Papillomatosis cutis carcinoides D48.5

Authors: Prof. Dr. med. Peter Altmeyer, Dr. med. Lucian Cajacob

All authors of this article

Last updated on: 03.12.2022

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Synonym(s)

carcinoma verrucosum; verrucous carcinoma

History
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Gottron, 1932

Definition
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Low-malignant, slowly exophytic growing, highly differentiated, keratinizing spinocellular carcinoma with only very low metastatic tendency. This clinical picture is summarized together with other low-malignant verrucous carcinomas (Buschke-Löwenstein tumor, florid oral papillomatosis and the carcinoma cuniculatum) under the term " carcinoma verrucous". Thus, the term "papillomatosis cutis carcinoides" is increasingly losing its meaning.

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Etiopathogenesis
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Variant of the verrucous carcinoma.

Manifestation
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Mainly occurs in older age and only in patients with lymphostasis and venous outflow disorders.

Localization
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Mainly located on the lower legs, on one or both sides.

Clinical features
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Long-standing chronic dermatitis, e.g. lichen planus or chronic venous insufficiency with consecutive venous leg ulcer or chronic lymphedema. Large, cauliflower-like growths covered with greasy secretion; possibly island-like hyperkeratosis or slightly bleeding granulations.

Histology
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Exophytic and endophytic growing epithelial tumor with pronounced acanthosis and papillomatosis. The surface is overlaid by ortho- and parakeratotic horn material. In the tumor parenchyma itself, duct-like or cystic structures filled with horn, sometimes also filled with cell debris. The epithelium in the upper part of the tumor consists of well differentiated keratinocytes with preserved stratification and only few mitoses. At the base of the tumor, clumsy epithelial strands of increasingly polymorphic keratinocytes are visible. Dyskeratoses and mitoses are also present. Displacement of the local connective tissue as a sign of invasiveness is present. Occasionally, solitary epithelial islands in the middle of the local connective tissue are also present. Moderate perilesional lymphocytic infiltrate is also present.

Differential diagnosis
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spinocellular carcinoma; pyodermia vegetans.

Therapy
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Excision in healthy tissue with a lateral safety margin of 1 cm. For depth, reliable tumor freedom through histological incision margin controls is required. Terrain rehabilitation (e.g., in chronic venous insufficiency) and consecutive defect coverage with split skin or suture flap plasty is required. Since metastasis is to be expected in rare cases (with long duration), sentinel lymph node biopsy is recommended.

Cryosurgery and laser therapy are not indicated as primary therapy.

Progression/forecast
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Chronic course over years. Favourable prognosis after surgical reconstruction.

Literature
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Ackerman LV (1948) Verrucous carcinoma of oral cavity. Surgery 23: 670-678
Bues M et al (1983) Pseudocancer of the skin following lower leg amputation. Rare case of Gottron's papillomatosis cutis carcinoides. Zentralbl Chir 108:895-899
Gottron HA (1932) Papillomatosis cutis of both lower legs. Dermatol Z 63: 409-410
Gottron HA (1932) Extensive rather symmetrically arranged papillomatosis cutis. Zentralbl Haut Geschlechtskr 40: 445
Houston GD (2004) Oral pathology: case history--verrucous carcinoma. J Okla Dent Assoc 94: 24-25.
Jungmann J et al (2012) Giant verrucous carcinoma of the lower extremity in women with dementia. BMJ Case Rep doi: 10.1136/bcr-2012-006357.
Pleat J et al (2001) Cutaneous verrucous carcinoma. Br J Plast Surg 54: 554-555.
Schon MP et al (2000) Presternal verrucous carcinoma. Dermatologist 51: 766-769
Schwartz RA (1995) Verrucous carcinoma of the skin and mucosa. J Am Acad Dermatol 32: 1-21