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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.

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http://dx.doi.org/10.1007/s00403-024-03543-7DOI Listing

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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.

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Article Synopsis
  • Pilomatrix carcinoma (PMC) is a rare and aggressive hair follicle tumor with high recurrence and metastasis risks, often stemming from benign pilomatricoma or arising independently.
  • A study analyzed various surgical treatments for PMC by reviewing 257 articles from major databases, ultimately including 50 relevant studies, highlighting the effectiveness of different surgical methods.
  • The findings revealed that Mohs micrographic surgery (MMS) had no recurrences in 17 treated cases, while surgical excision resulted in a 34.5% recurrence rate, suggesting that MMS is a superior option to minimize recurrence and preserve tissue.
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