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Mini Oral - Sarcoma

1627MO - Systemic therapies in advanced epithelioid haemangioendothelioma (EHE): A retrospective international series from the World Sarcoma Network

Date

18 Sep 2020

Session

Mini Oral - Sarcoma

Topics

Tumour Site

Sarcoma

Presenters

Anna Maria Frezza

Citation

Annals of Oncology (2020) 31 (suppl_4): S914-S933. 10.1016/annonc/annonc288

Authors

A.M. Frezza1, V. Ravi2, S. Lo Vullo3, F. Tolomeo4, T. Wei-Wu Chen5, P. Teterycz6, G.G. Baldi7, A. Italiano8, N. Penel9, A. Brunello10, F. Duffaud11, N. Hindi12, S. Iwata13, A. Smrke14, A. Fedenko15, H. Gelderblom16, W. Van Der Graaf17, A. Vozy18, B. Vincenzi19, S. Stacchiotti20

Author affiliations

  • 1 Oncologia Medica 2, Istituto Nazionale dei Tumori di Milano - Fondazione IRCCS, 20133 - Milan/IT
  • 2 Department Of Sarcoma Medical Oncology, University of Texas MD Anderson Cancer Center, Huston/US
  • 3 Unit Of Clinical Epidemiology And Trial Organization, Istituto Nazionale dei Tumori di Milano - Fondazione IRCCS, 20133 - Milan/IT
  • 4 Department Of Cancer Medicine, Fondazione del Piemonte per l'Oncologia, IRCCS, Candiolo, Turin/IT
  • 5 Department Of Oncology/ Phase I Center, National Taiwan University Hospital, Taipei/TW
  • 6 Department Of Soft Tissue/bone Sarcoma And Melanoma, The Maria Sklodowska-Curie Memorial Institute and Oncology Centre, 02-781 - Warsaw/PL
  • 7 Medical Oncology, Ospedale "S. Stefano", Prato/IT
  • 8 Early Phase Trials Unit, Institute Bergonié, 33076 - Bordeaux/FR
  • 9 General Oncology Department, Centre Oscar Lambret, 59020 - Lille/FR
  • 10 Medical, Istituto Oncologico Veneto, Padova/IT
  • 11 Medical Oncology, CHU La Timone Adultes, 13385 - Marseille/FR
  • 12 Dept. Medical Oncology, Hospital Universitario Virgen del Rocio, 41013 - Sevilla/ES
  • 13 Department Of Musculoskeletal Oncology, National Cancer Center Hospital, Tokyo/JP
  • 14 Sarcoma Unit, Royal Marsden NHS Foundation Trust/Institute of Cancer Research, London/GB
  • 15 Department Of Medical Oncology, N. N. Blokhin Russian Cancer Research, Moscow/RU
  • 16 Medical Oncology Dept, Leids Universitair Medisch Centrum (LUMC), 2333 ZA - Leiden/NL
  • 17 Medical Oncology, Netherlands Cancer Institute, Amsterdam/NL
  • 18 Medical Oncology, University Hospitals Pitié Salpêtrière, Paris/FR
  • 19 Medical Oncology, Università Campus Bio-Medico, Roma/IT
  • 20 Adult Mesenchymal Tumor Medical Oncology Unit, Cancer Medicine Dpt, Istituto Nazionale dei Tumori di Milano - Fondazione IRCCS, 20133 - Milan/IT

Resources

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Abstract 1627MO

Background

This observational, retrospective effort across Europe, US and Asia aims to assess the activity of systemic therapies in EHE, a ultra rare sarcoma, marked by WWTR1-CAMTA1 or YAP1-TFE3 fusion, typically metastatic at presentation and with a unpredictable clinical course.

Methods

18 sarcoma reference centres contributed data. Patients with advanced EHE diagnosed 2000 onwords, treated with systemic therapies, were selected. Local pathological review and molecular confirmation was 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

72 patients were included (characteristics in the table), 21 had more than one treatment. 33 patients received anthracycline-based regimens, achieving 1 (3%) partial response (PR), 25 (76%) stable disease (SD, 14 with prior PD), 7 (21%) progressive disease (PD). The median (m-) PFS and m-OS were 5 and 14 months respectively. 11 patients received weekly paclitaxel, achieving 1 (9%) PR, 6 (55%, 2 with prior PD) SD, 4 (36%) PD. The m-PFS and m-OS were 3 and 19 months respectively. 12 patients received pazopanib, achieving 3 (25%, 2 with prior PD) SD, 9 (75%) PD. The m-PFS and m-OS were.3 and 9 months respectively. 15 patients received INF-α 2b, achieving 1 (7%) PR, 11 (73%, 8 with prior PD) SD, 3 (20%) PD. The m-PFS and m-OS were 9 months and unreached respectively. Among 27 patients treated with other regimens, 1 PR (ifosfamide) and 9 SD (all with prior PD, 5 gemcitabine+docetaxel, 2 cyclophosphamide, 2 others) were reported. Table: 1627MO

Population characteristics

Anthracycline based regimens Paclitaxel Pazopanib INFalfa2b Others
Patients 33 11 12 15 27
Median follow up 35.8 on 72 patients - - - -
Marker CAMTA1-WWTR1* YAP1-TFE3 * IHC o molecular testing 69 (95%) 4 (5%) on 72 patients - - - -
Median age 49 38 43 46 48
Gender M F 15 (45%) 18 (55%) 4 (36%) 7 (64%) 4 (33%) 8 (67%) 7 (47%) 8 (53%) 12 (44%) 15 (56%)
Stage (treatment start) Locally advanced Metastatic 6 (18%) 27 (82%) 1 (9%) 10 (91%) 1 (8%) 12 (92%) 3 (20%) 12 (80%) 4 (14%) 23 (86%)
Evidence of prior PD: Yes No 19 (58%) 14 (42%) 6 (55%) 5 (45%) 10 (83%) 2 (17%) 12 (80%) 3 (20%) 24 (89%) 3 (11%)
Previous line 0 1 ≥ 2 29 (88%) 4 (12%) 0 8 (73%) 3 (27%) 0 5 (42%) 6 (50%) 1 (8%) 13 (86%) 1 (7%) 1 (7%) Not applicable

Conclusions

Systemic therapies available for advanced sarcomas exhibited limited activity in EHE. Although PFS should be interpreted with caution as non-progressive cases were included, m-PFS with chemotherapy and pazopanib was short (<6 months). The identification of new active compounds, especially for rapidly progressive cases, is needed.

Clinical trial identification

Editorial acknowledgement

Legal entity responsible for the study

Fondazione IRCCS Istituto Nazionale Tumori.

Funding

Has not received any funding.

Disclosure

A.M. Frezza: Research grant/Funding (institution): Amgen Dompè; Research grant/Funding (institution): Bayer; Research grant/Funding (institution): Daiichi Sankyo; Research grant/Funding (institution): Eli Lilly; Research grant/Funding (institution): Epizyme; Research grant/Funding (institution): Novartis; Research grant/Funding (institution), Travel/Accommodation/Expenses: Pharmamar; Research grant/Funding (institution): Pfizer; Research grant/Funding (institution): Eisai. G.G. Baldi: Honoraria (self), Travel/Accommodation/Expenses: Pharmamar; Honoraria (self), Travel/Accommodation/Expenses: Eli Lilly; Honoraria (self): Eisai. F. Duffaud: Honoraria (self): Bayer. N. Hindi: Honoraria (self), Research grant/Funding (institution), Travel/Accommodation/Expenses: Pharmamar; Honoraria (self), Research grant/Funding (institution): Eli Lilly; Research grant/Funding (institution): Eisai; Honoraria (institution): Novartis; Research grant/Funding (institution): Deciphera; Research grant/Funding (institution): Blueprint; Research grant/Funding (institution): Karyopharm; Research grant/Funding (institution): Daychii; Research grant/Funding (institution): GSK; Research grant/Funding (institution): Adaptimmune; Research grant/Funding (institution): Amgen. W. Van Der Graaf: Research grant/Funding (institution): Novartis; Honoraria (institution): Bayer; Honoraria (institution): Springworks. S. Stacchiotti: Advisory/Consultancy: Adaptimmune; Advisory/Consultancy, Research grant/Funding (institution): Bayer; Advisory/Consultancy, Research grant/Funding (institution): Daiichi Sankyo; Advisory/Consultancy, Research grant/Funding (institution): Eli Lilly; Advisory/Consultancy, Research grant/Funding (institution): Epizyme; Advisory/Consultancy: Immunodesign; Advisory/Consultancy: Karyopharm; Advisory/Consultancy: Maxivax; Advisory/Consultancy, Research grant/Funding (institution): Pharmamar; Research grant/Funding (institution): Amgen Dompè; Advisory/Consultancy: Takeda; Research grant/Funding (institution): Novartis; Research grant/Funding (institution): Pfizer. All other authors have declared no conflicts of interest.

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