Cancers of the vulva typically occur in women greater than 70 years of age and almost never occur before 40 years old. They account for 3-5% of all gynecological cancers and have shown a connection to smokers and human papilloma virus (HPV).1
Etiology:
There are currently no known causes for vulvar cancer, but there are a variety of factors that can increase ones risk. These include HPV, smoking, vulvar intraepithelial neoplasia, chronic vulvar dystrophies, and condyloma acuminatum.2
Signs & Symptoms:
A mass in the vulva is the most common symptom of vulva CA followed by pruritus, bleeding, and pain.1 However, up to 20% of women will have no symptoms of cancer. Women with advanced disease have local pain, bleeding, and surface drainage form the tumor. The groin lymph nodes or distant sites may present symptomatic metastatic disease.
Diagnostic Procedures:
Complete physical examination of the vulvar and anal area, perineum, vagina, and cervix is necessaryl.2
A Papanicolaou (Pap) smear of the cervix and vagina should be performed.
A examination of the bimanual pelvic is mandatory.
Assessment of the regional lymph nodes is critical.
Radiographic studies include: Chest X-ray, intravenous pyelogram, barium enema, lymphangiogram, CT or MRI scans, and PET-CT Scan.
Other studies include: cystoscopy, proctosigmoidoscopy, exfoliative cytology, colposcopy, and Schiller’s test.
Most invasive lesions are squamous cell and compromise 90% of lesions of the vulva.2 There are two variants of this type, adenosquamous and basaloid carcinoma. Thesevariants rarely metastasize. Other forms are: verrucous carcinoma, basal cell, adenoid cystic, adenocarcinoma, melanoma sarcomas
Lymph node drainage:
Lymph node metastasis is the single most important prognostic factor in vulva cancer.2 The presence of inguinal node metastasis routinely results in a 50% decrease in long-term survival.The labia, fourchette, perineum and prepuce drain to the superficial inguinal and femoral nodes, then anterior to the cribriform plate and fascia lata. Drainage penetrates the cribriform facia to the deep femoral nodes, following the path to the external and common iliac nodes in the pelvis. The glans clitoris typically drains directly into the inguinal and deep femoral nodes. However, some drainage may bypass the superficial femoral nodes and enter directly into the pelvis and connect with the obturator and external iliac nodes.
Metastatic spread:
Primary carcinomas in the vulvar area usually follow a pattern of spread to the regional lymphatic nodes.2Superficial inguinofemoral lymph nodes are involved first, followed by the deep inguinofemoral nodes. Metastasis to the contralateral inguinal or pelvic lymph nodes is unusual without ipsilateral inguinofemoral node metastasis. Metastases without inguinal node involvement is rare; although lesions involving the glans clitoris or urethra theoretically can spread to pelvic lymph nodes through the channels that bypass the inguinal areas. Lymph node involvement is related to tumor size and depth.3 The incidence of inguinal lymph node metastases in surgically staged patients is 6% to 50%, depending on the depth of tumor invasion. Approximately 20% to 30% of patients with histologically proven involvement of the femoral nodes show deep pelvic lymph node involvement if pelvic lymphadenectomy is performed. Hematogenous spreading is unusual and is a manifestation of late disease. The most common metastatic sites are the lung, liver, and bone.
Grading:
Histologic Grade (G)4: GX: Grade cannot be assessed G1: Well differentiated G2: Moderately differentiated G3: Poorly differentiated G4: Undifferentiated
Staging:
Internal Federation of Gynecology and Obstetrics (FIGO) Staging system5
Radiation side effects:
Side Effects of radiotherapy include6
Skin reaction
Fatigue
Diarrhea
Cystitis
Premature menopause
Vaginal dryness
Painful intercourse
Prognosis:
Survival rates for squamous cell carcinoma of the vulva3 Stage Relative 5-Year Survival Rate I 93% II 79% III 53% IV 29%
Survival rates for adenocarcinoma of the vulva Stage Relative 5-Year Survival Rate I 100% II 92% III 74% IV Not available Survival rates for vulvar melanoma Stage Relative 5-Year Survival Rate I 83% II 64% III 35% IV Not available
Treatments:
- Surgery is used to remove vulvar cancer and any involved lymph nodes.3 - Chemotherapy regimens have included various combinations of 5-fluorouracil (5-FU), cisplatin, mitomycin-C, or bleomycin. - Radiation therapy - 45 Gy–60 Gy at 1.8 Gy–2.0 Gy per fraction depending on the extent of the disease.
The glans clitoris typically drains directly into the inguinal and deep femoral nodes. However, some drainage may bypass the superficial femoral nodes and enter directly into the pelvis and connect with the obturator and external iliac nodes.
GX: Grade cannot be assessed
G1: Well differentiated
G2: Moderately differentiated
G3: Poorly differentiated
G4: Undifferentiated
Internal Federation of Gynecology and Obstetrics (FIGO) Staging system5
Stage Relative 5-Year Survival Rate
I 93%
II 79%
III 53%
IV 29%
Survival rates for adenocarcinoma of the vulva
Stage Relative 5-Year Survival Rate
I 100%
II 92%
III 74%
IV Not available
Survival rates for vulvar melanoma
Stage Relative 5-Year Survival Rate
I 83%
II 64%
III 35%
IV Not available
- Chemotherapy regimens have included various combinations of 5-fluorouracil (5-FU), cisplatin, mitomycin-C, or bleomycin.
- Radiation therapy - 45 Gy–60 Gy at 1.8 Gy–2.0 Gy per fraction depending on the extent of the disease.
Radiation field for cancer of the vulva. http://www.aboutcancer.com/vulva_xrt.htm. Accessed June 25, 2013.
Mild to Moderate Morbidity:
- Bladder: 60 Gy - contracture, frequency
- Mulcle: 60 Gy - Fibrosis
- Ovary: 200-300 cGy - Sterilization
- Rectum: 60 Gy - Ulcer, Stricture
- Skin: 55 Gy - Acute and chronic dermatitis
- Uterus: 75 Gy - Stricture
- Femoral Head: 52 Gy - Necrosis
Severe or Fatal Morbidity: