Pilomatrix carcinoma: 13 new cases and review of the literature with emphasis on predictors of metastasis

J Am Acad Dermatol. 2014 Jul;71(1):38-43.e2. doi: 10.1016/j.jaad.2014.02.042. Epub 2014 Apr 13.


Background: Pilomatrix carcinoma is a rare cutaneous tumor derived from follicular matrix cells with few cases documented in the literature.

Objective: We sought to better characterize this tumor by analyzing its epidemiologic, clinical, and histopathologic features in 13 new cases and by reviewing the literature.

Methods: All cases of pilomatrix carcinoma from a large regional dermatopathology practice were identified and analyzed by chart review for clinical and histopathologic characteristics. Similar characteristics were compiled from an additional 123 cases in the English-language literature. Cox proportional hazards regression models were used to determine risk factors associated with the development of metastasis for all identified metastatic tumors.

Results: Our 13 tumors were most common in middle-aged to older white men and presented mostly on the head/neck. Histopathologically, tumors were asymmetric, were poorly circumscribed, were composed of basaloid and "ghost" cells, had frequent atypical mitoses, and had infrequent lymphovascular invasion. Wide excision was considered the most definitive treatment modality, but local recurrence was common. When analyzing all reported cases of metastasis using statistics, metastasis was significantly associated (hazard ratio 3.45, P < .0413) with local tumor recurrence.

Limitations: The retrospective, single-center design and the reliance on electronic medical records are limitations.

Conclusions: This study helps better characterize pilomatrix carcinoma and identifies potential predictors of metastasis.

Keywords: calcifying epitheliocarcinoma of Malherbe; malignant pilomatricoma; matrical carcinoma; metastatic pilomatricoma; pilomatrical carcinoma; pilomatrix carcinoma.

Publication types

  • Review

MeSH terms

  • Aged
  • Female
  • Hair Diseases / pathology*
  • Head and Neck Neoplasms / pathology*
  • Humans
  • Lymphatic Metastasis
  • Male
  • Middle Aged
  • Neoplasm Invasiveness
  • Neoplasm Metastasis
  • Neoplasm Recurrence, Local / epidemiology
  • Pilomatrixoma / pathology*
  • Proportional Hazards Models
  • Retrospective Studies
  • Risk Factors
  • Skin Neoplasms / pathology*