Issn Print: 0193-1091
Publication Date: 1997/08/01
Syringofibrocarcinoma Altered by Epidermolytic Vacuolization and Acantholytic Dyskeratosis in a Patient with Bullous Congenital Ichthyosiform Erythrodermia (BCIE).
Excerpt
Syringofibrocarcinoma was recently described as the first case of malignancy in syringofibroadenoma (Mascaro). That tumor displayed a deep trichoblastoma although located on the back of the hand of an old man. It was asked if the case was fortuitous or repeatable in the future. This report will consider a syringofibrocarcinoma developing in a patient with BCIE. The patient was a 34-year-old woman, who had had a verrucous growth on her right flank for 4 years. At excision, it was 3 × 4 cm large. Soon she got axillary metastases and died after 1 year with general spread of the tumor. The sections showed a verrucous syringoacanthoma with a thick corneal layer. On its undersurface, anastomosing branches of clear pleomorphic cells intermingled with small dyskeratotic cells proliferating inward to the bottom of the specimen in the subcutaneous border. The dyskeratosis ensued in clefting of the epithelial aggregates and deep breaches opened to the surface. The sides were villous and the epithelial pouches and aggregates revealed sharply limited acrosyringeal complexes, i.e., clear cell lobules containing ductal elements with outer layers and lumina lined by luminal cells. The reticulated branches were all embedded in a richly vascularized fibrotic stroma that developed deep sclerotic centra and otherwise granulation tissue around the aggressive extensions. The axillary metastases were generally alike, although more anaplastic. It is thought that the clear cells and the dyskeratotic cells represent epidermolytic hyperkeratosis and focal acantholytic dyskeratosis, respectively, in this rare malignancy.
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