Vulvar Cancer: Symptoms, Types, Causes and Treatment

Vulvar cancer is an uncommon yet significant gynecological malignancy that can impact women of all ages, though it most frequently affects those in their postmenopausal years. Despite its rarity, awareness and early detection are crucial, as timely intervention can lead to favorable outcomes. This article provides a comprehensive overview of vulvar cancer, including its symptoms, the different types that exist, underlying causes, and modern approaches to treatment—aimed at empowering patients, families, and healthcare professionals with up-to-date, evidence-based information.

Symptoms of Vulvar Cancer

Vulvar cancer often develops slowly, and its symptoms can be subtle or mistaken for benign conditions. Knowing what to look for can make a crucial difference in early detection and successful treatment.

Symptom	Description	Frequency/Importance	Source(s)
Pruritus	Persistent itching, often chronic	Most common, long-lasting	1 4 5 8 15
Bleeding	Unusual vulvar bleeding or spotting	Common	1 8
Pain	Burning, soreness, or pain in the vulva	Common	1 2 5 8
Discharge	Unusual discharge from vulvar area	Less common	1
Lump/Ulcer	Noticed lump, sore, or non-healing ulcer	Common, warrants biopsy	8 9 15
Dysuria	Pain or difficulty with urination	Occasional	1
Irritation	General discomfort or irritation	Frequently reported	2

Table 1: Key Symptoms

Common Early Symptoms

Vulvar cancer often presents first with pruritus—persistent itching—that can last for months or even years before more obvious symptoms appear. Many women initially mistake this for a simple dermatological issue or infection, which can delay diagnosis and treatment 1 4 5 15.

Other early signs include:

• Burning, soreness, or ongoing pain in the vulva
• A change in the appearance of the vulvar skin, such as discoloration, thickening, or a visible lump

Progressive and Advanced Symptoms

As the disease progresses, more overt symptoms may develop:

• A non-healing ulcer, sore, or growth on the vulva, sometimes with raised or warty edges
• Unusual bleeding not related to menstruation
• Persistent vulvar discharge
• Painful urination or sexual intercourse

It’s essential to note that any persistent vulvar lesion—especially if it fails to heal—should be biopsied to rule out cancer 8 9 15.

Impact on Quality of Life

Chronic symptoms like pain and itching can significantly disrupt daily activities and contribute to psychological distress, including anxiety and depression 3. These symptoms may also affect sexual well-being and relationships, highlighting the importance of holistic care.

Types of Vulvar Cancer

Vulvar cancer is not a single disease but a group of malignancies that arise from different cell types within the vulva. Understanding these distinctions is key for diagnosis and treatment planning.

Type	Key Features	Prevalence	Source(s)
Squamous Cell Carcinoma (SCC)	Starts in squamous cells; HPV-linked and non-HPV forms	~90–95%	1 7 9 14 15
Melanoma	Arises from pigment cells; aggressive	~2–10%	6 9 14
Basal Cell Carcinoma	Slow-growing, rare	<2%	9 14
Paget Disease	Intraepithelial carcinoma	Rare	9 14
Bartholin Gland Adenocarcinoma	Originates in Bartholin gland	Very rare	14
Sarcoma	Connective tissue origin	Extremely rare	14
Verrucous Carcinoma	Warty, slow-growing SCC variant	Very rare	14

Table 2: Major Types of Vulvar Cancer

Squamous Cell Carcinoma (SCC)

• Prevalence: By far the most common, accounting for approximately 90–95% of all vulvar cancers 1 7 9 14 15.
• Subtypes: Includes keratinizing, basaloid, warty, and microinvasive forms. SCC can be further categorized based on HPV association (HPV-dependent and HPV-independent) 1 7 10 11.
• Age Distribution: HPV-related SCCs tend to occur in younger women, while HPV-independent forms are seen more in older, postmenopausal women 1 5 14 15.

Melanoma

• Prevalence: The second most common type, comprising 2–10% of vulvar malignancies 6 9 14.
• Characteristics: Often aggressive, with a poorer prognosis than SCC. Median survival is significantly lower, particularly for advanced-stage disease 6.

Other Rare Types

• Basal Cell Carcinoma: Slow-growing, less likely to metastasize, very rare 9 14.
• Paget Disease: Carcinoma in situ of the vulvar epidermis, may be associated with underlying adenocarcinoma 9 14.
• Bartholin Gland Adenocarcinoma, Sarcoma, Verrucous Carcinoma: All extremely rare but important to recognize for tailored management 14.

Importance of Accurate Diagnosis

Correct identification of the specific type is essential, as treatment approaches and prognosis can vary significantly. For example, vulvar melanomas are managed differently than SCCs and tend to require more aggressive therapy 6 9 14.

Causes of Vulvar Cancer

Vulvar cancer develops due to a complex interplay of risk factors and underlying biological mechanisms. While some causes are well understood, research continues to uncover new insights.

Cause/Risk Factor	Description	Relative Risk	Source(s)
HPV Infection	High-risk human papillomavirus types, esp. 16/18	Major, especially in young women	1 5 7 10 11 14 15
Age	Risk increases with age, especially postmenopausal	Major in older women	1 8 14 15
Smoking	Synergistic with HPV, increases risk	Notable in younger women	5 14 15
Immunodeficiency	Immunosuppression (e.g., HIV, transplant)	Increased risk	14
Chronic Inflammation	Lichen sclerosus, lichen planus	Associated with non-HPV SCC	1 5 14
Sexual Behavior	Early intercourse, multiple partners, STIs	Raises HPV exposure	5 11 14 15

Table 3: Key Causes and Risk Factors

Human Papillomavirus (HPV) Infection

High-risk HPV types are strongly implicated in the development of vulvar squamous cell carcinoma, especially among younger women 1 5 7 10 11 14 15. Key points include:

• HPV 16 and 18 are the most common oncogenic strains.
• HPV-associated SCC is rising, particularly in women over 50, likely reflecting historic exposure and changing sexual behaviors 11.
• HPV vaccination is expected to reduce incidence over time.

Non-HPV-Related Pathways

A significant subset of vulvar cancers—especially in older women—develop independently of HPV:

• Chronic inflammatory conditions such as lichen sclerosus and lichen planus are associated with increased risk of non-HPV SCC 1 5 14.
• Immunodeficiency (due to HIV infection or immunosuppressive therapy) raises the risk of both HPV-associated and independent vulvar cancers 14.

Lifestyle and Behavioral Risk Factors

Several modifiable factors increase risk, particularly for the HPV-related pathway:

• Early onset of sexual activity
• Multiple sexual partners
• History of sexually transmitted infections
• Cigarette smoking acts synergistically with HPV to increase risk, particularly in younger women 5 14 15.

Age and Demographics

• Age remains the most significant risk factor, with the majority of vulvar cancers diagnosed in women over 60 1 8 14 15.
• However, rising rates in younger women are attributed to increased HPV prevalence and lifestyle factors 11.

Treatment of Vulvar Cancer

Treatment for vulvar cancer has evolved remarkably, shifting toward more individualized and less invasive approaches. Multidisciplinary care and patient-centered planning are key to optimizing outcomes and quality of life.

Treatment	Approach/Indication	Key Considerations	Source(s)
Surgery	Mainstay for most stages; from local excision to radical vulvectomy	Lymph node assessment vital; less radical for early disease	1 7 8 13 14 15
Sentinel Node Biopsy	Minimally invasive lymph node evaluation	Reduces morbidity	1 13 14 15
Radiotherapy	Adjuvant, neoadjuvant, or primary for advanced/inoperable tumors	Often combined with chemo	8 12 13 14
Chemotherapy	For advanced, recurrent, or palliative cases	Combined with radiation in some protocols	8 12 14 15
Multidisciplinary Care	Involvement of gynecology, oncology, radiology, psychology	Improves outcomes, QOL	3 12 14

Table 4: Modern Treatment Approaches

Surgical Management

Surgery remains the cornerstone of vulvar cancer treatment 1 7 8 13 14 15:

• Early-stage disease: Radical wide local excision with adequate margins is often sufficient.
• Lymph node assessment: Sentinel lymph node biopsy (SLNB) is now preferred for many early-stage cancers, sparing patients the morbidity of full lymphadenectomy 1 13 14 15.
• Advanced disease: May require more extensive surgery, sometimes including vulvectomy and inguinofemoral lymph node dissection.

Radiotherapy

Radiation therapy has an increasingly important role, particularly for:

• Locally advanced tumors
• Patients unfit for surgery
• Adjuvant treatment when surgical margins are inadequate or there is lymph node involvement
• Neoadjuvant use to shrink tumors prior to surgery 8 12 14

Radiation may be combined with chemotherapy for optimal effect 12.

Chemotherapy

Chemotherapy is mainly reserved for:

• Locally advanced or recurrent disease
• Palliative care in metastatic cases
• Concurrent use with radiation therapy for radiosensitization 8 12 14 15

Multidisciplinary and Supportive Care

Given the profound psychosocial and sexual impact of vulvar cancer, an integrated care model is critical:

• Involvement of psychologists, physiotherapists, and sexual health specialists improves quality of life and addresses unmet needs 3 12 14.
• Careful follow-up is necessary for early detection of recurrences and management of long-term complications.

Prognosis

• Early detection and individualized treatment lead to high cure rates.
• Lymph node involvement is the most important prognostic factor 1 6 13.
• HPV-positive tumors tend to have a better prognosis than HPV-negative ones 10.

Conclusion

Vulvar cancer, though rare, is a serious disease with a profound impact on physical and emotional health. Early recognition of symptoms, accurate diagnosis of the cancer type, and understanding of underlying causes are all pivotal in guiding effective treatment. Advances in surgical techniques, radiotherapy, and supportive care have greatly improved outcomes and quality of life for affected women.

Key Takeaways:

• Persistent vulvar itching, pain, or lesions warrant timely medical evaluation and biopsy.
• The most common type is squamous cell carcinoma, with HPV infection playing a major role in many cases.
• Risk factors include age, HPV infection, smoking, chronic inflammatory conditions, and immunodeficiency.
• Treatment is increasingly individualized, with a shift toward less invasive surgery, sentinel node evaluation, and multidisciplinary care.
• Early-stage disease has a good prognosis; lymph node status is the most important predictor of survival.
• Integrated psychosocial support is essential for optimal recovery and quality of life.

Empowering women and healthcare providers with knowledge about vulvar cancer is the first step toward earlier detection, better treatment, and improved outcomes.