Abstract
Objective
The aim of this study is to analyze the outcome of renal angiomyolipomas (AML) at
two European institutions.
Methods
The data were collected from patients with a primary AML who were treated at Gustave
Roussy, Villejuif, France and Fondazione IRCCS Istituto Nazionale dei Tumori in Milan,
Italy from 1998 to 2014. The specimens were classified as classic AML (C AML) or epithelioid
AML (E AML) based on the percentage of epithelioid cells.
Results
There were 40 patients identified for the study (35 C AML, 5 E AML). One patient had
an associated tuberous sclerosis complex. Six patients (15%) had bilateral AML. The
imaging results were significantly different between C/E AML. E AML was associated
with fewer bilateral lesions, more renal vein/vena cava extension, and more poor or
non-fatty aspects. Surgery/active surveillance (AS)/chemo radiation were applied for
28/11/1 patients, respectively. The median tumor size was significantly smaller (3.75 cm)
in patients receiving AS (median 15 cm when surgically resected). The median patient
follow-up was 43 months. The three-year overall survival was significantly better
for patients with C AML than E AML (100% versus 50%, p < 0.0001). The univariate analysis
identified the OS prognostic factors were E AML histologic subtype (p < 0.001), poor/non
fatty features (p = 0.002), and renal vein extension on imaging (p = 0.01).
Conclusion
AML manifests as at least two different entities with significantly different outcomes.
Epithelioid subtype, poor/non-fatty features, and renal vein involvement are all associated
with worse survival.
Keywords
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Article info
Publication history
Published online: December 05, 2016
Accepted:
November 17,
2016
Identification
Copyright
© 2016 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.