Anna Maria Frezza1, Marta Sbaraglia2, Salvatore Lo Vullo3, Giacomo Giulio Baldi4, Noemi Simeone5, Filippo Frenos6, Domenico Campanacci6, Silvia Stacchiotti5, Sandro Pasquali7, Dario Callegaro7, Marco Gambarotti8, Marta Barisella9, Annarita Palomba10, Luigi Mariani3, Paolo G Casali11, Angelo Paolo Dei Tos2, Alessandro Gronchi7. 1. Department of Medical Oncology, IRCCS Fondazione Istituto Nazionale Tumori, Milan, Italy. Electronic address: annamaria.frezza@istitutotumori.mi.it. 2. Department of Pathology, Padua University Hospital, Padua, Italy. 3. Unit of Clinical Epidemiology and Trial Organization, IRCCS Fondazione Istituto Nazionale Tumori, Milan, Italy. 4. Department of Medical Oncology, Nuovo Ospedale "S.Stefano", Prato, Italy. 5. Department of Medical Oncology, IRCCS Fondazione Istituto Nazionale Tumori, Milan, Italy. 6. Department of Orthopaedic Oncology, Azienda Ospedaliero Universitaria Careggi, Florence, Italy. 7. Sarcoma Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. 8. Department of Surgical Pathology, IRCCS-Rizzoli Orthopaedic Institute, Bologna, Italy. 9. Department of Pathology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. 10. Department of Pathology, Azienda Ospedaliero Universitaria Careggi, Florence, Italy. 11. Department of Medical Oncology, IRCCS Fondazione Istituto Nazionale Tumori, Milan, Italy; University of Milan, Department of Oncology and Hemato-oncology, Milan, Italy.
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.
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.
Authors: Tatjana Maňáková; Jan Hojný; Martin Sedlář; Michal Vočka; Kristian Chrz; Petr Mitáš; Jiří Beneš; Robert Holaj Journal: SAGE Open Med Case Rep Date: 2022-06-24