title: Renal angiomyolipomas: At least two diseases. A series of patients treated at two European institutions.
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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Surgical Oncology, Gustave Roussy, 114 rue Edouard Vaillant, 94805, Villejuif Cedex, France.
authors.forename: J-B
authors.initials: JB
authors.lastname: Delhorme
authors.affiliation: Department of Surgical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, via Venezian, 1, 20133, Milano, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Fontana
authors.affiliation: Department of Radiation Oncology, Gustave Roussy, 114 rue Edouard Vaillant, 94805, Villejuif, France.
authors.forename: A
authors.initials: A
authors.lastname: Levy
authors.affiliation: Department of Pathology, Gustave Roussy, 114 rue Edouard Vaillant, 94805, Villejuif, France.
authors.forename: P
authors.initials: P
authors.lastname: Terrier
authors.affiliation: Department of Surgical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, via Venezian, 1, 20133, Milano, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Fiore
authors.affiliation: Department of Surgical Oncology, Institut Curie, PSL University, 26 rue d'Ulm, 75248, Paris, France.
authors.forename: D
authors.initials: D
authors.lastname: Tzanis
authors.affiliation: Department of Surgical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, via Venezian, 1, 20133, Milano, Italy.
authors.forename: D
authors.initials: D
authors.lastname: Callegaro
authors.affiliation: Department of Radiology, Institut Curie, PSL University, 26 rue d'Ulm, 75248, Paris, France.
authors.forename: C
authors.initials: C
authors.lastname: Dratwa
authors.affiliation: Department of Surgical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, via Venezian, 1, 20133, Milano, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Gronchi
authors.affiliation: Department of Surgical Oncology, Institut Curie, PSL University, 26 rue d'Ulm, 75248, Paris, France. Electronic address: sylvie.bonvalot@curie.fr.
authors.forename: S
authors.initials: S
authors.lastname: Bonvalot
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018207:Angiomyolipoma / Q000145:classification* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D059248:Chemoradiotherapy; D018450:Disease Progression; D005260:Female; D005602:France; D006801:Humans; D007558:Italy; D007680:Kidney Neoplasms / Q000145:classification* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D008175:Lung Neoplasms / Q000556:secondary; D008198:Lymph Nodes / Q000473:pathology; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology*; D009378:Neoplasms, Multiple Primary / Q000145:classification* / Q000473:pathology / Q000628:therapy; D009392:Nephrectomy; D011379:Prognosis; D012082:Renal Veins / Q000473:pathology; D012189:Retrospective Studies; D057832:Watchful Waiting
keywords: Active surveillance; Angiomyolipoma; Epithelioid angiomyolipoma; Prognostic factors; Surgery