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Renal Angiomyolipoma With Epithelioid Sarcomatous Transformation and Metastases

2000; Lippincott Williams & Wilkins; Volume: 24; Issue: 6 Linguagem: Inglês

10.1097/00000478-200006000-00017

1532-0979

Guido Martignoni, Maurizio Pea, Gilbert Rigaud, Erminia Manfrin, Chiara Colato, Giuseppe Zamboni, Aldo Scarpa, Regina Tardanico, Massimo Roncalli, Franco Bonetti,

Vascular Tumors and Angiosarcomas

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 morphoimmunophenotypic characters of PEC. After 7 years from surgical removal of the lesion, the patient developed a local recurrence and successive lung 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.