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

• Published in: European journal of surgical oncology 2020-07, Vol.46 (7), p.1320-1326
• Main Authors: 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
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Child; Child, Preschool; Distal type; Epithelioid sarcoma; Female; Groin; Head and Neck Neoplasms - pathology; Head and Neck Neoplasms - therapy; Humans; Infant; Infant, Newborn; Italy; Kaplan-Meier Estimate; Lower Extremity; Lung Neoplasms - pathology; Lung Neoplasms - secondary; Lymphatic Metastasis; Male; Middle Aged; Neoplasm Grading; Neoplasm Recurrence, Local - pathology; Neoplasm, Residual; Outcome; Proximal type; Retrospective Studies; Sarcoma - pathology; Sarcoma - secondary; Sarcoma - therapy; Soft Tissue Neoplasms - pathology; Soft Tissue Neoplasms - therapy; Survival Rate; Upper Extremity; Urogenital Neoplasms - pathology; Urogenital Neoplasms - therapy; Young Adult
• ISSN: 0748-7983; 1532-2157
• DOI: 10.1016/j.ejso.2020.03.215

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. 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. 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). 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.