Clear Cell Acanthoma with Malignant Cytologic Features: A Case Report and Review of the Literature

Clear cell acanthoma (CCA) is classically considered a benign epidermal tumor, although rare case reports have described CCA with malignant features. Here, we present a case of a patient with a biopsy proven CCA that regrew post-biopsy and was subsequently completely excised. Histologic examination of the tumor in the excision specimen revealed malignant cytologic features that were not present in the initial biopsy. A review of the literature identified five additional cases of CCA with similar malignant cytologic features. On analysis, common histopathologic characteristics included cellular pleomorphism, increased nuclear-to-cytoplasmic ratio, prominent nucleoli, and atypical mitotic figures. We support the designation of atypical clear cell acanthoma for these entities with features of both CCA and significant cytologic atypia. As none of these cases exhibited clinically aggressive behavior, further study is warranted.