Pilomatrix carcinoma unveiled: a systematic review of clinical characteristics, pathogenesis, diagnostic puzzles, and management strategies.

Pilomatrix carcinoma (PC) is a rare malignant adnexal tumor originating from follicular matrix cells primarily impacting Caucasian males. This review provides a comprehensive analysis of scientific literature on PC through an exploration of 206 cases reported between 1980 and 2024. We discuss the epidemiology, clinical presentation, histopathology, and diagnostic challenges of PC, and explore various treatment methods for this rare malignancy as well as their associated outcomes. Wide local excision (WLE) is the most common management method, but Mohs micrographic surgery (MMS) may yield superior margin control and lower risk of recurrence and metastasis. Our findings underscore the need for further studies with thoroughly documented follow-up in order to create standardized evidence-based management guidelines and reduce risk of recurrence and metastasis of this rare and aggressive tumor.