Fatal metastatic cutaneous squamous cell carcinoma evolving from a localized verrucous epidermal nevus

Abstract

A malignant transformation is known to occur in many nevi such as a sebaceous nevus or a basal cell nevus, but a verrucous epidermal nevus has only rarely been associated with neoplastic changes. Keratoacanthoma, multifocal papillary apocrine adenoma, multiple malignant eccrine poroma, basal cell carcinoma and cutaneous squamous cell carcinoma (CSCC) have all been reported to develop from a verrucous epidermal nevus. CSCC has also been reported to arise from other nevoid lesions like a nevus comedonicus, porokeratosis, a sebaceous nevus, an oral sponge nevus and an ichthyosiform nevus with CHILD syndrome. Here we report a case of progressive poorly differentiated CSCC arising from a localized verrucous epidermal nevus, which caused both spinal cord and brain metastasis.

Keywords: Cutaneous squamous cell carcinoma; Epidermal nevus; Fatal metastatic cutaneous squamous cell carcinoma; Localized verrucous epidermal nevus; Metastasis.

Fig. 1

A partially pigmented 2-year-old lesion on the upper left side of our patient's back. The lesion measured 3 cm in diameter, was round and had an irregular contour and surface. It had a verrucous pigmented rim on its medial side that measured 6–8 mm, while the prominent exophytic erythematous part measured 2.1 × 2.2 cm.

Fig. 2

The pathological examination confirmed an epidermal nevus at the periphery of the lesion. H&E staining, ×50 magnification.

Fig. 3

a, b The epidermis showed bowenoid features with irregularly sized nuclei, a loss of polarity, dyskeratosis and mitotic figures. c Some sections from the periphery of the specimen showed multiple intraepidermal eddies of atypical cells or the Borst-Jadassohn phenomenon (arrow).

Fig. 4

Sheets of poorly differentiated, atypical, pleomorphic keratinocytes migrated from the epidermis into hair follicles.

Fig. 5

Pathological examination proved a perivascular and perineural invasion.

Fig. 6

The tumour exhibited a maximal depth of 7 mm.

Fig. 7

After 6 weeks, the regional lymph nodes were palpable. The swelling was so extensive that it also infiltrated the surrounding subcutaneous tissue measuring 12 × 17 cm.

Fig. 8

Metastasis is visible in both lungs (a) and in the spinal cord (b) on a CT image and bone scan, respectively.

Fig. 9

Metastasis is visible in the brain on MRI images.