Squamous cell carcinoma of the vulva: Medical therapy and prognosis
- Amer Karam, MD
Amer Karam, MD
- Clinical Associate Professor
- Associate Director and Director of Outreach
- Division of Gynecologic Oncology
- Department of Obstetrics and Gynecology
- Stanford University School of Medicine
- Jonathan S Berek, MD, MMS
Jonathan S Berek, MD, MMS
- Laurie Kraus Lacob Professor
- Stanford University School of Medicine
- Fellow, Stanford Distinguished Careers Institute
- Director, Stanford Women's Cancer Center
- Senior Scientific Advisor, Stanford Cancer Institute
- Andrea L Russo, MD
Andrea L Russo, MD
- Assistant Professor
- Harvard Medical School
- Department of Radiation Oncology
- Massachusetts General Hospital
- Section Editors
- Barbara Goff, MD
Barbara Goff, MD
- Section Editor — Gynecologic Oncology
- Director, Gynecologic Oncology
- University of Washington Medical Center
- Arno J Mundt, MD
Arno J Mundt, MD
- Section Editor — Radiation Therapy
- Chairman of Radiation Oncology
- University of California, San Diego
- Don S Dizon, MD, FACP
Don S Dizon, MD, FACP
- Section Editor – Gynecologic Oncology
- Head of Women's Cancers, Lifespan Cancer Institute
- Director of Medical Oncology, Rhode Island Hospital
- Associate Professor of Medicine, Warren Alpert Medical School of Brown University
- Deputy Editors
- Sadhna R Vora, MD
Sadhna R Vora, MD
- Deputy Editor — Oncology
- Instructor in Medicine
- Harvard Medical School
- Sandy J Falk, MD, FACOG
Sandy J Falk, MD, FACOG
- Director, Editorial Relations — UpToDate
- Deputy Editor — Obstetrics, Gynecology and Women's Health
- Instructor of Obstetrics, Gynecology and Reproductive Biology, Part-time
- Harvard Medical School
Vulvar cancer is the fourth most commonly occurring gynecologic cancer in the United States, with approximately 6000 new cases each year . Most vulvar cancers are squamous cell carcinoma (SCC).
This topic discusses the medical treatment and prognosis of women with vulvar SCC, both for those who are receiving adjuvant therapy after surgical treatment and for those who are not surgical candidates. The staging and surgical management of vulvar cancer is discussed elsewhere. (See "Squamous cell carcinoma of the vulva: Staging and surgical treatment".)
The clinical presentation, diagnosis, and pathology of vulvar cancer; the techniques for radical vulvectomy and radiation therapy; and the management of other histologies are also reviewed separately.
●(See "Radical vulvectomy".)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Siegel RL, Miller KD, Jemal A. Cancer Statistics, 2017. CA Cancer J Clin 2017; 67:7.
- Chapman BV, Gill BS, Viswanathan AN, et al. Adjuvant Radiation Therapy for Margin-Positive Vulvar Squamous Cell Carcinoma: Defining the Ideal Dose-Response Using the National Cancer Data Base. Int J Radiat Oncol Biol Phys 2017; 97:107.
- Ignatov T, Eggemann H, Burger E, et al. Adjuvant radiotherapy for vulvar cancer with close or positive surgical margins. J Cancer Res Clin Oncol 2016; 142:489.
- Koh WJ, Greer BE, Abu-Rustum NR, et al. Vulvar Cancer, Version 1.2017, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw 2017; 15:92.
- Chan JK, Sugiyama V, Pham H, et al. Margin distance and other clinico-pathologic prognostic factors in vulvar carcinoma: a multivariate analysis. Gynecol Oncol 2007; 104:636.
- 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; 68:733.
- Parthasarathy A, Cheung MK, Osann K, et al. The benefit of adjuvant radiation therapy in single-node-positive squamous cell vulvar carcinoma. Gynecol Oncol 2006; 103:1095.
- Mahner S, Jueckstock J, Hilpert F, et al. Adjuvant therapy in lymph node-positive vulvar cancer: the AGO-CaRE-1 study. J Natl Cancer Inst 2015; 107.
- Gill BS, Bernard ME, Lin JF, et al. Impact of adjuvant chemotherapy with radiation for node-positive vulvar cancer: A National Cancer Data Base (NCDB) analysis. Gynecol Oncol 2015; 137:365.
- Kunos C, Simpkins F, Gibbons H, et al. Radiation therapy compared with pelvic node resection for node-positive vulvar cancer: a randomized controlled trial. Obstet Gynecol 2009; 114:537.
- Jackson KS, Fankam EF, Das N, et al. Unilateral groin and pelvic irradiation for unilaterally node-positive women with vulval carcinoma. Int J Gynecol Cancer 2006; 16:283.
- Gaffney DK, King B, Viswanathan AN, et al. Consensus Recommendations for Radiation Therapy Contouring and Treatment of Vulvar Carcinoma. Int J Radiat Oncol Biol Phys 2016; 95:1191.
- Berek JS, Heaps JM, Fu YS, et al. Concurrent cisplatin and 5-fluorouracil chemotherapy and radiation therapy for advanced-stage squamous carcinoma of the vulva. Gynecol Oncol 1991; 42:197.
- Wahlen SA, Slater JD, Wagner RJ, et al. Concurrent radiation therapy and chemotherapy in the treatment of primary squamous cell carcinoma of the vulva. Cancer 1995; 75:2289.
- Russell AH, Mesic JB, Scudder SA, et al. Synchronous radiation and cytotoxic chemotherapy for locally advanced or recurrent squamous cancer of the vulva. Gynecol Oncol 1992; 47:14.
- Montana GS, Thomas GM, Moore DH, 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; 48:1007.
- Moore DH, Ali S, Koh WJ, 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; 124:529.
- Shylasree TS, Bryant A, Howells RE. Chemoradiation for advanced primary vulval cancer. Cochrane Database Syst Rev 2011; :CD003752.
- Perez CA, Grigsby PW, Chao C, et al. Irradiation in carcinoma of the vulva: factors affecting outcome. Int J Radiat Oncol Biol Phys 1998; 42:335.
- Lee J, Kim SH, Kim G, et al. Treatment outcome in patients with vulvar cancer: comparison of concurrent radiotherapy to postoperative radiotherapy. Radiat Oncol J 2012; 30:20.
- Balat O, Edwards CL, Verschraegen C, Delclos L. The long term results of radiotherapy with or without surgery in management of advanced vulvar cancer: report of 76 patients. Eur J Gynaecol Oncol 2000; 21:426.
- Hacker NF, Berek JS, Juillard GJ, Lagasse LD. Preoperative radiation therapy for locally advanced vulvar cancer. Cancer 1984; 54:2056.
- Rotmensch J, Rubin SJ, Sutton HG, et al. Preoperative radiotherapy followed by radical vulvectomy with inguinal lymphadenectomy for advanced vulvar carcinomas. Gynecol Oncol 1990; 36:181.
- Kitagawa R, Katsumata N, Shibata T, et al. A randomized, phase III trial of paclitaxel plus carboplatin (TC) versus paclitaxel plus cisplatin (TP) in stage IVb, persistent or recurrent cervical cancer: Japan Clinical Oncology Group study (JCOG0505). J Clin Oncol 2012; 30:Abstr 5006.
- Gonzalez Bosquet J, Magrina JF, Gaffey TA, et al. Long-term survival and disease recurrence in patients with primary squamous cell carcinoma of the vulva. Gynecol Oncol 2005; 97:828.
- Beller U, Sideri M, Maisonneuve P, et al. Carcinoma of the vulva. J Epidemiol Biostat 2001; 6:155.
- Salani R, Backes FJ, Fung MF, et al. Posttreatment surveillance and diagnosis of recurrence in women with gynecologic malignancies: Society of Gynecologic Oncologists recommendations. Am J Obstet Gynecol 2011; 204:466.
- Andreasson B, Moth I, Jensen SB, Bock JE. Sexual function and somatopsychic reactions in vulvectomy-operated women and their partners. Acta Obstet Gynecol Scand 1986; 65:7.
- Green MS, Naumann RW, Elliot M, et al. Sexual dysfunction following vulvectomy. Gynecol Oncol 2000; 77:73.
- Maggino T, Landoni F, Sartori E, et al. Patterns of recurrence in patients with squamous cell carcinoma of the vulva. A multicenter CTF Study. Cancer 2000; 89:116.
- Salom EM, Penalver M. Recurrent vulvar cancer. Curr Treat Options Oncol 2002; 3:143.
- Stehman FB, Bundy BN, Ball H, Clarke-Pearson DL. Sites of failure and times to failure in carcinoma of the vulva treated conservatively: a Gynecologic Oncology Group study. Am J Obstet Gynecol 1996; 174:1128.
- Burger MP, Hollema H, Emanuels AG, et al. The importance of the groin node status for the survival of T1 and T2 vulval carcinoma patients. Gynecol Oncol 1995; 57:327.
- Gadducci A, Cionini L, Romanini A, et al. Old and new perspectives in the management of high-risk, locally advanced or recurrent, and metastatic vulvar cancer. Crit Rev Oncol Hematol 2006; 60:227.
- De Hullu JA, Hollema H, Lolkema S, et al. Vulvar carcinoma. The price of less radical surgery. Cancer 2002; 95:2331.
- Woolderink JM, de Bock GH, de Hullu JA, et al. Patterns and frequency of recurrences of squamous cell carcinoma of the vulva. Gynecol Oncol 2006; 103:293.
- Beller U, Quinn MA, Benedet JL, et al. Carcinoma of the vulva. FIGO 26th Annual Report on the Results of Treatment in Gynecological Cancer. Int J Gynaecol Obstet 2006; 95 Suppl 1:S7.
- Homesley HD, Bundy BN, Sedlis A, et al. Assessment of current International Federation of Gynecology and Obstetrics staging of vulvar carcinoma relative to prognostic factors for survival (a Gynecologic Oncology Group study). Am J Obstet Gynecol 1991; 164:997.
- Landrum LM, Lanneau GS, Skaggs VJ, et al. Gynecologic Oncology Group risk groups for vulvar carcinoma: improvement in survival in the modern era. Gynecol Oncol 2007; 106:521.
- Lee LJ, Howitt B, Catalano P, et al. Prognostic importance of human papillomavirus (HPV) and p16 positivity in squamous cell carcinoma of the vulva treated with radiotherapy. Gynecol Oncol 2016; 142:293.
- PATIENT SELECTION
- ADJUVANT THERAPY
- Node-negative disease
- Node-positive disease
- - Benefit of adjuvant treatment
- - Chemoradiation versus RT alone
- - Extent of radiation treatment
- DISEASE THAT CANNOT BE MANAGED SURGICALLY
- Unresectable, locally advanced disease
- - Primary treatment
- - Management after primary treatment
- Patients who are medically frail
- Distant metastases
- POSTTREATMENT SURVEILLANCE
- Management of recurrence
- - Local recurrence
- - Distant recurrence
- SUMMARY AND RECOMMENDATIONS