Abstract

Perivascular tumors include the myopericytoma/myofibroma family of tumors, as well as glomus tumors, all of which may show some degree of morphologic overlap and show myoid differentiation morphologically and immunohistochemically. PEComas are an unusual group of tumors with dual myomelanocytic differentiation immunohistochemically and often show a striking distribution around blood vessel walls, hence their inclusion in this group. All of these tumors show a range of biologic/clinical behavior–while most are benign, a small minority may pursue a malignant clinical course. Rare familial or syndromic associations are discussed where relevant.

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