Primary Cutaneous Perivascular Epithelioid Cell Tumor: Not the Same as the Noncutaneous Tumor

Perivascular epithelioid cell tumors (PEComas) affect internal organs and skin. Eight percent are primary cutaneous tumors (pcPEComas), dermal-based proliferations of clear cells. Originally categorized as myomelanocytic, pcPEComa is now included among perivascular tumors. However, the cell of origin is still elusive. These authors provide further insight.

They reviewed clinicopathologic and histologic features of eight pcPEComas. Tumors were more frequent among women, mean age 46 years. The most common clinical diagnosis was dermatofibroma. More than half of tumors affected lower extremities as indurated nodules. After complete excision and a mean follow-up period of 6.5 years, no local recurrences or metastases were detected. Tumors ranged…