Pilomatrix carcinoma, the malignant variant of the common pilomatrixoma, is a rare, locally aggressive tumour. It typically presents as an asymptomatic firm nodule, most commonly in the head and neck region. Patients are most often older Caucasian males in their 6th and 7th decades. Pilomatrix carcinoma is so rare that to date less than 150 cases have been reported in the English literature.(1, 2) Indeed, in their 2017 systematic literature review, which examined reports published between 2004 and 2017, Jones et al. identified case information for only 22 patients.(3)
First, we present a case of a 69 year old man from a nursing home who presented to our emergency department with a fast growing, mobile exophytic mass on the side of his neck. A punch biopsy of the lesion was reported as basal cell carcinoma. However, following wide local excision, the lesion was identified as a pilomatrix carcinoma.
Second, adding to the findings of Jones et al., we conduct our own updated literature review. We searched PubMed and Embase databases for literature on “pilomatrix carcinoma” published between 2016 and 2021. Publications included by Jones et al. were excluded, as were animal studies. We identified 22 new cases published in the past five years, suggesting an increase in either the incidence or recognition of this rare tumour.