Prolonged disease control with trabectedin in metastatic EWSR1::PATZ1 sarcoma: a case report

EWSR1::PATZ1 fusion sarcoma is an ultra-rare neoplasm with heterogeneous clinical behavior ranging from indolent to very aggressive tumor. Due to their relatively recent characterization, it is commonly treated according to Ewing sarcoma protocol similarly to other former undifferentiated small round cell sarcomas, although evidence suggests it does not share the same clinical features nor systemic treatment sensibility. In this study, we report on the case of a 29-year-old woman with chest-wall EWSR1::PATZ1 sarcoma progressing despite being treated with Ewing sarcoma protocols and experiencing a-5-year durable disease control under trabectedin.

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Journal
ESMO rare cancers.
Published
2026-09-21
DOI
https://doi.org/10.1016/j.esmorc.2026.100536
Primary Topic
Neurofibromatosis and Schwannoma Cases
Type
article
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article

Prolonged disease control with trabectedin in metastatic EWSR1::PATZ1 sarcoma: a case report

Elena Di Blasi, Salvatore Provenzano, Amalya Sargsyan, M.A. Stevens Pinochet et al.
ESMO rare cancers.
Neurofibromatosis and Schwannoma Cases
article

Prolonged disease control with trabectedin in metastatic EWSR1::PATZ1 sarcoma: a case report

Elena Di Blasi, Salvatore Provenzano, Amalya Sargsyan, M.A. Stevens Pinochet, V. Mentesidou, R. Bertulli, S. Brotea-Mosoiu, L. Simaskaite-Puzauskiene, I. Pellegrini, G. Apollonio
article en

Abstract

EWSR1::PATZ1 fusion sarcoma is an ultra-rare neoplasm with heterogeneous clinical behavior ranging from indolent to very aggressive tumor. Due to their relatively recent characterization, it is commonly treated according to Ewing sarcoma protocol similarly to other former undifferentiated small round cell sarcomas, although evidence suggests it does not share the same clinical features nor systemic treatment sensibility. In this study, we report on the case of a 29-year-old woman with chest-wall EWSR1::PATZ1 sarcoma progressing despite being treated with Ewing sarcoma protocols and experiencing a-5-year durable disease control under trabectedin.

ESMO rare cancers.Vol. 8
Carol Davila University of Medicine and Pharmacy (RO), Clínica Alemana (CL), Institut Gustave Roussy (FR), Hospital of Lithuanian University of Health Sciences Kaunas Clinics (LT), Azienda Ospedaliera San Camillo-Forlanini (IT), Hospital del Salvador (CL), Institutul Oncologic Bucuresti (RO), Ospedale Policlinico San Martino (IT), Fondazione IRCCS Istituto Nazionale dei Tumori (IT), Yerevan State Medical University (AM)
Good health and well-being
Openalex Percentile: Top 11%
Neurofibromatosis and Schwannoma Cases
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Prolonged disease control with trabectedin in metastatic EWSR1::PATZ1 sarcoma: a case report — Elena Di Blasi, Salvatore Provenzano, et al. · ESMO rare cancers. (2026) | TGRS Research Map | TGRS