Management of a pilomatrixoma with atypical features

In this case we report a 54-year-old man presenting with a large fungating mass that had been identified as a pilomatrixoma on a biopsy from an outside hospital. Based on the atypical clinical and radiographic findings we were concerned that the lesion may have aggressive features and decided to perform a wide local excision with 2 centimeter margins to manage this patient’s risk of recurrence. On surgical pathology, the lesion did have characteristics that were concerning for progression to a pilomatrix carcinoma, however, a definitive invasive component was not identified. This case emphasizes the preferred management in pilomatrixoma with atypical features is a wide local excision.