Vulva Cancer


  • 2-4% of Gynecological tumors (4th most common female reproductive tract malignancy)
  • Median age 65 to 70, with peak > 70.
  • only 30-50% associated with HPV (> 90% cervical cancer contain HPV-16)
  • HPV-negative tumors usually occur in older women (55-85), are often associated with vulvar inflammation orlichen scleroosis, are genereally unifocal, and are well differentiated with exuberant keratin formation.
  • OS-5 78.1%
  • 2-4% are malignant melanomas

Presentation and Evaluation


  • Inflammatory dermatoses
  • Vulva Dystrophies
    • Squamous hyperplasia - Fluorinated corticosteroids
    • Lichen Sclerosis - testosterone cream
    • Mixed Dystrophy - combination of both
  • VIN (vulva intraepithelium neoplasm)
    • May produce patterns indistinguishable from invasive cancer
    • Tx: surgical excision
    • 4% of treated VIN subsequently develop invasive cancer.
  • Paget’s disease
    • 5-10% of Paget’s lesions are associated with underlying adenocarcinoma of vulva or a distant site.
      • Treated with wide excision or simple vulvectomy


  • 90% of invasive tumors are SCCA.
  • HPV-positive tumors often have a warlike or basaloid growth pattern.
  • Small-cell anaplastic carcinomas can arise in the vulva and resemble neuroendocrine carcinomas of the lung of cervix.
  • Adenocarcinoma, melanomas, sarcomas (leiomyosarcomas)

Clinical Presentation

  • Localized at diagnosis 61% (93.2% OS-5)
  • adenopathy at diagnosis 28% (55.1% OS-5)
  • distant mets at diagnosis 4% (18.0% OS-5)

Routes of Spread

Lesion location Primary LN Drainage
lateralized lesions superficial inguinal
central lesions medial femoral
  • theoretically central lesions can spread to obturator nodes, but this is rarely seen
  • Lymphatic
    • Primary lymphatic drainage is superficial inguinal nodes
    • Clitoris and Bartholin’s gland occasionally spread directly to the deep femoral and obturator nodes.
    • Frequently crosses midline (except well-lateralized T1 lesions)
  • Hematogenous
    • Lung, bone, liver

Incidence of LN Metastasis


Chung Staging (Clark Varient) for melanomas of Vulva
Stage Depth OS-10
Stage I in epidermis 100%
Stage II <1mm 81%
Stage III 1-2mm 87%
Stage IV >2mm 11%
Stage V in adipose 33%

International Federation of Gynecology and Obstetrics Staging of Carcinoma of the Vulva (1994)
STAGE I Lesions 2 cm or less in size confined to the vulva or perineum (T1).a No nodal metastases (N0).
Stage IA Lesions 2 cm or less in size confined to the vulva or perineum and with stromal invasion no greater than 1 mm.bNo nodal metastases.
Stage IB Lesions 2 cm or less in size confined to the vulva orperineum and with stromal invasion greater than 1 mm.bNo nodal metastases.
STAGE II Tumor confined to the vulva and/or perineum ormore than 2 cm in the greatest dimension (T2). No
nodal metastasis (N0).
STAGE III Tumor of any size with adjacent spread to the lower urethra and/or the vagina, or the anus (T3), and/or
unilateral regional node metastasis (N1).
STAGE IVA Tumor invades any of the following: upper urethra, bladder mucosa, rectal mucosa, pelvic bone (T4),
and/or bilateral regional node metastasis (N2).
STAGE IVB Any distant metastasis, including pelvic lymph nodes (M1).

a Equivalent tumor, node, metastasis (TNM) groupings according to the TNM Committee of the International Union Against Cancer are indicated in parentheses.405
b The depth of invasion is defined as the measurement of the tumor from the epithelial-stromal junction of the adjacent most superficial dermal papilla to the deepest point of invasion.

Surgical Techniques

Radical Vulvectomy - Bassett-Way operation (1952, 1957)

  • Primary
    • removal of the entire vulva from the perineum to the upper margin of the mons pubis
    • The excision extends to the mymenal ring, pubic rami, and the urogenital diaphragm.
  • Inguinal lymphadenectomy
    • Bilateral excision of the tissues in the femoral triangle and those overlying the inguinal ligament (superficial & deep inguinal nodes and femoral nodes)
  • Pelvic Lymphadenectomy
    • External iliac nodes (if iliac nodes smear reveals disease)
  • Morley reported a 73.9% corrected 5-year survival for all patients using this technique

Wide Local Excision - DiSaia

  • In 1979, DiSaia et al reported a small series of 20 patients with primary lesions less than 1 cm in size and invasion less than 5 mm in depth
  • Surgery:
    • wide local excision
    • superficial inguinal nodes removed through an 8-cm incision
    • ie. removal of all nodal tissue, medial to the vessels, and above the fascia.
  • Approximately 8–10 nodes were removed from each groin.
  • No isolated groin recurrences were observed in this selected population.
  • The authors did not describe plucking 1 or 2 nodes from the groin but rather a complete removal of the intact fat pad.
  • Hacker et al urged caution that a portion of the lymph node dissection not be omitted, recognizing that groin recurrence is often fatal.
  • No longer is a single en bloc radical operation applied for all patients at all stages.
  • WLE with 1 cm margin
  • Ipsilateral groin node dissection can be performed through a separate incision removing 8–12 lymph nodes. Bilateral dissection should be performed if there is a midline lesion or if the ipsilateral nodes are positive.
From: Stehman: Obstet Gynecol, Volume 107(3).March 2006.719-733

Concerning Margins

Clitoral Margins

  • In this situation, a 1-cm margin must suffice at times in order to preserve sexual function.
  • Although there are no data on sexual function following radiotherapy to preserve the clitoris,
  • consultation with a radiation oncologist about nonsurgical options is appropriate in selected patients.

Urethral Margins

  • With partial urethral resection
    • 57% spraying of urine
    • change in continence

Anal Margin

  • In selected patients with very limited involvement of the rectum or anus, a local resection that preserves organ function is possible.
    • Two-thirds of patients in the series had their colostomies successfully reversed 6 months after the initial resection.
  • If the area of compromised margin is more than 1 to 2 cm, then chemoradiation and sparing of the anal sphincter should be considered.

Positive Margin Anticipated

  • If adequate margins can be achieved only by exenteration, chemoradiation should be strongly considered.

Sentinal Lymph Node

  • A collective review of the published literature in 2002 by Plante et al found 12 series with 353 patients with vulvar cancer.
  • Ninety-two percent had a sentinel node detected, and the positive predictive value was 99%.
  • The Gynecologic Oncology Group is currently conducting a prospective clinical trial (C. Levenback, personal communication, January 12, 2006). This large trial will be able to identify an increase in the relative risk of a false-negative sentinel node even though the absolute risk is low.
  • In their staging study of complete inguinal-femoral lymph node dissection, one patient in 387 (0.3%) suffered a groin recurrence despite having had a negative groin node dissection.
  • If sentinel node mapping has a 3% false-negative rate, this would represent a 10-fold increase in risk. The current trial is powered to determine how many more recurrences would be expected after a negative sentinel node dissection.

Complications of Deep Dissection

  • Deep nodes are rarely involved if the superficial LN(-).
  • Complication of deep node dissections are
  • Wound breakdown and 30% lymphedema
  • (Figge AJOG 74;Rutledge AJOG 70)
  • Europe series using modern techniques, Gaarenstroom et al
  • 172 patients with 187 groin dissections between 1993 and 2000
  • triple incision technique.
  • Both superficial and deep nodes were removed, and a mean of 10 nodes per groin were retrieved.
  • 66% of patients had some complication of the groin dissection.
  • Wound breakdown occurred in 17%
  • wound infection in 39%
  • lymphocyst formation in 40%
  • lymphedema in 28%.

Treatment of Inguinal Node

Option 1 (Preferred based on GOG 36)

  • WLE + superficial inguinal node dissection
  • If inguinal node is positive (>= 2) continue with pelvic RT covering bilateral inguinal to 45- 50 Gy

Option 2

  • WLE + post-operative pelvic and inguinal radiation for those with “high” nodal risk despite GOG 88 based on criticisms of the study and institutional data (MDACC).


  • Local
    • WLE with 1 cm margin (50% recurrence if < 8 mm margin [Heaps, Hullu])
  • Groin
    • < 1 mm invasion, No treatment
    • Well lateralized T1 (< 2 cm tumor) needs only ipsilateral inguinal treatment, otherwise contralateral treatment needs consideration
    • SLN under investigation (very good negative preditive value near 100%).
    • 71% chronic leg edema reported by patients (Boutselis)
    • 20% chronic leg edema reported by Rutledge et al.
  • Superficial inguinal lymphadenectomy
    • 16.5% 5-yr recurrence rate (A, Katz, P. Eifel 2003).
    • 9/121 (8.3%) inguinal recurrence rate with 6 of the 9 having grade 3 disease
1. Cox J, Ang K. Radiation Oncology: rationale Technique Results. 8th ed. New York: Mosby, 2003.
2. Gunderson L, Tepper J: Clinical Radiation Oncology. 2nd ed. China: Elsevier 2007.
3. Devita V, Hellman S, Rsenberg S: Cancer: Principles and practice of Oncology. 7th ed. Philadelphia: Lippincott, 2005.
4. Stehman. Obstet Gynecol, Volume 107(3).March 2006.719-733
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