Cancer of the Vulva and Vagina

  • Lauren KrillEmail author
  • Leslie M. Randall


Vaginal and vulva carcinomas are uncommon gynecologic neoplasms. This chapter provides a synopsis of the available data on the epidemiology, diagnosis, staging, pathology, treatment, and prognosis of vaginal and vulvar cancers from the literature. Effective treatment for these malignancies requires utilization of multiple therapeutic modalities including radical surgery, radiation and chemotherapy. An overview of the management options as well as the evidence that has shaped contemporary practice is presented here. Due to the rarity of these diseases, optimal care requires access to specialty trained surgeons and multidisciplinary specialists whenever possible. Patients should be referred to tertiary centers to gain access to any open clinical trials and practitioners with the most experience in the specialized techniques used to effectively treat vulvar and vaginal cancers. This chapter also includes a brief summary of the management of preinvasive lesions of the vulva and vagina.


Vaginal cancer Vulvar carcinoma Recurrence Surgical management Radiation Treatment complications Prognostic factors Vaginal and vulvar intraepithelial neoplasia 



American Joint Committee on Cancer


Clear cell adenocarcinoma


Deep vein thrombosis


External beam radiation




Gracilis myocutaneous flap


Gynecologic Oncology Group


Human papillomavirus


International Federation of Gynecology and Obstetrics


Inguinofemoral lymphadenectomy


Intensity-modulated radiotherapy


International Society for the Study of Vulvar Diseases


Lactate dehydrogenase




Magnetic resonance imaging


Pulmonary embolism


Positron emission tomography


Radiation therapy


Squamous cell carcinomas


Surveillance Epidemiology End Report


Sentinel lymph node


Sentinel lymph node biospy


Stereotactic radiosurgery


TNM classification of malignant tumors


United States of America


Vaginal intraepithelial neoplasia


Vulvar intraepithelial neoplasia


World Health Organization


  1. 1.
    Society AC. American Cancer Society Key Statistics Detailed guide to vaginal and vulvar cancer, 2013.
  2. 2.
    Kosary CL. Cancer of the Vulva. In: Ries LAG, Young JL, Keel GE, Eisner MP, Lin YD, Horner M-J, editors. SEER Survival monograph: cancer survival among adults: U.S. SEER Program, 1988–2001, Patient and tumor characteristics. National Cancer Institute, SEER Program, NIH Pub. No. 07-6215, Bethesda, MD, 2007.Google Scholar
  3. 3.
    Trimble CL, Hildesheim A, Brinton LA, Shah KV, Kurman RJ. Heterogeneous etiology of squamous carcinoma of the vulva. Obstet Gynecol. 1996;87(1):59–64. PubMed PMID: 8532267.PubMedCrossRefGoogle Scholar
  4. 4.
    Sideri M, Jones RW, Wilkinson EJ, Preti M, Heller DS, Scurry J, et al. Squamous vulvar intraepithelial neoplasia: 2004 modified terminology, ISSVD Vulvar Oncology Subcommittee. J Reprod Med. 2005;50(11):807–10. PubMed PMID: 16419625.PubMedGoogle Scholar
  5. 5.
    Hacker NF. Revised FIGO staging for carcinoma of the vulva. Int J Gynaecol Obstet. 2009;105(2):105–6. PubMed PMID: 19329116.PubMedCrossRefGoogle Scholar
  6. 6.
    Berek JS, Hacker NF. Berek & Hacker’s gynecologic oncology. 5th ed. Philadelphia: Wolters Kluwer/Lippincott Williams & Wilkins Health; 2010. xv, 895 p.Google Scholar
  7. 7.
    Gonzalez Bosquet J, Magrina JF, Magtibay PM, Gaffey TA, Cha SS, Jones MB, et al. Patterns of inguinal groin metastases in squamous cell carcinoma of the vulva. Gynecol Oncol. 2007;105(3):742–6. PubMed PMID: 17379281.PubMedCrossRefGoogle Scholar
  8. 8.
    Clark Jr WH, Mihm Jr MC. Lentigo maligna and lentigo-maligna melanoma. Am J Pathol. 1969;55(1):39–67. PubMed PMID: 5776171, Pubmed Central PMCID: 2013384.PubMedCentralPubMedGoogle Scholar
  9. 9.
    Chung AF, Woodruff JM, Lewis Jr JL. Malignant melanoma of the vulva: a report of 44 cases. Obstet Gynecol. 1975;45(6):638–46. PubMed PMID: 1143724.PubMedCrossRefGoogle Scholar
  10. 10.
    Breslow A. Thickness, cross-sectional areas and depth of invasion in the prognosis of cutaneous melanoma. Ann Surg. 1970;172(5):902–8. PubMed PMID: 5477666, Pubmed Central PMCID: 1397358.PubMedCentralPubMedCrossRefGoogle Scholar
  11. 11.
    Karlan BY, Bristow RE, Li AJ. Gynecologic oncology: clinical practice and surgical atlas. 1st ed. New York: McGraw-Hill; 2012.Google Scholar
  12. 12.
    Balch CM, Gershenwald JE, Soong SJ, Thompson JF, Atkins MB, Byrd DR, et al. Final version of 2009 AJCC melanoma staging and classification. J Clin Oncol. 2009;27(36):6199–206. PubMed PMID: 19917835, Pubmed Central PMCID: 2793035.PubMedCentralPubMedCrossRefGoogle Scholar
  13. 13.
    Trimble EL, Lewis Jr JL, Williams LL, Curtin JP, Chapman D, Woodruff JM, et al. Management of vulvar melanoma. Gynecol Oncol. 1992;45(3):254–8. PubMed PMID: 1612500.PubMedCrossRefGoogle Scholar
  14. 14.
    Fanning J, Lambert HC, Hale TM, Morris PC, Schuerch C. Paget’s disease of the vulva: prevalence of associated vulvar adenocarcinoma, invasive Paget's disease, and recurrence after surgical excision. Am J Obstet Gynecol. 1999;180(1 Pt 1):24–7. PubMed PMID: 9914572.PubMedCrossRefGoogle Scholar
  15. 15.
    Horowitz IR. Female genital system. In: Wood WC, Skandalakis JE, Staley CA, editors. Berlin: Springer; 2010, pp 637–678.Google Scholar
  16. 16.
    Berman ML, Soper JT, Creasman WT, Olt GT, DiSaia PJ. Conservative surgical management of superficially invasive stage I vulvar carcinoma. Gynecol Oncol. 1989;35(3):352–7. PubMed PMID: 2599471.PubMedCrossRefGoogle Scholar
  17. 17.
    Farias-Eisner R, Cirisano FD, Grouse D, Leuchter RS, Karlan BY, Lagasse LD, et al. Conservative and individualized surgery for early squamous carcinoma of the vulva: the treatment of choice for stage I and II (T1-2 N0-1 M0) disease. Gynecol Oncol. 1994;53(1):55–8. PubMed PMID: 8175023.PubMedCrossRefGoogle Scholar
  18. 18.
    Heaps JM, Fu YS, Montz FJ, Hacker NF, Berek JS. Surgical-pathologic variables predictive of local recurrence in squamous cell carcinoma of the vulva. Gynecol Oncol. 1990;38(3):309–14. PubMed PMID: 2227541.PubMedCrossRefGoogle Scholar
  19. 19.
    Hacker NF, Van der Velden J. Conservative management of early vulvar cancer. Cancer. 1993;71 Suppl 4:1673–7. PubMed PMID: 8431905.PubMedCrossRefGoogle Scholar
  20. 20.
    Stehman FB, Bundy BN, Thomas G, Varia M, Okagaki T, Roberts J, et al. Groin dissection versus groin radiation in carcinoma of the vulva: a Gynecologic Oncology Group study. Int J Radiat Oncol Biol Phys. 1992;24(2):389–96. PubMed PMID: 1526880.PubMedCrossRefGoogle Scholar
  21. 21.
    Levenback CF, Ali S, Coleman RL, Gold MA, Fowler JM, Judson PL, et al. Lymphatic mapping and sentinel lymph node biopsy in women with squamous cell carcinoma of the vulva: a gynecologic oncology group study. J Clin Oncol. 2012;30(31):3786–91. PubMed PMID: 22753905, Pubmed Central PMCID: 3478573.PubMedCentralPubMedCrossRefGoogle Scholar
  22. 22.
    Coleman RL, Ali S, Levenback CF, Gold MA, Fowler JM, Judson PL, et al. Is bilateral lymphadenectomy for midline squamous carcinoma of the vulva always necessary? An analysis from Gynecologic Oncology Group (GOG) 173. Gynecol Oncol. 2013;128(2):155–9. PubMed PMID: 23201592, Pubmed Central PMCID: 3638213.PubMedCentralPubMedCrossRefGoogle Scholar
  23. 23.
    Oonk MH, van Hemel BM, Hollema H, de Hullu JA, Ansink AC, Vergote I, et al. Size of sentinel-node metastasis and chances of non-sentinel-node involvement and survival in early stage vulvar cancer: results from GROINSS-V, a multicentre observational study. Lancet Oncol. 2010;11(7):646–52. PubMed PMID: 20537946.PubMedCrossRefGoogle Scholar
  24. 24.
    Zhang SH, Sood AK, Sorosky JI, Anderson B, Buller RE. Preservation of the saphenous vein during inguinal lymphadenectomy decreases morbidity in patients with carcinoma of the vulva. Cancer. 2000;89(7):1520–5. PubMed PMID: 11013366.PubMedCrossRefGoogle Scholar
  25. 25.
    Dardarian TS, Gray HJ, Morgan MA, Rubin SC, Randall TC. Saphenous vein sparing during inguinal lymphadenectomy to reduce morbidity in patients with vulvar carcinoma. Gynecol Oncol. 2006;101(1):140–2. PubMed PMID: 16364412.PubMedCrossRefGoogle Scholar
  26. 26.
    Geisler JP, Manahan KJ, Buller RE. Neoadjuvant chemotherapy in vulvar cancer: avoiding primary exenteration. Gynecol Oncol. 2006;100(1):53–7. PubMed PMID: 16257042.PubMedCrossRefGoogle Scholar
  27. 27.
    Montana GS, Thomas GM, Moore DH, Saxer A, Mangan CE, Lentz SS, et al. Preoperative chemo-radiation for carcinoma of the vulva with N2/N3 nodes: a gynecologic oncology group study. Int J Radiat Oncol Biol Phys. 2000;48(4):1007–13. PubMed PMID: 11072157.PubMedCrossRefGoogle Scholar
  28. 28.
    Homesley HD, Bundy BN, Sedlis A, Adcock L. Radiation therapy versus pelvic node resection for carcinoma of the vulva with positive groin nodes. Obstet Gynecol. 1986;68(6):733–40. PubMed PMID: 3785783.PubMedGoogle Scholar
  29. 29.
    Kunos C, Simpkins F, Gibbons H, Tian C, Homesley H. Radiation therapy compared with pelvic node resection for node-positive vulvar cancer: a randomized controlled trial. Obstet Gynecol. 2009;114(3):537–46. PubMed PMID: 19701032.PubMedCrossRefGoogle Scholar
  30. 30.
    Moore DH, Ali S, Koh WJ, Michael H, Barnes MN, McCourt CK, et al. A phase II trial of radiation therapy and weekly cisplatin chemotherapy for the treatment of locally-advanced squamous cell carcinoma of the vulva: a gynecologic oncology group study. Gynecol Oncol. 2012;124(3):529–33. PubMed PMID: 22079361.PubMedCrossRefGoogle Scholar
  31. 31.
    Helm CW, Hatch KD, Partridge EE, Shingleton HM. The rhomboid transposition flap for repair of the perineal defect after radical vulvar surgery. Gynecol Oncol. 1993;50:164–7.PubMedCrossRefGoogle Scholar
  32. 32.
    John HE, Jessop ZM, Di Candia M, et al. An algorithmic approach to perineal reconstruction – experience from two international centers. Ann Plast Surg. 2013;71:96–102.PubMedCrossRefGoogle Scholar
  33. 33.
    Burke TW, Morris M, Roh MS, et al. Perineal reconstruction using single gracilis myocutaneous flaps. Gynecol Oncol. 1995;57:221–5.PubMedCrossRefGoogle Scholar
  34. 34.
    Fowler JM. Incorporating pelvic/vaginal reconstruction into radical pelvic surgery. Gynecol Oncol. 2009;115:154–61.PubMedCrossRefGoogle Scholar
  35. 35.
    Maggino T, Landoni F, Sartori E, Zola P, Gadducci A, Alessi C, et al. Patterns of recurrence in patients with squamous cell carcinoma of the vulva. A multicenter CTF Study. Cancer. 2000;89(1):116–22. PubMed PMID: 10897008.PubMedCrossRefGoogle Scholar
  36. 36.
    Origoni M, Sideri M, Garsia S, Carinelli SG, Ferrari AG. Prognostic value of pathological patterns of lymph node positivity in squamous cell carcinoma of the vulva stage III and IVA FIGO. Gynecol Oncol. 1992;45(3):313–6. PubMed PMID: 1612509.PubMedCrossRefGoogle Scholar
  37. 37.
    van Seters M, van Beurden M, ten Kate FJ, Beckmann I, Ewing PC, Eijkemans MJ, et al. Treatment of vulvar intraepithelial neoplasia with topical imiquimod. N Engl J Med. 2008;358(14):1465–73. PubMed PMID: 18385498.PubMedCrossRefGoogle Scholar
  38. 38.
    Gurumurthy M, Cruickshank ME. Management of vaginal intraepithelial neoplasia. J Lower Genital Tract Dis. 2012;16(3):306–12. PubMed PMID: 22460272.CrossRefGoogle Scholar
  39. 39.
    Buck HW, Guth KJ. Treatment of vaginal intraepithelial neoplasia (primarily low grade) with imiquimod 5 % cream. J Lower Genital Tract Dis. 2003;7(4):290–3. PubMed PMID: 17051086.CrossRefGoogle Scholar
  40. 40.
    Di Saia PJ, Di Saia PJ, Creasman WT, Di Saia PJ. Clinical gynecologic oncology. 8th ed. Philadelphia, PA: Elsevier/Saunders; 2012. xii, 708 p.Google Scholar
  41. 41.
    Creasman WT. Vaginal cancers. Curr Opin Obstet Gynecol. 2005;17(1):71–6. PubMed PMID: 15711415.PubMedCrossRefGoogle Scholar
  42. 42.
    Hacker NF, Eifel PJ, van der Velden J. Cancer of the vagina. Int J Gynaecol Obstet. 2012;119 Suppl 2:S97–9. PubMed PMID: 22999510.PubMedCrossRefGoogle Scholar
  43. 43.
    Frank SJ, Deavers MT, Jhingran A, Bodurka DC, Eifel PJ. Primary adenocarcinoma of the vagina not associated with diethylstilbestrol (DES) exposure. Gynecol Oncol. 2007;105(2):470–4. PubMed PMID: 17292459.PubMedCrossRefGoogle Scholar
  44. 44.
    Ball HG, Berman ML. Management of primary vaginal carcinoma. Gynecol Oncol. 1982;14(2):154–63. PubMed PMID: 7129211.PubMedCrossRefGoogle Scholar
  45. 45.
    Davis KP, Stanhope CR, Garton GR, Atkinson EJ, O’Brien PC. Invasive vaginal carcinoma: analysis of early-stage disease. Gynecol Oncol. 1991;42(2):131–6. PubMed PMID: 1894171.PubMedCrossRefGoogle Scholar
  46. 46.
    Stock RG, Chen AS, Seski J. A 30-year experience in the management of primary carcinoma of the vagina: analysis of prognostic factors and treatment modalities. Gynecol Oncol. 1995;56(1):45–52. PubMed PMID: 7821847.PubMedCrossRefGoogle Scholar
  47. 47.
    Tjalma WA, Monaghan JM, de Barros LA, Naik R, Nordin AJ, Weyler JJ. The role of surgery in invasive squamous carcinoma of the vagina. Gynecol Oncol. 2001;81(3):360–5. PubMed PMID: 11371123.PubMedCrossRefGoogle Scholar
  48. 48.
    Creasman WT, Phillips JL, Menck HR. The National Cancer Data Base report on cancer of the vagina. Cancer. 1998;83(5):1033–40. PubMed PMID: 9731908.PubMedCrossRefGoogle Scholar
  49. 49.
    Benedetti Panici P, Bellati F, Plotti F, Di Donato V, Antonilli M, Perniola G, et al. Neoadjuvant chemotherapy followed by radical surgery in patients affected by vaginal carcinoma. Gynecol Oncol 2008;111(2):307–311.Google Scholar
  50. 50.
    Chyle V, Zagars GK, Wheeler JA, Wharton JT, Delclos L. Definitive radiotherapy for carcinoma of the vagina: outcome and prognostic factors. Int J Radiat Oncol Biol Phys. 1996;35(5):891–905. PubMed PMID: 8751398.PubMedCrossRefGoogle Scholar
  51. 51.
    Perez CA, Grigsby PW, Garipagaoglu M, Mutch DG, Lockett MA. Factors affecting long-term outcome of irradiation in carcinoma of the vagina. Int J Radiat Oncol Biol Phys. 1999;44(1):37–45. PubMed PMID: 10219792.PubMedCrossRefGoogle Scholar
  52. 52.
    Frank SJ, Jhingran A, Levenback C, Eifel PJ. Definitive radiation therapy for squamous cell carcinoma of the vagina. Int J Radiat Oncol Biol Phys. 2005;62(1):138–47. PubMed PMID: 15850914.PubMedCrossRefGoogle Scholar
  53. 53.
    Shah CA, Goff BA, Lowe K, Peters 3rd WA, Li CI. Factors affecting risk of mortality in women with vaginal cancer. Obstet Gynecol. 2009;113(5):1038–45. PubMed PMID: 19384118, Pubmed Central PMCID: 2746762.PubMedCentralPubMedCrossRefGoogle Scholar
  54. 54.
    Di Donato V, Bellati F, Fischetti M, Plotti F, Perniola G, Panici PB. Vaginal cancer. Crit Rev Oncol Hematol. 2012;81(3):286–95. PubMed PMID: 21571543.PubMedCrossRefGoogle Scholar
  55. 55.
    Thigpen JT, Blessing JA, Homesley HD, Berek JS, Creasman WT. Phase II trial of cisplatin in advanced or recurrent cancer of the vagina: a Gynecologic Oncology Group Study. Gynecol Oncol. 1986;23(1):101–4. PubMed PMID: 3943746.PubMedCrossRefGoogle Scholar
  56. 56.
    Barakat RR, Markman M, Randall M. Principles and practice of gynecologic oncology. 5th ed. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2009. xv, 1072 p., 16 p. of plates p.Google Scholar

Copyright information

© Springer Science+Business Media New York 2015

Authors and Affiliations

  1. 1.Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, Irvine Medical CenterUniversity of CaliforniaOrangeUSA

Personalised recommendations