Perivascular epithelioid cell tumor (PEComa) of the cheek.
We present the unusual case of a perivascular epithelioid cell tumor (PEComa) occurring within the cheek of a 32-year-old woman. PEComa is a rare, recently described, family of tumors with diverse clinicopathologic expression and which express melanocytic and muscle markers. It mainly affects the abdominopelvic region and rarely occurs in somatic soft tissue or skin. To our knowledge, this is the first reported case of PEComa occurring in the facial cutaneous tissues. Other possible diagnoses considered included benign mesenchymal tumors of smooth muscle or neural origin. However, the cytomorphologic and immunohistochemical profile were most suggestive of PEComa. The tumor was completely excised, but in view of uncertainty as to how this entity would behave in an unusual location, lifelong follow up is recommended. After complete excision, there was no recurrence in 4 years.
Ghazali
Naseem
N
Queen Victoria Hospital, East Grinstead, United Kingdom. naseemghazali@doctors.org.uk
Cascarini
Luke
L
Norris
Paul
P
Barrett
A W
AW
Lavery
Kenneth M
KM
D000199:Actins / Q000032:analysis; D000328:Adult; D000951:Antigens, Neoplasm / Q000032:analysis; D001160:Arterioles / Q000473:pathology; D002610:Cheek / Q000473:pathology*; D003937:Diagnosis, Differential; D015622:Epithelioid Cells / Q000473:pathology; D005153:Facial Neoplasms / Q000175:diagnosis* / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D058965:MART-1 Antigen; D009131:Muscle, Smooth, Vascular / Q000473:pathology; D009363:Neoplasm Proteins / Q000032:analysis; D054973:Perivascular Epithelioid Cell Neoplasms / Q000175:diagnosis* / Q000473:pathology; D012983:Soft Tissue Neoplasms / Q000175:diagnosis* / Q000473:pathology
D000199:Actins; D000951:Antigens, Neoplasm; D058965:MART-1 Antigen; C548667:MLANA protein, human; D009363:Neoplasm Proteins