Pilomatrixomas are thought to arise from mutation in the Wnt pathway and has been linked to several genetic conditions. It is commonly misdiagnosed preoperatively; however, with better awareness of the lesion, it can be appropriately treated while avoiding unnecessary diagnostic tests. Complete surgical excision with clear margins is almost always curative.

Pilomatrix carcinoma is a rare, locally aggressive tumor with a tendency to recur. Distant metastases have been reported, with pulmonary lesions being the most frequent manifestation. Similar to pilomatrixoma, pilomatrix carcinoma typically presents as a nontender, firm dermal swelling and is found most commonly in the head and neck region. Although pilomatrixomas and pilomatrix carcinoma are well-recognized lesions, clinically they are frequently misdiagnosed as other skin conditions. By reviewing the literature over the past 10 years, the aims of this review are to analyze the cause, clinical presentation, histopathologic features, management and outcomes of pilomatrix carcinoma amongst children and adults. Pilomatrix carcinoma is a rare, locally aggressive tumor with a tendency to recur. 6-8 Distant metastases have been reported, with pulmonary lesions being the most frequent manifestation. 7-10 Similar to pilomatrixoma, pilomatrix carcinoma typically presents as a nontender, firm dermal swelling [3][4][5][6]8,11 and is found most commonly in the head and neck region. 9,11In contrast to benign pilomatrixomas, which show a bimodal occurrence with a slight female predominance, 2,5 pilomatrix carcinoma commonly occurs in the 5th to the 7th decade with a male to female ratio of 4:1. 9,12Given its rarity, a gold standard treatment regimen has not been established, however, most studies recommend wide local excision with or without adjuvant radiotherapy.

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