What are the management options for vulvar carcinoma?

Management of Vulvar Carcinoma

Surgery is the cornerstone of vulvar carcinoma management, with treatment tailored by tumor stage, size, location, and depth of invasion, followed by adjuvant radiation ± chemotherapy for node-positive disease. 1

Early-Stage Disease (Stage IA-IB, Select Stage II)

Microinvasive Disease (≤1 mm invasion)

• Wide local excision with observation only 1
• No lymph node evaluation required due to <1% risk of lymphatic metastases 1
• If final pathology reveals >1 mm invasion, additional surgery is indicated 1

Stage IB and Select Stage II (>1 mm invasion)

Treatment depends critically on tumor location relative to the vulvar midline: 1

Lateral Lesions (≥2 cm from midline)

• Radical partial vulvectomy or modified radical vulvectomy with 1-2 cm margins 1
• Ipsilateral inguinofemoral lymph node evaluation only 1
• Options: sentinel lymph node biopsy (SLNB) OR ipsilateral inguinofemoral lymphadenectomy 1
• If no sentinel node detected, complete ipsilateral lymphadenectomy is mandatory 1

Midline Lesions (within 2 cm of midline or crossing midline)

• Radical partial vulvectomy or modified radical vulvectomy 1
• Bilateral inguinofemoral lymph node evaluation required 1
• Options: bilateral SLNB OR bilateral inguinofemoral lymphadenectomy 1

Sentinel Lymph Node Biopsy Criteria

SLNB is appropriate only when ALL of the following criteria are met: 1

• Unifocal tumor <4 cm
• Clinically non-suspicious groin nodes
• No previous vulvar surgery that disrupted lymphatic drainage
• Experienced high-volume surgeon available
• Combined technique using both technetium-99m radiocolloid AND blue dye for optimal detection 1

Critical technical point: Perform SLNB before excising the primary tumor to avoid disrupting lymphatic drainage 1

Management of Positive Nodes After Unilateral Surgery

If ipsilateral nodes are positive, the contralateral groin must be addressed: 1

• Either contralateral lymphadenectomy OR radiation to contralateral groin 1
• Evaluate suspicious nodes intraoperatively with frozen section to guide extent of dissection 1

Adjuvant Therapy After Surgery

Node-Positive Disease

Postoperative radiation to the groins is mandatory for node-positive disease 1

• Adjuvant radiation significantly decreases recurrence and improves relapse-free and overall survival 1
• Nodal involvement is the strongest independent predictor of relapse 1
• Dose: 50.4 Gy in 1.8 Gy fractions for adjuvant therapy 1

Consider concurrent chemotherapy with radiation for: 1

• Multiple positive lymph nodes
• Extranodal extension
• Chemotherapy options: cisplatin alone, 5-FU + cisplatin, or 5-FU + mitomycin C 1

Close or Positive Margins (<8 mm)

Two options exist, though evidence for superiority is lacking: 1

• Re-resection to obtain adequate margins (if feasible without major morbidity)
• Adjuvant local radiation therapy 1

Important caveat: Do not re-resect margins involving urethra, anus, or vagina if this would cause significant morbidity or quality of life impairment 1

Do not re-resect in node-positive patients who will receive radiation anyway 1

Locally Advanced Disease (Stage III-IVA)

Primary chemoradiation is the preferred approach for locally advanced disease to preserve organ function and avoid exenterative surgery 1

Primary Chemoradiation Protocol

• Radiation dose: 59.4-64.8 Gy in 1.8 Gy fractions for unresectable disease 1
• Concurrent chemotherapy: cisplatin-based regimens 1
• Large nodes may be boosted to approximately 70 Gy 1
• Treat once daily, 5 days per week, minimizing treatment breaks 1

Chemoradiation demonstrates superior survival compared to radiation alone: 5-year overall survival 49.9% vs 27.4% (P<0.001) 1

Surgery After Chemoradiation

• If complete response not achieved, surgical resection of residual disease is recommended 1
• Complete response rates exceed 60% in many cohorts 1
• Radical surgery remains viable for treatment failures 2

Bulky Inguinofemoral Nodes with Unresectable Primary

Two reasonable approaches: 1

1. Primary cytoreductive surgery of bulky nodes followed by chemoradiation to groins and primary tumor
2. Chemoradiation to bilateral groins and primary tumor alone

Advanced, Recurrent, or Metastatic Disease

Systemic Chemotherapy Options

For advanced/recurrent/metastatic disease: 1

• Cisplatin alone
• Cisplatin + vinorelbine
• Cisplatin + paclitaxel

Recurrent Disease Management

Location of recurrence dictates prognosis and treatment: 1

• Local vulvar recurrences: occur later, often cured with additional surgery (70% cure rate for lateralized recurrences) 3
• Groin failures: occur early, rarely curable 1

Treatment by recurrence site: 3

• Lateralized local recurrence: wide radical local excision with inguinal lymphadenectomy
• Central pelvic recurrence (involving urethra/vagina/rectum): consider pelvic exenteration in select patients
• Inguinal recurrence without prior radiation: excision followed by chemoradiation
• Inguinal recurrence with prior radiation: avoid excision due to high complication rates; offer brachytherapy for palliation 3

Critical Surgical Principles

Modern surgical approach uses separate incisions for vulvar tumor and lymph nodes, replacing historical en bloc resection that caused excessive morbidity 1

Goal margins: 1-2 cm negative margins 1

Depth of resection: to the urogenital diaphragm for both radical local excision and radical vulvectomy 1

No difference in recurrence between radical local excision and radical vulvectomy based on retrospective data 1

Radiation Technique Considerations

Use 3D conformal or IMRT to ensure adequate coverage while minimizing toxicity 1

Brachytherapy boost can be used for anatomically amenable primary tumors 1

Minimize treatment breaks and aggressively manage acute toxicities (diarrhea, bladder irritation, mucocutaneous reactions) 1

Initiate postoperative adjuvant treatment within 6-8 weeks once adequate healing achieved 1