Renal angiomyolipoma is a tumor composed of a mixture of thick-walled
blood vessels, smooth muscle, and mature adipose tissue. It may presen
t as a single unilateral lesion or as a multifocal uni- or bilateral n
eoplasm, The histologic spectrum of angiomyolipoma is wide but as far
as we know intratumoral fibrinoid vasculitis has not been described in
the tumor. This is the first report of isolated intratumoral fibrinoi
d vasculitis observed in two nodules of a unilateral multifocal renal
angiomyolipoma. The vasculitis arose in a hypertensive 62-year-old wom
an who presented with a history of dull right flank and low back pain
of 3 months' duration. There were no signs of generalized disease. Imm
unophenotyping of the vascular cellular infiltrate disclosed abundant
T lymphocytes, significant numbers of histiocytes, and absence of B ly
mphocytes. The diagnosis of isolated intratumoral arteritis depends on
the exclusion of systemic disease. It is important to distinguish cas
es like this one to avoid misdiagnosis and to prevent unnecessary trea
tment.