Epithelioid angiomyolipoma of the kidney: Pathological features and clinical outcome in a series of consecutively resected tumors Journal Article


Authors: He, W.; Cheville, J. C.; Sadow, P. M.; Gopalan, A.; Fine, S. W.; Al-Ahmadie, H. A.; Chen, Y. B.; Oliva, E.; Russo, P.; Reuter, V. E.; Tickoo, S. K.
Article Title: Epithelioid angiomyolipoma of the kidney: Pathological features and clinical outcome in a series of consecutively resected tumors
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. © 2013 USCAP, Inc. All rights reserved.
Keywords: pecoma; epithelioid angiomyolipoma; renal angiomyolipoma
Journal Title: Modern Pathology
Volume: 26
Issue: 10
ISSN: 0893-3952
Publisher: Nature Research  
Date Published: 2013-10-01
Start Page: 1355
End Page: 1364
Language: English
DOI: 10.1038/modpathol.2013.72
PROVIDER: scopus
PUBMED: 23599151
DOI/URL:
Notes: --- - "Export Date: 1 November 2013" - "CODEN: MODPE" - "Source: Scopus"
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MSK Authors
  1. Paul Russo
    581 Russo
  2. Satish K Tickoo
    483 Tickoo
  3. Anuradha Gopalan
    417 Gopalan
  4. Yingbei Chen
    398 Chen
  5. Wenlei He
    2 He
  6. Samson W Fine
    462 Fine
  7. Victor Reuter
    1228 Reuter