What every physician needs to know:
Vulvar cancer is an uncommon malignancy accounting for only 0.5% of female cancer and 4.5% of gynecologic malignancies. Approximately 3400 new cases are diagnosed in the united States annually. Most cases occur in post-menopausal women but a bimodal trend toward an earlier age of diagnosis has been observed in recent years. Eighty-five percent of vulvar cancers are squamous carcinomas; melanoma and bartholin’s carcinoma are the next more common.
Are you sure your patient has vulvar cancer? What should you expect to find?
The most common signs and symptoms are a vulvar mass, irritation, pruritus, and bleeding. Delay in diagnosis is common, either affected by patients who ignore their symptoms or physicians who treat vulvar lesions medically without obtaining a histologic diagnosis. Biopsy of any suspicious lesion on the vulva is the hallmark of diagnosis.
Beware of other conditions that can mimic vulvar cancer:
Benign conditions that can mimic vulvar cancer are the vulvar dystrophies (lichen sclerosis and hyperplasia), dysplasia, and condyloma.
Which individuals are most at risk for developing vulvar cancer:
Women with a history of HPV-related disease of the lower genital tract, condyloma, dysplasia, or carcinoma, are at highest risk for vulvar cancer. Several studies have linked the presence of HPV infection in carcinoma and dysplastic tissue of the vulva.
Non-HPV associated risks are related to chronic inflammatory conditions of the vulva. Observational studies have also linked hypertension, diabetes, obesity, and chronic immunosuppression as associated risk factors.
What laboratory and imaging studies should you order to characterize this patient's tumor (i.e., stage, grade, CT/MRI vs PET/CT, cellular and molecular markers, immunophenotyping, etc.) How should you interpret the results and use them to establish prognosis and plan initial therapy?
A thorough clinical assessment of disease status is required before initiating therapy. This begins with a detailed examination of the vulva and surrounding tissues to measure vulvar lesion, examine inguinal lymph nodes, and surrounding vaginal and anal mucosal structures.
Radiographic imaging may include CT (computed tomographic) scan, barium enema, and intravenous pyelogram. Cystoscopy and sigmoidoscopy are useful procedures to determine direct involvement of the bladder or rectum. Although clinical assessment (clinical stage) is utilized to plan treatment (see below), final stage is assigned from surgical and pathologic findings.
Table I. TNM and FIGO staging of vulvar cancer.
Table II. TNM and FIGO staging of vaginal cancer.
What therapies should you initiate immediately i.e., emergently?
Emergent life-threatening bleeding directly from the cancer can be treated with a short course of radiation.
What should the initial definitive therapy for the cancer be?
Treatment of vulvar cancer is predominantly surgical. Radical vulvectomy removes the primary tumor with a wide margin of skin and deep tissue to the urogenital diaphragm. In addition, unilateral or bilateral inguinal lymph node dissection is performed.
Recent studies have examined the role of sentinal lymph node biopsy as a substitute for full inguinal lymphadenectomy and have shown similar outcomes with reduced surgical morbidity. For more advanced tumors (stage III, IV) multimodality therapy combining pelvic radiation (external beam radiation therapy +/- intracavitary brachytherapy) with radiation sensitizing chemotherapy (most commonly cisplatin, but 5FU and mitomycin C also used in recent series), followed by surgical excision, may be the optimal therapy.
Some of these advanced stage patients can also be candidates for more extensive primary surgical excision to include pelvic exenteration. Patients with positive lymph nodes or surgical margins are treated post-operatively with inguinal and pelvic radiation. The following summarizes treatment modality by clinical stage:
Stage IA: (microinvasive < 1mm) – wide local excision
Stage I: (< 2cm) – Radical vulvectomy + unilateral or bilateral inguinal lymphadenectomy**
Stage II: (> 2cm) – Radical vulvectomy + bilateral lymphadenectomy**
Stage III: (tumor of any size with spread to adjacent urethra, vagina, anus or unilateral lymph node metastasis) – Radical vulvectomy + bilateral inguinal lymphadenectomy +/- post-operative radiation; Pre-operative radiation with chemotherapy followed by surgical excision.
Stage IV: (Tumor invades upper vagina, bone, bladder mucosa, rectal mucosa or bilateral lymph node metastasis). Combined modality therapy with radiation and chemotherapy +/- surgical excision.
What should you tell the patient and the family about prognosis?
Five year survival rates by stage are as follows:
Stage I – 90%
Stage II – 80%
Stage III – 60%
Stage IV – 15%
Follow-up surveillance and therapy/management of recurrences.
Patients with vulvar cancer require regular schedule follow-up. For the first 2 years after completion of therapy, examination should be performed every 3-4 months. Physician exams can then be scheduled at 6 month intervals from years 3-5. Interval radiographic imaging with chest x-ray or CT scan should be performed as clinically indicated.
Vulvar cancer spreads in decreasing order of frequency by direct extension to adjacent organs, lymphatic drainage to regional lymph nodes, or hematogenous to distant sites. Risk of lymph node metastasis is directly related to depth of primary tumor invasion, size of tumor, poor differentiation, and lymphovascular space involvement. In 2010, the International Federation of Gynecology and Obstetrics (FIGO) revised the surgical pathologic staging system for vulvar cancer.
IA: Tumor confined to vulva or perineum under 2cm in size and less than 1mm invasion, negative nodes.
IB: Tumor confined to vulva or perineum greater than 2cm in size and greater than 1mm invasion, negative nodes.
II: Tumor of any size with adjacent spread (1/3 lower urethra, 1/3 lower vagina, anus), negative nodes.
IIIA: Tumor any size with positive inguinal nodes; (i) One lymph node metastasis greater than 5mm (ii) One to two lymph node metastasis less than 5mm.
IIIB: (i) Two or more lymph node metastasis greater than 5 mm (ii) Three or more lymph node metastasis less than 5mm.
IIIC: Positive node(s) with extracapsular spread.
IVA: (i) Tumor invades other regional structures (2/3 upper vagina, 2/3 upper urethra), bladder mucosa, rectal mucosa, fixed to pelvic bone (ii) Fixed or ulcerated inguinal nodes.
IVB: Any distant metastasis including pelvic lymph nodes.
What other clinical manifestations may help me to diagnose vulvar cancer?
Vulvar lesion and length of its presence on the vulva.
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- Vulvar Cancer
- What every physician needs to know:
- Are you sure your patient has vulvar cancer? What should you expect to find?
- Beware of other conditions that can mimic vulvar cancer:
- Which individuals are most at risk for developing vulvar cancer:
- What laboratory and imaging studies should you order to characterize this patient's tumor (i.e., stage, grade, CT/MRI vs PET/CT, cellular and molecular markers, immunophenotyping, etc.) How should you interpret the results and use them to establish prognosis and plan initial therapy?
- What therapies should you initiate immediately i.e., emergently?
- What should the initial definitive therapy for the cancer be?
- What should you tell the patient and the family about prognosis?
- Follow-up surveillance and therapy/management of recurrences.
- What other clinical manifestations may help me to diagnose vulvar cancer?