What Is Vulvar Cancer?

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Overview

Vulvar cancer is a rare type of gynecological cancer that affects women. It occurs when abnormal cells in the vulva begin to multiply and grow uncontrollably.

The vulva includes:

  • 2 pairs of lips including the labia minora and labia majora
  • Clitoris
  • Bartholin’s glands1

Vulvar cancer mainly occurs in the inner edges of the labia majora or labia minora. With this in mind, let’s delve further into this article to gain a comprehensive understanding of vulvar cancer where we will explore its types through to its risk factors.

Types of vulvar cancer

Vulvar cancer can be classified based on the type of cell it originates from. There are different types of vulvar cancer including:

Squamous cell carcinoma (SCC): Almost 90% of cases account for this vulvar cancer type.2 Typically begins on the lips of the vulva. There are also various subtypes of SCC according to the American Cancer Society such as: 

Vulval melanoma: This is the second most common vulvar cancer4 It normally starts on the lips of the vulva and clitoris and typically affects women who are no longer on their periods (post-menopause).2

Adenocarcinoma: Accounts for around 8% of cases and normally starts in cells of the Bartholin glands.

Paget disease of the vulva: It is a skin cancer in which adenocarcinoma cells are found in the outermost layer of the vulvar skin. Typically affects post-menopausal women.

Sarcoma: Account for less than 2% of vulvar cancers and are distinguished into angiomyxoma and rhabdomyosarcoma.4 

Stages of vulvar cancer

In the UK, doctors stage cancer using the FIGO system (International Federation of Gynaecology and Obstetrics), from stage 1 to stage 4.2 

Stage 1A: The tumour is small (2 cm or less) and hasn't grown deep into the underlying skin and tissues.

Stage 1B: The tumour is larger (more than 2 cm) and has grown deeper into the underlying skin and tissues (about 1 mm).

 Stage 2: The tumour has spread into nearby tissues but not the lymph nodes.

Stage 3A: The tumour has spread to the upper part of the nearby tissues OR small lymph nodes (less than 5 mm).

Stage 3B: The cancer has spread to 1 or more larger lymph nodes (more than 5 mm)  

Stage 3C: The cancer has spread to the outer capsule of the lymph nodes 

Stage 4A: The cancer has spread to the pelvic bone where the lymph nodes are fixed or it has formed an open sore. 

Stage 4B: The cancer has spread to distant areas of the body beyond the vulva, such as the lungs.2, 5

Causes of vulvar cancer

The exact causes of all vulvar cancers remain unknown, but certain risk factors can elevate the likelihood of developing the disease. 

Risk factors

The following risk factors include:

  • Age: Peaks in women aged over 70

Human papillomavirus (HPV): two types of HPV include; Vulvar intraepithelial neoplasia (VIN) and vulvar nonneoplastic epithelial disorders (VNED)6

  • Smoking: Especially in women with high-risk HPV
  • Weakened immune system: Includes people with HIV and/or organ transplant
  • Lichen sclerosis: Long-term vulval skin condition
  • Genital cancers: Cervical cancer or pre-cancerous changes in the cells of the cervix (CIN)
  • Melanoma or atypical moles: Particularly in women who have had this and a family history

Signs and symptoms of vulvar cancer

Early signs and symptomof vulvar cancer may not be easily noticeable. However the following symptoms could happen;2,7

  • Persistent itching in the vulvar area
  • Pain 
  • Colour changes/thickness of the skin
  • lumps or ulcers on the vulva
  • bleeding that is unrelated to menstruation (periods)
  • Burning sensation when you pass urine

Management and treatment for vulvar cancer

The following types of treatment are used:7

  • Surgery: Most common treatment for vulvar cancer. The types include excision, vulvectomy, and pelvic exenteration
  • Radiation therapy: Uses radiation to kill vulval cancer cells
  • Chemotherapy: Uses drugs to stop the growth of cancer cells. Topical chemotherapy may be applied to the skin in a cream to treat vulvar cancer. However, chemotherapy treatment is dependent upon the type and stage of cancer
  • Immunotherapy: Uses the body’s immune system to fight against cancer cells. Imiquimod is normally used to treat vulvar lesions

Diagnosis of vulvar cancer

If vulvar cancer is suspected based on symptoms or findings during a pelvic exam, further tests may be recommended by your GP. These tests can include

Biopsy: A small sample of tissue is taken from the abnormal area on the vulva and examined under a microscope to check for the presence of cancer cells.8

Imaging tests: CT, PET, and MRI scans may be used to assess the extent of cancer and determine if it has spread to nearby lymph nodes or other organs.9 

Lymph node biopsy: A biopsy may be performed to check for the presence of cancer cells in the lymph nodes.10

FAQs

How can I prevent vulvar cancer

Practice good hygiene, use protection during sex, quit smoking, get the HPV vaccine, and have regular check-ups and cervical screenings.

How common is vulvar cancer

Vulvar cancer is relatively rare, accounting for 0.6% of all cancers in women, compared to other types of cancer.11

When should I see a doctor

See a doctor if you experience unusual symptoms in the vulvar area, such as persistent itching, pain, sores, lumps, or changes in the skin, or if you have concerns about your reproductive health.

Summary

Vulvar cancer is a type of gynecologic cancer that primarily affects the vulva. Early detection and diagnosis are crucial for effective treatment, management, and improving your survival.

References

  1. Vulval cancer - Causes [Internet]. nhs.uk. 2018 [cited 2023 Jun 14]. Available from: https://www.nhs.uk/conditions/vulval-cancer/causes/
  2. What is vulval cancer? [Internet]. [cited 2023 Jun 14]. Available from: https://www.cancerresearchuk.org/about-cancer/vulval-cancer/about
  3. Krissi H, Ben-Shitrit G, Aviram A, Weintraub AY, From A, Wiznitzer A, et al. Anatomical diversity of the female external genitalia and its association with sexual function. European Journal of Obstetrics & Gynecology and Reproductive Biology [Internet]. 2016 Jan [cited 2023 Jun 15];196:44–7. Available from: https://linkinghub.elsevier.com/retrieve/pii/S0301211515004078 
  4. Vulvar cancer | cancer of the vulva [Internet]. [cited 2023 Jun 15]. Available from: https://www.cancer.org/cancer/types/vulvar-cancer.html 
  5. Olawaiye AB, Cotler J, Cuello MA, Bhatla N, Okamoto A, Wilailak S, et al. FIGO staging for carcinoma of the vulva: 2021 revision. Intl J Gynecology & Obste [Internet]. 2021 Oct [cited 2023 Jun 15];155(1):43–7. Available from: https://onlinelibrary.wiley.com/doi/10.1002/ijgo.13880 
  6. Alkatout I, Günther V, Schubert M, Weigel M, Garbrecht N, Jonat W, et al. Vulvar cancer: epidemiology, clinical presentation, and management options. IJWH [Internet]. 2015 Mar [cited 2023 Jun 16];305. Available from: http://www.dovepress.com/vulvar-cancer-epidemiology-clinical-presentation-and-management-option-peer-reviewed-article-IJWH 
  7. Vulvar cancer treatment - nci [Internet]. 2005 [cited 2023 Jun 16]. Available from: https://www.cancer.gov/types/vulvar/patient/vulvar-treatment-pdq 
  8. Royal College of Obstetricians and Gynaecologists [Internet]. Guidelines for the Diagnosis and Management of Vulval Carcinoma. 2023. Available from: https://www.rcog.org.uk/guidance/browse-all-guidance/other-guidelines-and-reports/guidelines-for-the-diagnosis-and-management-of-vulval-carcinoma/ 
  9. Koh WJ, Greer BE, Abu-Rustum NR, Campos SM, Cho KR, Chon HS, et al. Vulvar cancer, version 1. 2017, nccn clinical practice guidelines in oncology. J Natl Compr Canc Netw [Internet]. 2017 Jan [cited 2023 Jun 16];15(1):92–120. Available from: https://jnccn.org/doi/10.6004/jnccn.2017.0008 
  10. Röttger M, Hertel H, Kaukemüller L, Brodowski L, Flentje M, Hillemanns P, et al. Sentinel lymph node biopsy in vulvar cancer: status, level of knowledge, and counseling in outpatient setting. Arch Gynecol Obstet [Internet]. 2020 Oct [cited 2023 Jun 16];302(4):1001–7. Available from: https://link.springer.com/10.1007/s00404-020-05701-4 
  11. Vulvar cancer [Internet]. 2021 [cited 2023 Jun 16]. Available from: https://www.hopkinsmedicine.org/health/conditions-and-diseases/vulvar-cancer

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This content is purely informational and isn’t medical guidance. It shouldn’t replace professional medical counsel. Always consult your physician regarding treatment risks and benefits. See our editorial standards for more details.

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