Evidence map›Paper›PMID 42435082›Full record

ArticleJournal of cancer research and clinical oncology2026

Characteristics of patients with epithelioid hemangioendothelioma (EHE): a retrospective analysis of the Charité- Universitätsmedizin Berlin.

Jana K Striefler, F Brandes, A Strönisch, M Schmiester, A Dörr, L E Heil Olaizola, J Benckert, J Ihlow, A Jarosch, M Stiller and 10 more

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Article in Journal of cancer research and clinical oncology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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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.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

0 citing papers in PubMed.

No citing paper in PubMed yet.

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

20 authors.

Jana K Striefler *Department of Internal Medicine II, Oncology/Hematology/BMT/Pneumology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. j.striefler@uke.de.ORCID https://orcid.org/0000-0002-0612-3023
F Brandes *Inselspital, Universitätsspital Bern, Universitätsklinik für Medizinische Onkologie, Bern, Schweiz.
A StrönischDepartment of Hematology, Oncology, and Tumor Immunology, Charité-Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Berlin Institute of Health, Campus Virchow-Klinikum, Berlin, Germany.
M SchmiesterDepartment of Hematology, Oncology, and Tumor Immunology, Charité-Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Berlin Institute of Health, Campus Virchow-Klinikum, Berlin, Germany.
A Dörr, Alexianer St. Hedwig Krankenhaus, Berlin, Germany.
L E Heil OlaizolaDepartment of Hematology, Oncology, and Tumor Immunology, Charité-Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Berlin Institute of Health, Campus Virchow-Klinikum, Berlin, Germany.
J BenckertDepartment of Hepatology and Gastroenterology, Charité-Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Berlin Institute of Health, Humboldt-Universität zu Berlin, Campus Virchow-Klinikum and Campus Charité Mitte, Berlin, Germany.
J IhlowCharité-Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Berlin Institute of Health, Institute of Pathology, Humboldt-Universität zu Berlin, Campus Mitte, Berlin, Germany.
A JaroschCharité-Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Berlin Institute of Health, Institute of Pathology, Humboldt-Universität zu Berlin, Campus Mitte, Berlin, Germany.
M StillerDepartment für Diagnostik, Institut für Pathologie, Universitätsklinikum Leipzig AöR, Leipzig, Germany.
M von LaffertDepartment für Diagnostik, Institut für Pathologie, Universitätsklinikum Leipzig AöR, Leipzig, Germany.
S RoohaniDepartment of Radiation Oncology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany.
D KaulDepartment of Radiation Oncology, Health and Medical University Potsdam, Potsdam, Germany.
R ÖllingerDepartment of Surgery, Charité-Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Berlin Institute of Health, Humboldt-Universität zu Berlin, Campus Virchow-Klinikum, Berlin, Germany.
S WittenbergCharité-Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Berlin Institute of Health, Centre for Musculoskeletal Surgery, Humboldt-Universität zu Berlin, Campus Virchow-Klinikum, Berlin, Germany.
D RauCharité-Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Berlin Institute of Health, Centre for Musculoskeletal Surgery, Humboldt-Universität zu Berlin, Campus Virchow-Klinikum, Berlin, Germany.
S MärdianKlinik für Unfall-, Hand- und Wiederherstellungschirurgie, Chirurgische Klinik und Poliklinik, Universitätsmedizin Rostock, Rostock, Germany.
F TackeDepartment of Hepatology and Gastroenterology, Charité-Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Berlin Institute of Health, Humboldt-Universität zu Berlin, Campus Virchow-Klinikum and Campus Charité Mitte, Berlin, Germany.
L BullingerDepartment of Hematology, Oncology, and Tumor Immunology, Charité-Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Berlin Institute of Health, Campus Virchow-Klinikum, Berlin, Germany.
A FlörckenDepartment of Hematology, Oncology, and Tumor Immunology, Charité-Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Berlin Institute of Health, Campus Virchow-Klinikum, Berlin, Germany. anne.floercken@charite.de.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundEpithelioid hemangioendothelioma (EHE) represents an extremely uncommon vascular sarcoma subentity. The clinical characteristics vary from low grade localized to high grade systemic disease and treatment ranges from active surveillance versus intensive therapeutic strategies. Due to its rarity, a standardized clinical management including an appropriate therapeutic approach has not yet been defined.

methodsPatients with EHE presenting at the Charité-Universitätsmedizin Berlin between 1997 and 2022 were included in the analysis. A retrospective database was established comprising demographic specifications, treatment, pathological features (including TFE3 expression level) and prognosis. Our single-centre data was then compared to previously published cohorts.

resultsThe cohort included n = 41 patients, with n = 16 men (39%) and n = 25 women (61%) with a median age of 53 years (range 20-88). The patients had limited comorbidities (median Charlson Comorbidity Index, CCI: 3) and a good performance status (median ECOG: 1). Median overall survival was not reached, with only n = 3 patients (7%) showing an aggressive course of disease (progression-free survival (PFS) ≤ 28 days). Isolated liver involvement was the predominant clinical presentation, observed in n = 26 patients (63%). At initial diagnosis, n = 17 (41%) patients showed systemic symptoms (e.g. anemia, weight loss) and n = 12 (29%) already had metastatic disease. Surgery was performed for therapeutic and/or diagnostic purposes in n = 20 (49%) patients, whereas only n = 5 (12%) received chemotherapy. In n = 2 of the latter, disease stabilization was achieved. Liver transplantation was performed in n = 5 (12%) patients and was associated with persisting disease-free survival. Molecular genetic data was retrieved in n = 21 (51%) patients regarding WWTR1-CAMTA1 fusion, and in n = 18 (44%) patients regarding TFE3 positivity. In 67% of analyzed samples, CAMTA1 fusion was found. In contrast, TFE3 expression was observed in 22% only. Both CAMTA1 fusion and TFE3 positivity were observed in n = 3 patients (18%).

conclusionsThis analysis of EHE patients treated at Charité-Universitätsmedizin Berlin mirrors published cohorts regarding clinical behavior and OS. The observed clinical heterogeneity underlines the necessity for personalized therapeutic strategies in this rare disease. Molecular characterization is increasingly recognized as a component of contemporary clinical care, as it may identify molecular subclasses associated with aggressive disease and guide management strategies in the future. Moreover, standardized molecular profiling also enables evaluation of patient eligibility for clinical trials.

Indexed as

Hemangioendothelioma, EpithelioidAdultAgedAged, 80 and overBasic Helix-Loop-Helix Leucine Zipper Transcription FactorsBiomarkers, TumorFemaleHumansMaleMiddle AgedPrognosisRetrospective StudiesYoung AdultBasic Helix-Loop-Helix Leucine Zipper Transcription FactorsBiomarkers, TumorTFE3 protein, humanEpithelioid hemangioendotheliomaPrognostic markersSystemic therapy

Identifiers

PMID42435082
PMCPMC13369096

What Socratic holds

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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 Socratic graph.