Case Reports

Early Pilomatrix Carcinoma: A Case Report With Emphasis on Molecular Pathology and Review of the Literature

Cutis. 2021 October;108(4):E24-E28 | doi:10.12788/cutis.0393

References

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Our case was strongly and diffusely positive for β-catenin in a nuclear and cytoplasmic pattern and CDX-2 in a nuclear pattern, supporting the role of the Wnt signaling pathway in such tumors. Furthermore, our case demonstrated the presence of few intralesional normal dendritic melanocytes, a rare finding^1,24,25 but not unexpected, as melanocytes normally are present within the hair follicle matrix.

Pilomatrix carcinomas are aggressive tumors with a high risk for local recurrence and tendency for metastasis. In a study of 13 cases of pilomatrix carcinomas, Herrmann et al¹³ found that metastasis was significantly associated with local tumor recurrence (P<.0413). They concluded that the combination of overall high local recurrence and metastatic rates of pilomatrix carcinoma as well as documented tumor-related deaths would warrant continued patient follow-up, especially for recurrent tumors.¹³ Rapid growth of a tumor, either de novo or following several months of stable size, should alert physicians to perform a diagnostic biopsy.

Management options of pilomatrix carcinoma include surgery or radiation with close follow-up. The most widely reported treatment of pilomatrix carcinoma is wide local excision with histologically confirmed clear margins. Mohs micrographic surgery is an excellent treatment option.^2,13-15 Adjuvant radiation therapy may be necessary following excision. Currently there is no consensus on surgical management, and standard excisional margins have not been defined.²⁶ Jones et al² concluded that complete excision with wide margins likely is curative, with decreased rates of recurrence, and better awareness of this carcinoma would lead to appropriate treatment while avoiding unnecessary diagnostic tests.²

Conclusion

We report an exceptionally unique case of early pilomatrix carcinoma with a discussion on the pathogenesis and molecular pathology of hair matrix tumors. A large cohort of patients with longer follow-up periods and better molecular characterization is essential in drawing accurate information about their prognosis, identifying molecular markers that can be used as therapeutic targets, and determining ideal management strategy.

Early Pilomatrix Carcinoma: A Case Report With Emphasis on Molecular Pathology and Review of the Literature

References

Conclusion

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