Other locations are less frequent
•
Usually solitary
Multicentric tumors can occur
•
Some cases may be associated with viral warts (plantar lesions)
•
Verrucous carcinoma (VC) may arise in setting of longstanding lichen sclerosus (genital tumors)
Macroscopic
•
Exophytic papillary tumor
•
Deep burrowing pattern is hallmark of carcinoma cuniculatum (VC variant)
Microscopic
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Acanthotic papillae
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Slender fibrovascular cores
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Prominent (orange) keratin craters between papillae
•
Extremely well differentiated
•
Epithelium of papillae and keratin predominate over fibrovascular core
•
Pushing, club-shaped base
•
Higher grade areas &/or infiltrative borders are not features of pure VC
Raises possibility of hybrid (mixed) VC
Top Differential Diagnoses
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Carcinoma cuniculatum (VC variant)
•
Mixed/hybrid VC with foci of SCC of usual type
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Condyloma acuminatum/giant condyloma
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Warty (condylomatous) SCC
•
Papillary carcinoma (SCC variant)
TERMINOLOGY
Abbreviations
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Verrucous carcinoma (VC)
•
Squamous cell carcinoma (SCC)
Synonyms
•
Buschke-Loewenstein tumor
Confusing term that has been used in reference to different papillary/verrucous squamous lesions, including VC
We recommend abandoning this term
•
Ackerman tumor (oral florid papillomatosis)
Older term
Definitions
•
Usually human papillomavirus (HPV)-unrelated variant of SCC with papillary surface and bulbous deep borders
•
Very well differentiated
ETIOLOGY/PATHOGENESIS
Unknown Pathogenesis
•
Most VC are unrelated to HPV
•
p53 may play role in its pathogenesis
•
Cutaneous lesions may be related to scarring and chronic inflammation
•
Anogenital cases may be associated with
Phimosis
Lichen sclerosus (LS)
Lichen simplex chronicus
•
Oral VC (Ackerman tumor) may be related to tobacco chewing
•
Cutaneous (particularly plantar) lesions may arise within preexisting warts
•
Rare cases of anogenital and cutaneous VCs may be associated with low- or high-risk HPV
CLINICAL ISSUES
Epidemiology
•
Incidence
Rare
•
Age
6th-7th decade
Site
•
Originally described by Lauren Ackerman in oral cavity
•
May also affect anogenital area and skin (sole of foot, finger, nail bed, scalp, wrist, buttocks, etc.)
Penile tumors are more frequent on foreskin
Presentation
•
Exophytic white-gray neoplasm
•
Unicentric tumors are more frequent than multicentric ones
•
Anogenital lesions often arise in background of longstanding LS
Prognosis
•
Pure VCs have excellent prognosis
Tumors may recur but almost never metastasize
•
Hybrid/mixed VCs have worse prognosis than pure VC
Behavior relates to least differentiated component
•
There are sporadic reports of sarcomatoid/anaplastic transformation after radiation therapy
MACROSCOPIC
General Features
•
Exophytic white-gray neoplasms with papillary, sometimes spiky surface
•
Cut sections reveal broad base between tumor and stroma
•
Tumors may invade deep dermis and deeper structures
•
Irregular jagged borders or foci of necrosis are not features of pure VC