Pilomatrix Carcinoma: Report of Two Cases of the Head and Review of the Literature

Abstract

Background: Pilomatrix carcinoma (PC) is a rare skin tumor arising from hair follicle matrix cells. It is locally aggressive with a high rate of local recurrence after surgical excision. Few cases in the literature have been described and the management is not well defined. Objectives: The aim of this study was to present two cases of PC located on the head and review the relevant literature about epidemiology, clinical and dermoscopic evaluation, characteristics of local and distant metastases, local recurrence rate and management of this rare skin tumor. Methods: We consulted databases from PubMed, Research Gate and Google Scholar, from January 2012 to November 2022. We reviewed the literature and reported two additional cases. Results: We selected 52 tumors in middle-aged to older patients located mostly on the head. Dermoscopy evaluation was rarely performed in the pre-operative diagnostic setting. The most definitive treatment was wide local excision, but local recurrences were common. In total, we observed 11 cases of recurrences and 9 patients with locoregional or distant metastases. Four patients received adjuvant radiotherapy, two patients needed chemotherapy and local cancer therapy and one patient received radiochemotherapy. Conclusions: Our reports and the review of the literature can provide a better awareness and management of this rare tumor.

Giant pilomatrix carcinoma of the scalp with intracranial extension: A case report

“…Despite the prompt neurosurgery and radiotherapy, the patient' final outcome was fatal. Similarly, in their study Toffoli et al 14 found that distant metastases were uncommon, occurring in 10%-13% of patients, of which two (4%) had metastases at the first tumor staging following primary tumor excision, and six (12%) had metastases during follow-up. Most common sites were lung and regional lymph nodes, followed by local bone infiltration, skull bones, brain, and parotid gland.…”

“…Most common sites were lung and regional lymph nodes, followed by local bone infiltration, skull bones, brain, and parotid gland. Only one patient had diffuse metastases to the lung, pleura, liver, and bones 14 . The role of chemotherapy is still debated 9,12 .…”

Metastatic pilomatrix carcinoma treated with stereotactic body radiation therapy

“… 3 Rates of metastasis are reported to be as high as 17%. 4 The challenge is further compounded by resistance to chemotherapy. 5 The case herein demonstrated a rapid in-field progression of metastatic pilomatrix carcinoma during radiation, reflecting the aggressive nature of this cutaneous malignancy and perhaps suggesting a component of radioresistance to even high doses of radiation delivered with SBRT.…”

Pilomatrix carcinoma of the lower extremity: A rare case report and literature review

“…Pilomatrix carcinoma (PC) is an extremely rare, locally aggressive, malignant skin tumor arising from aberrant proliferation of the hair follicles matrix [ 1 ]. PC arises de novo or through malignant transformation of a pre-existing pilomatrixoma [ 2 ].…”

“…PC arises de novo or through malignant transformation of a pre-existing pilomatrixoma [ 2 ]. It most predominantly affects male with a male-to-female ratio of 1.3:1 and demonstrates a bimodal age distribution [ 1 , 2 ]. PC mostly arises in the head and neck, and usually presents as a nodular, violaceous, firm, painless lesion that grows rapidly with overlying ulceration [ 1 , 3 ].…”