Vulvar Cancer

Vulvar Cancer
By: Suzanne Garcia, CRNP, BSN
Johns Hopkins School of Medicine & Baltimore City Health Department

Vulvar cancer includes cancer of the mons pubis, labia majora, labia minora, clitoris, vestibular bulb, and greater vestibular glands. Geographically, it’s most common in Europe, North and South America, and Oceania.¹ Women between the ages of 60 and 80 years old are more likely to develop vulvar cancer; however, it may occur in younger women as well.

The etiology of the disease process is different in these two groups. In younger women, especially those who smoke, have a history of sexually transmitted infections, are immunodeficient, or have a low socioeconomic status are more like to have HPV associated vulvar cancer.¹  These women experience the precursor lesion, vulvar intraepithelial neoplasia (VIN) that is HPV dependent and seldom progresses to squamous cell carcinoma (SCC).² Women over 60 years old typically develop VIN that is HPV independent and progresses to warty/basaloid SCC.² HPV independent vulvar SCC arises from lichen sclerosus, lichen planus, or another form of chronic dermatitis.³ From inflammation, to cellular atypia with advancement to VIN and squamous cell carcinoma¹, SCC is usually diagnosed early and may present as a mass, scaly patch, plaque, pruritis, or ulcer.³

Although women between 60-80 years old typically develop HPV independent neoplasia, recommendations for examination and diagnosis include vaginal and cervical colposcopy³ because HPV is present in 86% of precancerous changes in the vulva.¹ Cystoscopy and/or proctoscopy may be indicated for invasive disease.³

Surgery is the mainstay of treatment for early stage disease.³ Evaluation of lymph nodes is necessary if the lesion is >1mm deep, or ≥2 cm from vulvar midline. Furthermore, biopsy of the sentinel node is warranted according to Wohlmuth and Wohlmuth-Wieser (2019) “. . . if the tumor is unifocal, has a diameter of less than 4 cm, and the lymph nodes are clinically negative.” ^3(p1260)

Nodal disease is a predictor of outcome. If the sentinel node is positive, treatment with external beam radiation therapy (EBRT), and possibly chemotherapy with dissection of the inguinal node is preferred.³ Although these patients are less likely to have progressive disease, involvement of at least one node drops the 3-year overall survival rate to 56.2% from the 90% of negative nodal disease per the retrospective AGO-CaRE-1-multi center study. Additionally, the five-year recurrence rate is 37% treatment completion.⁴

Vulvar cancer is often diagnosed in women over 60, but some younger women may be affected. HPV is a factor in this group, while it typically arises from prolonged inflammation later in life. However, colposcopy is a mainstay of diagnosis in both groups. Nodal involvement forecasts outcomes.

References

1. Merlo, S. (2020). Modern treatment of vulvar cancer. Radiol Oncol, 54(4), 371-376. doi: 2478/raon-2020-0053
2. van der Avoort, I. A. M., Shirango, H., Hoevenaars, B. M., et al. (2006). Vulvar squamous cell carcinoma is a multifactorial disease following two separate and independent pathways. Int J Gynecol Pathol, 25(1), 22-29. doi: 10.1097/01.pgp.0000177646.38266.6a
3. Wohlmuth, C., & Wohlmuth-Wieser, I. (2019). Vulvar malignancies: An interdisciplinary perspective. Journal der Deutschen Dermatologischen Gesellschaft, 1257-1273. doi: 10.1111/ddg.13995
4. Te Grootenhuis, N. C., van der Zee, A. G. J., van Doorn, H. C., et al. (2016). Sentinal nodes in vulvar cancer: Long-term follow-up of the Groningen International. Study on Sentinel nodes in Vulvar cancer (GROINSS-V) I. Gynecol Oncol, 140(1). 8-14. doi: 10.1016/j.ygyno.2015.09.077