TY - JOUR
T1 - The natural history of epithelioid sarcoma. A retrospective multicentre case-series within the Italian Sarcoma Group
AU - Frezza, Anna Maria
AU - Sbaraglia, Marta
AU - Lo Vullo, Salvatore
AU - Baldi, Giacomo Giulio
AU - Simeone, Noemi
AU - Frenos, Filippo
AU - Campanacci, Domenico
AU - Stacchiotti, Silvia
AU - Pasquali, Sandro
AU - Callegaro, Dario
AU - Gambarotti, Marco
AU - Barisella, Marta
AU - Palomba, Annarita
AU - Mariani, Luigi
AU - Casali, Paolo G
AU - Dei Tos, Angelo Paolo
AU - Gronchi, Alessandro
N1 - Copyright © 2020 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.
PY - 2020/7
Y1 - 2020/7
N2 - 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 70% and 47% in the whole series, 57% and 37% in patients with proximal-type ES, 77% and 54% in patients with classic-type ES (P = 0.02). In classic-type ES, 5- and 10-year OS was higher for low-grade (95% and 72%, respectively) than high-grade tumours (P = 0.002). 5- and 10-year CCI estimates for LR were 21% and 33% in the whole series. 5- and 10-year CCI estimates for DM were 35% and 39% in the whole series, both 28% in classic-type ES, 47% and 59% in proximal-type 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.
AB - 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 70% and 47% in the whole series, 57% and 37% in patients with proximal-type ES, 77% and 54% in patients with classic-type ES (P = 0.02). In classic-type ES, 5- and 10-year OS was higher for low-grade (95% and 72%, respectively) than high-grade tumours (P = 0.002). 5- and 10-year CCI estimates for LR were 21% and 33% in the whole series. 5- and 10-year CCI estimates for DM were 35% and 39% in the whole series, both 28% in classic-type ES, 47% and 59% in proximal-type 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.
KW - Distal type
KW - Epithelioid sarcoma
KW - Outcome
KW - Proximal type
U2 - 10.1016/j.ejso.2020.03.215
DO - 10.1016/j.ejso.2020.03.215
M3 - Article
C2 - 32307188
VL - 46
SP - 1320
EP - 1326
JO - Eur. J. Surg. Oncol.
JF - Eur. J. Surg. Oncol.
SN - 0748-7983
IS - 7
ER -