The natural history of epithelioid sarcoma. A retrospective multicentre case-series within the Italian Sarcoma Group.

Anna Maria Frezza, Marta Sbaraglia, Salvatore Lo Vullo, Giacomo Giulio Baldi, Noemi Simeone, Filippo Frenos, Domenico Campanacci, Silvia Stacchiotti, Sandro Pasquali, Dario Callegaro, Marco Gambarotti, Marta Barisella, Annarita Palomba, Luigi Mariani, Paolo G. Casali, Angelo Paolo Dei Tos, Alessandro Gronchi

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Abstract

INTRODUCTION: This case-series is aimed to describe the natural history of epithelioid sarcoma (ES) and to provide insights into the differential clinical behaviour of its two variants ("classic-type" and "proximal-type"). The value of a subtype-adapted grading system based on pathological features is explored. METHODS: Data from consecutive, primary, localised, INI1-deleted ES operated at three Italian sarcoma reference centres (1995-2015) were included. Centralised pathological review was performed. Classic-type ES was broken down into "high-grade" and "low-grade", according to number of mitoses, evidence of necrosis and nuclear atypia. Five- and 10-year overall survival (OS) and crude cumulative incidence (CCI) of local recurrence (LR) and distant metastasis (DM) were estimated. RESULTS: Fifty-two patients were included. 5- and 10-year OS estimates were 707 577type ES, 774type ES (P = 0.02). In classic-type ES, 5- and 10-year OS was higher for low-grade (952 respectively) than high-grade tumours (P = 0.002). 5- and 10-year CCI estimates for LR were 213- and 10-year CCI estimates for DM were 359 both 28type ES, 479type ES (P = 0.03). CONCLUSIONS: Suffering from a proximal- or a classic-type is the stronger predictor of outcome in patients with localised ES, with proximal-type ES patients having lower survival due to a higher tendency toward metastatic spreading. However, the "high-grade" classic-type ES was associated with outcomes close to proximal-type ES.
Original languageEnglish
JournalEuropean Journal of Surgical Oncology
Issue number7
Publication statusPublished - Jul 1 2020

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