Myofibromatosis in adults, glomangiopericytoma, and myopericytoma - A spectrum of tumors showing perivascular myoid differentiation

The clinicopathologic features of 24 tumors showing perivascular myoid differentiation are described. These included tumors with histologic features of "infantile-type" myofibromatosis occurring in adult patients (8 cases), tumors with composite features of "hemangiopericytoma" and glomus tumor (9 cases), and tumors with a distinctive concentric perivascular proliferation of spindle cells. (7 cases). Evidence of morphologic overlap among these groups suggests they are closely related neoplasms that form a single spectrum. Age of patients with lesions resembling infantile-type myofibromatosis ranged from 23 to 67 years (median, 37 years). Clinicopathologic manifestations of this disease included multicentricity (4 cases), local recurrence (3 cases), persistance of congenital lesions into adulthood (4 cases), and tumors that were multifocal within the confines of one anatomic region. Histologically, all cases showed a biphasic pattern that consisted of fascicles of spindle cells with abundant eosinophilic cytoplasm that resemble smooth muscle, in addition to a population of more primitive spindled cells associated with hemangiopericytoma-like vascular pattern. Six cases showed reversal of the typical zonation seen in pediatric cases in that the primitive component surrounded the more mature fascicular areas. Also described are nine tumors with features that are intermediate between glomus tumor and hemangiopericytoma, which we have designated glomangiopericytoma. These tumors are characterized by prominent branching vessels lined by a single row of endothelial cells surrounded by epithelioid cells with a glomoid appearance. In other areas, the tumors showed typical hemangiopericytomatous foci similar to those in the myofibromatosis cases. The principal points of distinction were a lack of myoid nodules and an absence of small primitive cells with basophilic cytoplasm. Ages of these patients ranged from 17 to 78 years (median, 35 years). All tumors were located in the subcutaneous tissue and the superficial soft tissue of the extremities. Recurrence developed in one of six patients with follow-up information. The recurrent tumor had features of angiomatoid malignant fibrous histiocytoma. Finally, we describe a subset of tumors characterized by concentric periluminal proliferation of bland, round to ovoid cells, which we have designated as myopericytoma. Patients ages ranged from 10 to 66 years (median, 40 years). All were located in subcutaneous and superficial soft tissue of distal extremities. One patient had two recurrences in 3 years after initial excision. Our study suggests that these three lesional groups comprise a histologic continuum of tumors that share clinical similarities and that, perhaps, are designated more appropriately as perivascular myomas. The relationship of this family of tumors to so-called hemangiopericytomas is discussed.