Evidence map›Paper›PMID 33713582›Full record

ReviewCancer medicine2021

Systemic therapies in advanced epithelioid haemangioendothelioma: A retrospective international case series from the World Sarcoma Network and a review of literature.

Anna M Frezza, Vinod Ravi, Salvatore Lo Vullo, Bruno Vincenzi, Francesco Tolomeo, Tom Wei-Wu Chen, Pawel Teterycz, Giacomo G Baldi, Antoine Italiano, Nicolas Penel and 20 more

Registry-linked trialOpen access · goldAbstract readReview
In one paragraph

Review in Cancer medicine, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT06408441 (The Observational EURACAN Prospective Clinical Registry Dedicated to Epithelioid Hemangioendothelioma), which is not on this map. Cited by 34 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
34citing papers in PubMed, 1 pooled it
3.1field-weighted citation impact, top 6% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

NCT06408441 recruitingnot on this mapstarted 2023, after this paper: background citation

The Observational EURACAN Prospective Clinical Registry Dedicated to Epithelioid Hemangioendothelioma: the Protocol of an International and Collaborative Effort on an Ultra-rare Entity

Typeobservational_patient_registrySponsorFondazione IRCCS Istituto Nazionale dei Tumori, MilanoRan2023 to 2033Enrolled100ConditionsEpithelioid Hemangioendothelioma, Sarcoma,Soft Tissue
3 · Its place in the literature

Who cites it

34 citing papers in PubMed, 1 synthesis or guideline pooled it, 48 citations in OpenAlex.

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  5. How I treat soft tissue sarcomas.Future oncology (London, England) · 2026
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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

30 authors at 20 institutions in 11 countries.

Anna M FrezzaDepartment of Medical Oncology, IRCCS Fondazione Istituto Nazionale Tumori, Milano, Italy.ORCID 0000-0003-2335-7224
Vinod RaviDepartment of Sarcoma Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Italy.
Salvatore Lo VulloUnit of Clinical Epidemiology and Trial Organization, IRCCS Fondazione Istituto Nazionale Tumori, Milano, Italy.ORCID 0000-0002-4444-9526
Bruno VincenziDepartment of Medical Oncology, Università Campus Bio-Medico di Roma, Roma, Italy.
Francesco TolomeoDivision of Medical Oncology, Candiolo Cancer Institute, FPO - IRCCS, Candiolo, Torino, Italy.
Tom Wei-Wu ChenDepartment of Oncology, National Taiwan University Hospital and Graduate Institute of Oncology, National Taiwan University College of Medicine, Taipei, Taiwan.ORCID 0000-0003-4112-4029
Pawel TeteryczDepartment of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland.ORCID 0000-0001-7304-714X
Giacomo G BaldiDepartment of Medical Oncology, Nuovo Ospedale "S.Stefano", Prato, Italy.
Antoine ItalianoEarly Phase Trials and Sarcoma Units, Institut Bergonié, Bordeaux, France.ORCID 0000-0002-8540-5351
Nicolas PenelMedical Oncology Department, Centre Oscar Lambret, Lille, France.
Antonella BrunelloDepartment of Oncology, Medical Oncology 1 Unit, Istituto Oncologico Veneto, IRCCS, Padova, Italy.ORCID 0000-0003-2583-5226
Florance DuffaudDepartment of Medical Oncology, Medical Oncology, La Timone University Hospital, Aix-Marseille Université (AMU, Marseille, France.
Nadia HindiMedical Oncology Department, University Hospital Virgen del Rocio, and Institute of Biomedicine, Sevilla, Spain.
Shintaro IwataDepartment of Musculoskeletal Oncology, National Cancer Center Hospital, Tokyo, Japan.
Alannah SmrkeSarcoma Unit, Royal Marsden NHS Foundation Trust/ Institute of Cancer Research, Chelsea, London, United Kingdom.
Alexander FedenkoDivision of Medical Oncology, P.A. Herzen Cancer Research Institute, Moscow, Russian Federation.
Hans GelderblomDepartment of Medical Oncology, Leiden University Medical Center, Leiden, the Netherlands.
Winette Van Der GraafDepartment of Medical Oncology, Radboud University Medical Centre Nijmegen, Nijmegen, The Netherlands.
Aurore VozyDepartment of Medical Oncology, Pitié Salpétrière Hospital, Paris, France.
Elizabeth ConnollyDepartment Of Medical Oncology, Chris O'Brien Lifehouse, Sydney, Australia.
Massimiliano GrassiMedical Oncology Unit 1, Ospedale Policlinico San Martino IRCCS, University of Genoa, Genoa, Italy.
Robert S BenjaminDepartment of Sarcoma Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Italy.
Javier-Martin BrotoMedical Oncology Department, University Hospital Virgen del Rocio, and Institute of Biomedicine, Sevilla, Spain.
Giovanni GrignaniDivision of Medical Oncology, Candiolo Cancer Institute, FPO - IRCCS, Candiolo, Torino, Italy.ORCID 0000-0001-5515-569X
Robin L JonesSarcoma Unit, Royal Marsden NHS Foundation Trust/ Institute of Cancer Research, Chelsea, London, United Kingdom.ORCID 0000-0003-4173-3844
Akira KawaiDepartment of Musculoskeletal Oncology, National Cancer Center Hospital, Tokyo, Japan.ORCID 0000-0003-2116-586X
Andrzej TysarowskiPathology department, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland.
Luigi MarianiUnit of Clinical Epidemiology and Trial Organization, IRCCS Fondazione Istituto Nazionale Tumori, Milano, Italy.
Paolo G CasaliDepartment of Medical Oncology, IRCCS Fondazione Istituto Nazionale Tumori, Milano, Italy.
Silvia StacchiottiDepartment of Medical Oncology, IRCCS Fondazione Istituto Nazionale Tumori, Milano, Italy.ORCID 0000-0002-1742-8666
Fondazione IRCCS Istituto Nazionale dei Tumori · ITCandiolo Cancer Institute · ITInstituto de Biomedicina de Sevilla · ESNational Institute of Oncology · HURoyal Marsden NHS Foundation Trust · GBThe University of Texas MD Anderson Cancer Center · USAix-Marseille Université · FRChris O’Brien Lifehouse · AUInstitut Bergonié · FRIstituto Oncologico Veneto · ITLeiden University Medical Center · NLNational Taiwan University Hospital · TWOspedale di Prato Santo Stefano · ITOspedale Policlinico San Martino · ITP.A. Hertzen Moscow Oncology Research Institute · RURadboud University Nijmegen · NLSorbonne Université · FRUniversità Campus Bio-Medico · ITUniversité de Lille · FRUniversity of Milan · IT

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundThis 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.

methodsTwenty 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.

resultsOverall, 73 patients were included; 21 had more than one treatment. Thirty-three patients received anthracyclines regimens, achieving 1 (3%) partial response (PR), 25 (76%) stable disease (SD), 7 (21%) progressive disease (PD). The median (m-) PFS and m-OS were 5.5 and 14.3 months respectively. Eleven patients received paclitaxel, achieving 1 (9%) PR, 6 (55%) SD, 4 (36%) PD. The m-PFS and m-OS were 2.9 and 18.6 months, respectively. Twelve patients received pazopanib, achieving 3 (25%) SD, 9 (75%) PD. The m-PFS and m-OS were.2.9 and 8.5 months, respectively. Fifteen patients received INF-α 2b, achieving 1 (7%) PR, 11 (73%) SD, 3 (20%) PD. The m-PFS and m-OS were 8.9 months and 64.3, respectively. Among 27 patients treated with other regimens, 1 PR (ifosfamide) and 9 SD (5 gemcitabine +docetaxel, 2 oral cyclophosphamide, 2 others) were reported.

conclusionSystemic therapies available for advanced sarcomas have limited activity in EHE. The identification of new active compounds, especially for rapidly progressive cases, is acutely needed.

Indexed as

AdultAntineoplastic Combined Chemotherapy ProtocolsFemaleFollow-Up StudiesHemangioendothelioma, EpithelioidHumansInternational AgenciesMaleMiddle AgedPrognosisRetrospective StudiesSarcomaSurvival Rateanthracyclinechemotherapyepithelioid haemangioendotheliomainterferonpaclitaxelpazopanib

Identifiers

PMID33713582
PMCPMC8026938
OpenAlexW3139450089

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Registered trials

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.