Epithelioid angiomyolipoma of the kidney: pathological features and clinical outcome in a series of consecutively resected tumors View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2013-10

AUTHORS

Wenlei He, John C Cheville, Peter M Sadow, Anuradha Gopalan, Samson W Fine, Hikmat A Al-Ahmadie, Ying-Bei Chen, Esther Oliva, Paul Russo, Victor E Reuter, Satish K Tickoo

ABSTRACT

The 2004 World Health Organization classification of tumors defines epithelioid angiomyolipoma of kidney as a potentially malignant mesenchymal neoplasm with reported metastasis in approximately one-third of the cases. However, this conclusion was based primarily on individual case reports and small retrospective series. More recently reported larger series have shown varying results. We reviewed 437 consecutive renal angiomyolipomas with primary resection at three tertiary-care institutions with high nephrectomy volumes. Only tumors showing >80% epithelioid histology were included in this study. Tumors resected elsewhere and reviewed in consultation were not included. Twenty of these 437 (4.6%) were classified as epithelioid angiomyolipoma. The female to male ratio was 11:9, mean age 49.7 (range, 30-80) years, and mean tumor size 8.7 (range, 1-25) cm. Microscopic tumor necrosis was present in 10 (50%) tumors and mitotic activity (range, <1-5/10 high power fields) in 8 (40%); atypical mitoses were seen in only 1 (5%) tumor. Pleomorphic ganglion-like or multinucleated giant cells were seen in 18 (90%) tumors. With a mean follow-up of 82.5 (range, 1-356) months, seventeen patients were alive with no-evidence-of-disease at the time of last follow-up; two patients died of unrelated causes with no-evidence-of-disease, and one patient (5%) developed distant metastases. Our data, based on consecutively resected angiomyolipomas with long clinical follow-up, suggests that epithelioid angiomyolipomas constitute a small proportion of all angiomyolipomas, and the rate of aggressive behavior among epithelioid angiomyolipomas, even when showing morphologic features previously reported to portend aggressive clinical behavior, is very low. More... »

PAGES

1355

Identifiers

URI

http://scigraph.springernature.com/pub.10.1038/modpathol.2013.72

DOI

http://dx.doi.org/10.1038/modpathol.2013.72

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1007491127

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/23599151


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