Systemic therapies in advanced epithelioid haemangioendothelioma: A retrospective international case series from the World Sarcoma Network and a review of literature
- Frezza A. M.
- Ravi V.
- Lo Vullo S.
- Vincenzi B.
- Tolomeo F.
- Chen T. W. -W.
- Teterycz P.
- Baldi G. G.
- Italiano A.
- Penel N.
- Brunello A.
- Duffaud F.
- Hindi N.
- Iwata S.
- Smrke A.
- Fedenko A.
- Gelderblom H.
- Van Der Graaf W.
- Vozy A.
- Connolly E.
- Grassi M.
- Benjamin R. S.
- Broto J. -M.
- Grignani G.
- Jones R. L.
- Kawai A.
- Tysarowski A.
- Mariani L.
- Casali P. G.
- Stacchiotti S.
DOIAbstract
Background: This observational, retrospective effort across Europe, US, Australia, and Asia aimed to assess the activity of systemic therapies in EHE, an ultra-rare sarcoma, marked by WWTR1-CAMTA1 or YAP1-TFE3 fusions. Methods: Twenty sarcoma reference centres contributed data. Patients with advanced EHE diagnosed from 2000 onwards and treated with systemic therapies, were selected. Local pathologic review and molecular confirmation were required. Radiological response was retrospectively assessed by local investigators according to RECIST. Progression free survival (PFS) and overall survival (OS) were estimated by Kaplan-Meier method. Results: Overall, 73 patients were included; 21 had more than one treatment. Thirty-three patients receive...
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