Myopericytoma: A Series of 5 Cases Affecting the Nervous System.

Abstract

Myopericytomas are benign vascular tumors composed of perivascular myoid cells that usually arise in superficial soft tissues of the extremities. We here report on 5 cases of myopericytoma, malignant myopericytoma, and glomangiopericytoma arising from subcutaneous, paraspinal, orbital, and sellar regions that secondarily involved the nervous system. Patients experienced orbital swelling, progressing neuropathic pain, dermatoma specific pain, and oculomotor paresis. Mean age at diagnosis was 56.8 years (range, 38-74 years). After a mean follow-up of 38 months, no recurrence was detected. All tumors were unencapsulated but relatively well-circumscribed lesions, showing typical histologic and immunohistochemical features of soft-tissue myopericytoma, including one malignant case. We conclude that myopericytoma may affect the nervous system and for prognostic and therapeutic reasons, needs to be differentiated from other vascular tumors.