Basics
Description
A wide variety of pathological processes can present as a vulvar mass. These can be classified as benign, precancerous, or malignant, although many of these entities are points on a spectrum of disease. For example, lichen sclerosus can lead to vulvar intraepithelial neoplasia (VIN), which can progress to squamous cell carcinoma.
- Benign disease
- Benign tumors can be cystic (mucous cyst, Bartholin 's cyst), anatomic (hernia), or solid (fibroma, lipoma).
- Infections and abscesses can also present as a mass.
- Premalignant disease
- The undifferentiated type of VIN is associated with human papillomavirus (HPV) infection (especially subtypes 16 and 18) and often affects younger women.
- The differentiated type of VIN is often keratinizing and is associated with lichen sclerosis but not with HPV infection.
- Malignant disease
- The most common form of vulvar cancer is squamous cell carcinoma.
Epidemiology
Vulvar cancer is most common in women between the ages of 65 and 75. However, 20% of newly diagnosed patients are under the age of 50.
Incidence
- According to the American Cancer Society, approximately 3,900 vulvar cancers were diagnosed in the US in 2010.
- Among elderly women the annual incidence is 20/100,000.
Prevalence
Vulvar cancer accounts for 0.6% of all cancers in women.
Risk Factors
- HPV infection
- HIV infection
- Advanced age
- Smoking
- Melanoma
- Cervical cancer
- Vulvar or cervical intraepithelial neoplasia
- Lichen sclerosis
General Prevention
- VIN and squamous cell carcinoma are associated with HPV infection. Preventing HPV infection by postponing the age of first intercourse, limiting the number of sexual partners, and immunization with the HPV vaccine may help prevent these diseases.
- Smoking cessation
- Regular gynecological exams including visual inspection of the external genitalia to diagnose and treat early premalignant disease.
Pathophysiology
- The pathological processes underlying vulvar diseases are variable.
- The mechanism of HPV-associated malignant transformation is due to cell cycle augmentation by HPV-encoded proteins.
- Vulvar cancers not related to HPV infection often show spontaneous mutations in a tumor suppressor gene.
Etiology
- HPV infection is associated with VIN, a premalignant finding which can progress to squamous cell carcinoma.
- Vulvar cancer in older women is associated with chronic inflammation (often reported by the patient as chronic itching), leading to squamous cell hyperplasia, which may progress to VIN and squamous cell carcinoma.
Diagnosis
Malignant diseases:
- VIN
- Long history of vulvar pruritus (most common symptom of VIN)
- Affected area is often thicker and lighter in color than the surrounding skin.
- Invasive vulvar cancer
- Vulvar lump or mass (most common sign)
- Vulvar bleeding, discharge, or pain
- Dysuria
History
- Importantly, women with vulvar cancer present to their physician an average of 6 months after symptoms develop. Women should be asked routinely about vulvar itching and discomfort. They should be examined and not assumed to have benign conditions, particularly with persistent symptoms.
- Patients with vulvar disorders often complain of chronic pruritus, pain, burning, irritation, or a palpable mass.
Physical Exam
- Vulvar cancer usually presents as a raised lesion which may be ulcerated or condylomatous.
- Most squamous cell carcinomas occur on the labia majora and are unifocal.
- Mucous cysts are found on the labia minora or at the introitus.
- Bartholin 's cysts are found in the labia minora at 4 and 8 o 'clock. Usually they are pea-sized, but can be quite large when inflamed or infected.
- Skene 's duct cysts are located adjacent to the urethral meatus.
Tests
Imaging
Imaging is generally not useful in identifying or diagnosing tumors of the vulva. However, MRI or CT scan can be used to detect regional lymph node metastasis.
Surgery
- Any suspicious lesion on the vulva should be biopsied using excisional or punch biopsy, which can be done under local anesthesia in the primary care office. Large or highly suspicious lesions may be referred to a gynecologist for biopsy.
- Benign tumors, such as fibromas, lipomas, or hidradenomas, should be excised if they are painful, bleeding, rapidly growing, or cosmetically bothersome.
- Asymptomatic sebaceous cysts can be left untreated. Incision and drainage is indicated for infected cysts.
- A firm nodule on the labia minora can be mistaken for a Bartholin 's cyst when it is actually a Bartholin 's gland carcinoma. Due to the increased risk of this type of carcinoma in women over 40 years old, masses in women of this age group should be biopsied.
- Bartholin 's gland abscesses can be treated by placement of a Word catheter or by marsupialization.
Pathological Findings
Histologically, squamous cell carcinoma is the most common type of vulvar cancer. However, melanoma, basal cell carcinoma, verrucous carcinoma, Paget 's disease, and other types of cancers also occur on the vulva.
Differential Diagnosis
- Benign disease:
- Fibroma (most common benign solid tumor of the vulva)
- Lipoma
- Hidradenoma
- Sebaceous cyst
- Epidermoid cyst
- Pilonidal cyst
- Condyloma acuminatum
- Bartholin 's gland cyst or abscess
- Fistula or abscess due to Crohn 's disease
- Malignant and premalignant disease:
- VIN I, II, III (precancerous lesions)
- Squamous cell carcinoma (most common malignancy of the vulva)
- Melanoma
- Bartholin 's gland carcinoma
- Basal cell carcinoma
- Sarcoma
- Lymphoma
- Endodermal sinus tumor
- Merkel cell carcinoma
- Paget 's disease
Treatment
Surgery
Surgical excision is the treatment of choice for patients with vulvar cancer.
- Vulvar cancers are staged surgically.
- Small primary lesions (<2 cm) with superficial invasion can undergo wide local excision.
- Larger lesions or those with stromal invasion necessitate lymph node evaluation.
- Local recurrences are treated with repeat excision and/or radiation.
Excision of asymptomatic benign lesions should be delayed until after delivery due to increased risk of bleeding. However, diagnostic biopsy and excision for potential malignancy should not be postponed.
Ongoing Care
Follow-Up Recommendations
Patient Monitoring
- Patients with a history of vulvar cancer should be monitored closely for disease recurrence at the excision site as well as metastasis to the inguinal lymph nodes.
- Examine patients for new masses, skin breakdown and/or ulceration.
Prognosis
- 5-year survival rate for Stage I vulvar cancer approaches 90%.
- Early detection and treatment improves survival.
Additional Reading
1 Vulvar cancer. Available at: http://www.cancer.org2Canavan T, Cohen D. Vulvar cancer. Am Fam Physician. 2002;66:1269 " 1274. [View Abstract]3Jhingran A, Russell AH, Seiden MV. Cancers of the cervix, vulva, and vagina. In: Abeloff MD, Armitage JO, Lichter AS, et al., eds. Abeloff 's clinical oncology, 4th ed. Philadelphia, PA: Elsevier, 2008.4O 'Connell TX, Nathan LS, Satmary WA Non-neoplastic epithelial cell disorders of the vulva. Am Fam Physician. 2008;77(3):321 " 326. [View Abstract]5 The 2004 ASGO annual meeting proceedings (post-meeting edition). J Clin Oncol. 2004;22:5028.
Codes
ICD9
- 184.4 Malignant neoplasm of vulva, unspecified site
- 221.2 Benign neoplasm of vulva
- 625.8 Other specified symptoms associated with female genital organs
- 233.32 Carcinoma in situ, vulva
- 624.01 Vulvar intraepithelial neoplasia I [VIN I]
- 624.02 Vulvar intraepithelial neoplasia II [VIN II]
- 616.2 Cyst of Bartholin 's gland
- 616.3 Abscess of Bartholin 's gland
ICD10
- C51.9 Malignant neoplasm of vulva, unspecified
- D28.0 Benign neoplasm of vulva
- N90.3 Dysplasia of vulva, unspecified
- N75.0 Cyst of Bartholin 's gland
- N75.1 Abscess of Bartholin 's gland
SNOMED
- 289477004 mass of vulva (finding)
- 92486005 benign neoplasm of vulva (disorder)
- 363367000 malignant tumor of vulva (disorder)
- 399933001 vulval intraepithelial neoplasia (disorder)
- 57044006 cyst of Bartholin 's gland duct (disorder)
- 67624004 abscess of Bartholin 's gland (disorder)
Clinical Pearls
- Vulvar mass has a broad differential diagnosis, the most worrisome being cancer.
- Any lesion that is increasing in size or has an unusual appearance should be biopsied.
- Patients should be referred to a surgeon for any diagnostic biopsy that cannot be done safely in the office.
- Risk factors for vulvar cancer include smoking, human papillomavirus (HPV) infection, HIV infection, and advanced age.