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

doi:10.1016/j.ejso.2020.03.215

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

Research output: Contribution to journal › Article › peer-review

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 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.

Original language	English
Pages (from-to)	1320-1326
Number of pages	7
Journal	Eur. J. Surg. Oncol.
Volume	46
Issue number	7
DOIs	https://doi.org/10.1016/j.ejso.2020.03.215
Publication status	Published - Jul 2020

Keywords

Distal type
Epithelioid sarcoma
Outcome
Proximal type

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Frezza, A. M., Sbaraglia, M., Lo Vullo, S., Baldi, G. G., Simeone, N., Frenos, F., Campanacci, D., Stacchiotti, S., Pasquali, S., Callegaro, D., Gambarotti, M., Barisella, M., Palomba, A., Mariani, L., Casali, P. G., Dei Tos, A. P., & Gronchi, A. (2020). The natural history of epithelioid sarcoma. A retrospective multicentre case-series within the Italian Sarcoma Group. Eur. J. Surg. Oncol., 46(7), 1320-1326. https://doi.org/10.1016/j.ejso.2020.03.215

The natural history of epithelioid sarcoma. A retrospective multicentre case-series within the Italian Sarcoma Group. / Frezza, Anna Maria; Sbaraglia, Marta; Lo Vullo, Salvatore; Baldi, Giacomo Giulio; Simeone, Noemi; Frenos, Filippo; Campanacci, Domenico; Stacchiotti, Silvia ; Pasquali, Sandro ; Callegaro, Dario ; Gambarotti, Marco ; Barisella, Marta; Palomba, Annarita; Mariani, Luigi ; Casali, Paolo G; Dei Tos, Angelo Paolo; Gronchi, Alessandro.

In: Eur. J. Surg. Oncol., Vol. 46, No. 7, 07.2020, p. 1320-1326.

Research output: Contribution to journal › Article › peer-review

Frezza, AM, Sbaraglia, M, Lo Vullo, S, Baldi, GG, Simeone, N, Frenos, F, Campanacci, D, Stacchiotti, S , Pasquali, S , Callegaro, D , Gambarotti, M , Barisella, M, Palomba, A, Mariani, L , Casali, PG, Dei Tos, AP & Gronchi, A 2020, 'The natural history of epithelioid sarcoma. A retrospective multicentre case-series within the Italian Sarcoma Group', Eur. J. Surg. Oncol., vol. 46, no. 7, pp. 1320-1326. https://doi.org/10.1016/j.ejso.2020.03.215

Frezza, Anna Maria ; Sbaraglia, Marta ; Lo Vullo, Salvatore ; Baldi, Giacomo Giulio ; Simeone, Noemi ; Frenos, Filippo ; Campanacci, Domenico ; Stacchiotti, Silvia ; Pasquali, Sandro ; Callegaro, Dario ; Gambarotti, Marco ; Barisella, Marta ; Palomba, Annarita ; Mariani, Luigi ; Casali, Paolo G ; Dei Tos, Angelo Paolo ; Gronchi, Alessandro. / The natural history of epithelioid sarcoma. A retrospective multicentre case-series within the Italian Sarcoma Group. In: Eur. J. Surg. Oncol. 2020 ; Vol. 46, No. 7. pp. 1320-1326.

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

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 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.",

keywords = "Distal type, Epithelioid sarcoma, Outcome, Proximal type",

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

year = "2020",

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doi = "10.1016/j.ejso.2020.03.215",

language = "English",

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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

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 -