Malignant Transformation of Renal Angiomyolipoma
- 1 April 2002
- journal article
- case report
- Published by Ovid Technologies (Wolters Kluwer Health) in The American Journal of Surgical Pathology
- Vol. 26 (4), 523-529
- https://doi.org/10.1097/00000478-200204000-00017
Abstract
We report a case of renal angiomyolipoma (AML) with malignant transformation. A 28-year-old woman developed large bilateral renal masses 5 months before admission to our hospital. She was diagnosed with tuberous sclerosis when she was 4 years old. Total nephrectomy of the left kidney was performed, but she died during the operation. Although the focal region of the resected tumor had the appearance of a classic AML, most of the lesion showed a diffuse proliferation of atypical epithelioid cells resembling that in renal cell carcinoma. The epithelioid cells had extremely pleomorphic and hyperchromatic nuclei with frequent mitotic figures, including atypical forms. Immunohistochemical analysis revealed that the atypical epithelioid cells and the typical AML lesions were both positive for HMB-45 but that the former were negative for epithelial and myogenic markers. The smooth muscle cells and thick-walled vessels were focally positive for muscle-specific actins. Furthermore, the atypical epithelioid cells were immunoreactive for p53, whereas the foci of the typical AML were negative. Examination of the microdissected paraffin-embedded tissues revealed p53 mutations in the malignant epithelioid areas in AML but not in the renal parenchyma or typical AML areas. In this case it is proposed that p53 mutation may play an important role in malignant transformation of renal AML.Keywords
This publication has 22 references indexed in Scilit:
- Malignant Epithelioid Angiomyolipoma (`Sarcoma Ex Angiomyolipoma') of the KidneyThe American Journal of Surgical Pathology, 2001
- MALIGNANT TRANSFORMATION OF RENAL ANGIOMYOLIPOMAJournal of Urology, 1999
- Malignant tumors of the kidney, brain, and soft tissues in children and young adults with the tuberous sclerosis complexCancer, 1998
- Atypical angiomyolipoma of the kidneyCancer, 1998
- Epithelioid Angiomyolipoma of the KidneyThe American Journal of Surgical Pathology, 1997
- Apparent preferential loss of heterozygosity atTSC2 overTSC1 chromosomal region in tuberous sclerosis hamartomasGenes, Chromosomes and Cancer, 1996
- Loss of heterozygosity in the tuberous sclerosis (TSC2) region of chromosome band l6p13 occurs in sporadic as well as TSC‐associated renal angiomyolipomasGenes, Chromosomes and Cancer, 1995
- Renal angiomyolipomas and HMB-45 reactivityCancer, 1993
- Renal Angiomyolipoma With Sarcomatous Transformation and Pulmonary MetastasesThe American Journal of Surgical Pathology, 1991
- Increased expression of mutant forms of p53 oncogene in primary lung cancerThe Lancet, 1990