General
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• Not a common tumor
• Verrucous carcinoma was first described in 1948 in the oral cavity (Surgery 1948;23:670)
• Though verrucous carcinoma has traditionally been considered to be in the spectrum of giant condylomas histologically, recent studies have suggested that verrucous carcinomas are distinct entities with a non-HPV etiology (Am J Surg Pathol 2004;28:638, Int J Gynecol Cancer 2003;13:317)

Epidemiology
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• Most commonly elderly, postmenopausal women
• Almost all lesions in younger women with similar features are giant condyloma or warty carcinoma / basaloid carcinoma

Sites
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• Most commonly affected sites are labium majora > labia minora > posterior commissure

Etiology
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• Though initially considered HPV associated (Eur J Gynaecol Oncol 1998;19:319), recent studies show no HPV nucleic acids detected

Clinical features
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• Slow growing firm mass, usually accompanied by pruritus and occasionally pain and discharge (especially if large, ulcerated or infected)

Diagnosis
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• Histologic examination alone can be misleading due to superficial sampling or lack of an obvious invasive component
• Therefore, clinical history indicating exophytic tumor in an elderly woman is often helpful

Radiology
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• May be useful to assess extent of tumor and exclude metastases (Pan Afr Med J 2014;17:303)

Prognostic factors
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• Most important prognostic factors are depth of invasion and surgical margin status
• Local recurrence is common (30-50%), especially after resection with inadequate margins (Br J Dermatol 2000;142:1195)

Case reports
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• 41 year old woman with Turner syndrome (Gynecol Oncol 2004;92:380)
• 72 and 78 year old women (Eur J Gynaecol Oncol 2011;32:680, Case Rep Obstet Gynecol 2013;2013:932712)
• Verrucous carcinoma of vulva (J Indian Med Assoc 1996;94:456)

Treatment
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• Usually excision by partial, simple or radical vulvectomy, with or without inguinal or femoral lymphadenectomy
• Local radiotherapy may be used as an adjuvant, but has been associated with development of higher grade squamous cell carcinoma
• Systemic acitretin treatment (dose: 25 mg/day, for 4-6 weeks) is effective, with tumor regression in most patients (Br J Dermatol 2000;142:1195)
• Chemotherapy (cisplatin, bleomycin, methotrexate and leucovorin) (Eur J Gynaecol Oncol 2011;32:680)

Clinical images
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Abdominal pelvic CT

Gross description
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• Large, hyperkeratotic papillary or exophytic lesion, usually slow-growing
• Rarely multiple lesions (Br J Dermatol 2000;142:1195, Am J Surg Pathol 2004;28:638)

Gross images
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Vulvectomy

Micro description
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• An exo-endophytic, well circumscribed tumor composed of closely packed papillary structures lined by well differentiated stratified squamous epithelium, with minimal cellular atypia
• By definition, no invasion by clusters or single tumors cells should be present
• The epithelium demonstrates prominent acanthosis with (bulbous) expansion of rete ridges that push into the dermis / submucosa with rounded borders or a broad front
• In tangentially cut sections, the tumor is composed of large, back-to-back nests of well differentiated squamous epithelium
• Massive hyperkeratosis and parakeratosis is present
• Cells have abundant pale, eosinophilic cytoplasm with low nuclear to cytoplasmic ratio, no/mild nuclear pleomorphism, basally located mitotic figures
• Mild to moderate chronic inflammation in stroma is common
• If infection / ulceration, neutrophils may be prominent
• Lymphovascular and perineural invasion are extremely rare
• Coexisting non-HPV associated carcinomas of higher grade or precursors are not uncommon and should therefore be ruled out (Am J Surg Pathol 2004;28:638)
• Background lichen simplex chronicus with diffuse verrucous features or lichen sclerosus may be seen; VIN is not usually present

Micro images
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Globoid projections

Exophytic growth

Mild atypia

Cytology description
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• Anucleate squamous cells (Acta Cytol 1993;37:871)

Positive stains
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• Ki67 proliferation index is variably increased, but in 70-80% of cases, Ki67 expression is localized to basal layer of squamous epithelium

Negative stains
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• HPV in-situ hybridization is negative (Int J Gynecol Cancer 2003;13:317)
• p53 is not overexpressed

Molecular / cytogenetics description
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• HPV in-situ hybridization is negative (Int J Gynecol Cancer 2003;13:317)

Differential diagnosis
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• Giant condyloma acuminatum: predominantly exophytic; HPV associated cytopathic effects of koilocytes (wrinkled or raisinoid nuclei, perinuclear halo, multinucleation, suprabasal mitoses) are easily identified; HPV nucleic acids demonstrated by in-situ hybridization (Arch Gynecol Obstet 2013;287:1047)
• Other keratinizing squamous cell carcinomas: demonstrate at least focal stromal invasion by single cells or small clusters of tumors cells; cellular atypia may be prominent; variable differentiation; rare metastases to regional lymph nodes

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