Local recurrence of cutaneous mixed tumor (chondroid syringoma) as malignant mixed tumor of the thumb 20 years after initial diagnosis

Benign cutaneous mixed tumor (chondroid syringoma) is the cutaneous counterpart of the benign mixed tumor (pleomorphic adenoma) of salivary glands, consisting of both epithelial and mesenchymal elements. The incidence of cutaneous mixed tumor is rare, composing < 0.01% of all primary skin tumors. Herein, we report a case of malignant mixed tumor which recurred in the right thumb 20 years after the reported initial diagnosis of a benign mixed tumor at this site. Histologically, the lesion consisted of highly atypical and infiltrative cells in cords and ductal structures, with an adjacent focus of residual benign mixed tumor present. Perineural invasion of multiple dermal and subcutaneous nerves was also seen. Immunohistochemical staining was strongly and diffusely positive for CK5/6 and p63, with patchy positive S100 and CK7 staining. Wide excision was performed, with no evidence of recurrence or metastasis 5 years later.