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      Myofibromatosis in adults, glomangiopericytoma, and myopericytoma: a spectrum of tumors showing perivascular myoid differentiation.

      The American Journal of Surgical Pathology
      Actins, analysis, Adolescent, Adult, Aged, Child, Desmin, Female, Glomus Tumor, chemistry, pathology, Hemangiopericytoma, Humans, Immunohistochemistry, Male, Middle Aged, Myofibromatosis, Myoma

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          Abstract

          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), persistence of congenital lesions into adulthood (4 cases), and tumors that were multifocal within the confines of one anatomic region (7 cases). Histologically, all cases showed a biphasic pattern that consisted of fascicles of spindle cells with abundant eosinophilic cytoplasm that resembled smooth muscle, in addition to a population of more primitive spindled cells associated with a hemangiopericytomalike 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. Patient age 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 hemangiopericytoma is discussed.

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          Most cited references26

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          HEMANGIOPERICYTOMA: A VASCULAR TUMOR FEATURING ZIMMERMANN'S PERICYTES.

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            Infantile myofibromatosis.

            The clinical and pathologic features of 61 cases of congenital and infantile myofibromatosis are presented. The tumor affected almost exclusively infants and young children; 88% of cases occurred before the age of two years, and 60% were noted at birth or shortly thereafter. Solitary (45 cases) and multicentric (16 cases) forms of the tumor could be distinguished; the solitary form was more common in males (69%) and affected chiefly the soft tissues of the head-neck region and the trunk. The multicentric form, on the other hand, predominated in females (63%) and was found not only in soft tissues but also in bones and viscera. Fourteen of the 16 cases with multicentric lesions were present at birth, and four of them had evidence of visceral involvement. Among the 16 cases were two sets of siblings, both brother and sister. Grossly, nearly all of the tumors were well demarcated; they measured from 0.5 to 7 cm in greatest diameter. Microscopically, they formed well-circumscribed nodules consisting of short bundles of plump, spindle-shaped cells displaying staining characteristics intermediate between fibroblasts and smooth muscle cells. Necrosis or a hemangiopericytoma-like pattern was often found in the center of the tumor nodules. Intravascular growth was encountered in several instances, but this feature did not seem to affect the clinical behavior. Of the 43 patients with follow-up data (mean-follow up 5.1 years), 36 were alive and well. Of the solitary lesions, three recurred. Of the multicentric lesions, five of the 15 patients showed evidence of spontaneous regression of both soft tissue and bone lesions. Four died, three of extensive involvement of multiple viscera, especially the lung, and one of "crib-death."
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              Juvenile fibromatoses.

              Tom Stout (1954)
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