Introduction 

 Primary vulvar malignancy is a rare gynecological neoplasm constituting 5–8% of cases.   

It is the fourth most common gynecological malignancy that usually affects post-menopausal women with a median age of 68 years, but there has been a concerning rise in the incidence of vulvar cancer in younger women, which is often linked to human papillomavirus (HPV) infection.  

The most common form of vulvar cancer is squamous cell carcinoma, which makes up approximately 90% of cases. Less frequently, we encounter melanoma, basal cell carcinoma, and other rarer types.  

 Risk factors 

Several key risk factors contribute to the development of vulvar cancer:  

• Age: As mentioned, vulvar cancer predominantly affects older women, particularly those over 60.  

• HPV Infection: up to 40% of vulvar cancers are related to HPV.  

• Chronic Vulvar Inflammation: Chronic inflammation such as lichen sclerosus increase the likelihood of malignant transformation in the vulva.  

• Smoking: Smokers are at a higher risk for developing various cancers, including vulvar carcinoma.  

• Weakened Immune System: Immunosuppressed individuals, including those with HIV/AIDS or organ transplant recipients, face a greater risk of vulvar cancer.  

• Previous Cancers: A history of cervical, vaginal, or anal cancer also increases the likelihood of vulvar cancer due to shared risk factors like HPV.  

 Local extent 

Vulvar cancer generally begins in the external layers of the vulva, such as the labia majora, labia minora, clitoris, urethra, or perineal area. Most cases (approximately 70%) affect the labia majora area.  

In its early stages, vulvar cancer may present as a painless ulcer or an elevated lesion with a rough or flat surface. Sometimes, it appears as a lump or mass.  

As the disease advances, the tumor can invade adjacent tissues, such as the vagina and perineum, and in more advanced stages, it may affect deeper structures, including the inguinal lymph nodes and pelvic lymph nodes, and eventually spread to distant organs.  

 Spread and metastases 

Vulvar cancer spreads mainly through lymphatic channels. The inguinal lymph nodes are the first site where cancer typically metastasizes. As the tumor progresses, it may spread to the pelvic lymph nodes, and in more advanced stages, to non-regional lymph nodes or distant organs, such as the lungs, liver, or bones.  

• Inguinal Lymph Nodes: These are the first lymph nodes affected in vulvar cancer spread. This occurs in 50-60% of cases.  

• Pelvic Lymph Nodes: If cancer spreads beyond the inguinal nodes, the pelvic lymph nodes (iliac, obturator, sacral) are the next in line to be affected. Their involvement is usually associated with a poorer prognosis.  

• Distant Metastases: Although vulvar cancer typically spreads through the lymphatic system, in rare cases, it can spread hematogenously affecting distant organs. 

 Role of imaging 

Imaging plays a crucial role in the diagnosis, staging, treatment planning, and follow-up of vulvar cancer.  

MRI is the technique of choice to evaluate the local extent and PET-CT for lymph node involvement and metastases detection  

Imaging is critical for treatment planning determining if surgical resection is feasible and planning for radiation therapy for non-surgical tumors or as an adjuvant therapy after surgery.  

It is important to exclude involvement of  urethra, anus, or vagina.  

The exact depth of invasion and whether the tumor has spread to local lymph nodes are key factors in determining the surgical approach and the need for lymphadenectomy.  MRI and CT sacans are used for follow-up and surveillance particularly in patients with positive lymph nodes or in advanced stages. 

Treatment and follow-up 

Treatment of vulvar cancer depends on the stage of the cancer and the extent of the disease:  

• Surgery: Surgery is the primary treatment for vulvar tumors in early stages. This may include a partial or total vulvectomy  
• Radiotherapy: Radiation therapy may be used in cases where surgery is not possible, or when there are positive surgical margins. It is also helpful for treating involved lymph nodes or metastases.  
• Chemotherapy: Chemotherapy is used in more advanced or metastatic vulvar cancer. 

Prognosis 

The prognosis for vulvar cancer largely depends on the stage at diagnosis and the presence of metastases. Early Stages (I and II) have a high survival rate.  Surgical removal with negative margins can cure the cancer in these stages. Advanced Stages (III and IV) becomes less favorable if cancer has spread to the inguinal lymph nodes, and much worse if there is metastasis to distant organs.