Pilomatrix carcinoma: 12‐year experience and review of the literature

Abstract

Pilomatrix carcinoma is a rare, locally aggressive tumor with a tendency to recur. Distant metastases have been reported, with pulmonary lesions being the most frequent manifestation. Similar to pilomatrixoma, pilomatrix carcinoma typically presents as a nontender, firm dermal swelling and is found most commonly in the head and neck region. Although pilomatrixomas and pilomatrix carcinoma are well-recognized lesions, clinically they are frequently misdiagnosed as other skin conditions. By reviewing the literature over the past 10 years, the aims of this review are to analyze the cause, clinical presentation, histopathologic features, management and outcomes of pilomatrix carcinoma amongst children and adults. Pilomatrix carcinoma is a rare, locally aggressive tumor with a tendency to recur. 6-8 Distant metastases have been reported, with pulmonary lesions being the most frequent manifestation. 7-10 Similar to pilomatrixoma, pilomatrix carcinoma typically presents as a nontender, firm dermal swelling [3][4][5][6]8,11 and is found most commonly in the head and neck region. 9,11In contrast to benign pilomatrixomas, which show a bimodal occurrence with a slight female predominance, 2,5 pilomatrix carcinoma commonly occurs in the 5th to the 7th decade with a male to female ratio of 4:1. 9,12Given its rarity, a gold standard treatment regimen has not been established, however, most studies recommend wide local excision with or without adjuvant radiotherapy.

Sarcomatoid pilomatrix carcinoma

“…In addition, some cases show frank pleomorphism. Ghost cells are a unique diagnostic feature of matrical differentiation; however, this feature is missing in about half of the cases of pilomatrix carcinoma . In addition, it should be remembered that tumors that also show ghost cells belong to fields other than dermatopathology, such as odontoma, ameloblastic fibroma, odontogenic carcinoma, dentinogenic ghost cell tumor or calcifying cystic odontogenic tumor .…”

“…However, ghost cells are only seen in half of the cases of pilomatrix carcinoma, and intralesional calcification or giant cells are even rarer. Other signs of matrical differentiation are matrical/supramatrical cells, bright red trichohyaline granules and blue‐gray corneocytes .…”

“…Other signs of matrical differentiation are matrical/supramatrical cells, bright red trichohyaline granules and blue‐gray corneocytes . Histopathological features of malignancy include infiltration into fat or underlying structures, necrosis, as well as cytologic atypia in the form of hyperchromasia, prominent nucleoli, atypical mitotic figures and pleomorphic nuclei …”

“…Most authors recommend wide local excision, with or without radiotherapy . Since the relapse rate is particularly high, Mohs surgery has been suggested as a successful alternative for the improvement of patients’ outcomes . This is most important since local tumor recurrence has been statistically associated with metastases …”

“…Pilomatrix (pilomatrical) carcinoma is a rare malignant tumor with matrical differentiation which was first reported in 1980 . Contrary to its benign counterpart (pilomatricoma/pilomatrixoma), which shows a bimodal age‐range presentation, with a female predominance, pilomatrix carcinoma is more common in adults (fifth to seventh decade), with a male predominance …”