Table of Contents
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Epidemiology
- 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
Differential
- 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
- 5-10% of Paget’s lesions are associated with underlying adenocarcinoma of vulva or a distant site.
Pathology
- 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
Staging
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).
Surgery
- 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