Verrucous Carcinoma (and Variants)

 Other locations are less frequent



• Usually solitary
image 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

• Broad and pushing base

• Deep burrowing pattern is hallmark of carcinoma cuniculatum (VC variant)


Microscopic




• Acanthotic papillae
• Slender fibrovascular cores

• Prominent (orange) keratin craters between papillae

• Lack of koilocytosis

• 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

image Raises possibility of hybrid (mixed) VC


Top Differential Diagnoses




• Carcinoma cuniculatum (VC variant)

• Mixed/hybrid VC with foci of SCC of usual type

• Condyloma acuminatum/giant condyloma

• Warty (condylomatous) SCC

• Papillary carcinoma (SCC variant)

image
Multicentric Verrucous Carcinoma
Clinical image shows 2 separate verrucous carcinomas (VCs) (one on the right image and the other on the left image) affecting the foreskin of a patient with chronic lichen sclerosus (LS) image.


image
Partial Penectomy Cut Section
Cut section of a partial penectomy specimen shows a verruciform tumor with sharp bulbous base image confined to the lamina propria. Note the papillomatous and spiky surface.

image
Verrucous Carcinoma and Lichen Sclerosus
Histologic section illustrates a VC image confined to the lamina propria arising in a background of LS image.

image
Verrucous Carcinoma
Well-differentiated tumor with a well-defined stromal-tumor interface shows papillomatosis, acanthosis, and hyperkeratosis.


TERMINOLOGY


Abbreviations




• Verrucous carcinoma (VC)

• Squamous cell carcinoma (SCC)


Synonyms




• Buschke-Loewenstein tumor
image Confusing term that has been used in reference to different papillary/verrucous squamous lesions, including VC

image We recommend abandoning this term

• Ackerman tumor (oral florid papillomatosis)
image 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

image Phimosis

image Lichen sclerosus (LS)

image 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
image Rare

• Age
image 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.)

image 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


Treatment




• Surgical


Prognosis




• Pure VCs have excellent prognosis
image Tumors may recur but almost never metastasize

• Hybrid/mixed VCs have worse prognosis than pure VC
image 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

Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Verrucous Carcinoma (and Variants)

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