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Background <p>Epithelioid 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.</p> Methods <p>Patients 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.</p> Results <p>The cohort included <i>n</i> = 41 patients, with <i>n</i> = 16 men (39%) and <i>n</i> = 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 <i>n</i> = 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 <i>n</i> = 26 patients (63%). At initial diagnosis, <i>n</i> = 17 (41%) patients showed systemic symptoms (e.g. anemia, weight loss) and <i>n</i> = 12 (29%) already had metastatic disease. Surgery was performed for therapeutic and/or diagnostic purposes in <i>n</i> = 20 (49%) patients, whereas only <i>n</i> = 5 (12%) received chemotherapy. In <i>n</i> = 2 of the latter, disease stabilization was achieved. Liver transplantation was performed in <i>n</i> = 5 (12%) patients and was associated with persisting disease-free survival. Molecular genetic data was retrieved in <i>n</i> = 21 (51%) patients regarding WWTR1-CAMTA1 fusion, and in <i>n</i> = 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 <i>n</i> = 3 patients (18%).</p> Conclusions <p>This 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.</p>

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

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  • 发表日期：

    2026年7月11日

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  • Jana K. Striefler,
  • F. Brandes,
  • A. Strönisch,
  • M. Schmiester,
  • A. Dörr,
  • L. E. Heil Olaizola,
  • J. Benckert,
  • J. Ihlow,
  • A. Jarosch,
  • M. Stiller,
  • M. von Laffert,
  • S. Roohani,
  • D. Kaul,
  • R. Öllinger,
  • S. Wittenberg,
  • D. Rau,
  • S. Märdian,
  • F. Tacke,
  • L. Bullinger,
  • A. Flörcken

摘要

Background

Epithelioid 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.

Methods

Patients 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.

Results

The 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%).

Conclusions

This 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.