期刊
AMERICAN JOURNAL OF SURGICAL PATHOLOGY
卷 24, 期 6, 页码 889-894出版社
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/00000478-200006000-00017
关键词
perivascular epithelioid cell; angiomyolipoma; melanoma markers; tuberous sclerosis; immunohistochemistry; loss of heterozygosity
Angiomyolipoma (AML) is a benign neoplasm that occurs either sporadically or in patients with tuberous sclerosis complex (TSC) and shows frequent allelic losses at chromosome arm 16p. It has been suggested recently that the melanogenesis marker-positive perivascular epithelioid cell (PEC) has been found consistently in AML, The authors report a 50-year-old woman without evidence of TSC affected by classic renal AML containing an area composed of atypical epithelioid cells with the same morphoimmunuphenotypic characters of PEG. After 7 years from surgical removal of the lesion, the patient developed a local recurrence and successive lungs and abdominal metastases that showed morphologic and immunohistochemical features overlapping those of the epithelioid area of the previously removed AML, Genetic analysis showed that the classic AML and its epithelioid area as well as the pulmonary and abdominal metastases shared the same allelic loss on chromosome arm 16p. Based on these findings, the authors view this case as evidence of a malignant transformation of a classic AML with morphologic, immunophenotypic, and genetic demonstration of its clonal origin.
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