1486P - An epidemiological insight into epithelioid sarcoma (ES): the open issue of distal-type (DES) versus proximal-type (PES)

Date 11 September 2017
Event ESMO 2017 Congress
Session Poster display session
Topics Sarcoma
Presenter Anna Maria Frezza
Citation Annals of Oncology (2017) 28 (suppl_5): v521-v538. 10.1093/annonc/mdx387
Authors A.M. Frezza1, L. Botta2, S. Pasquali3, S. Stacchiotti1, A. Gronchi3, P.G.G. Casali1, A. Trama2, W.G. Rarecarenet2
  • 1Adult Mesenchymal Tumour And Rare Cancer Medical Oncology Unit, Fondazione IRCCS - Istituto Nazionale dei Tumori, 20133 - Milan/IT
  • 2Evaluative Epidemiology Unit, Fondazione IRCCS - Istituto Nazionale dei Tumori, 20133 - Milan/IT
  • 3Department Of Surgery, Fondazione IRCCS - Istituto Nazionale dei Tumori, 20133 - Milan/IT



ES is a rare sarcoma with distinctive pathologic and clinical features as well as the potential to respond to new targeted agents. We used the RARECAREnet and the SEER18 cancer registry (CR) databases (DB) to highlight the epidemiological hallamrks of ES in the EU and in the US.


The ICD-O3 code 8804 applies both to the DES and PES. Thus we broke down the primary sites using the ICD-O3 topography codes, to separate the anatomically proximal and distal forms, as a partial surrogate for the two entities of DES and PES. Incidence rate (IR), world-age adjusted IR and relative survival (RS) were calculated for ES patients diagnosed in the period 2000-07 and followed-up at least up to 31st Dec 2008. RS was estimated by the Ederer II method. 497 new cases of ES were identified from available registries in the EU, 301 in the US.


Crude IR was 0.03/100,000 in the EU and 0.05/100,000 in the US. Age-adjusted IR were 0.02/100,000 and 0.05/100,000 respectively. M:F ratio was 1.6 in both sets of registries. Mean age at diagnosis was 46 in the EU and 44 yrs in the US. IR increased with age (higher in > 75-yr patients). IR in the childhood population ( 40EU: 0.03 US: 0.06EU: 39% (32-46) US: 39% (30-47)EU: 49% (37-61) US: 62% (45-75)EU: 33% (25-41) US: 26% (17-35)


ES is a very rare cancer, exceedingly low in childhood. Prognosis is serious, making the identification of new treatments a priority. New targeted agents are currently under study. However, the distinction between DES and PES is likely to be of major prognostic significance. In fact, using anatomical location as a proxy, though inappropriate, we could see clear-cut differences in the epidemiological outcome. An effort to stratify ES patients by their pathologic subtype is warranted in all studies, all the more on new agents.

Clinical trial identification

Legal entity responsible for the study

Fondazione IRCCS Istituto Nazionale Tumori Milano




All authors have declared no conflicts of interest.