
  10. Malignant melanoma of the vulva treated by radical hemivulvectomy. A prospective study of the Gynecologic Oncology Group. - G L Phillips;B N Bundy;T Okagaki;P R Kucera;F B Stehman - Cancer (1994)

引用本文的文献 ​

  1. Comprehensive management of vulvovaginal cancers. - Angélica Nogueira-Rodrigues;Maaike H M Oonk;Domenica Lorusso;Brian Slomovitz;Mario M Leitão;Glauco Baiocchi - CA: a cancer journal for clinicians (2025)
  2. Comparative Evaluation of Three Primary Antibody Clones for p16 Immunohistochemistry in Gynecologic Tumors. - Hiroshi Yoshida;Ayumi Sugitani;Mayumi Kobayashi-Kato;Masaya Uno;Mitsuya Ishikawa - Antibodies (Basel, Switzerland) (2025)

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