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Factors Predicting Survival in Childhood Malignant and Intermediate Vascular Tumors

Retrospective Analysis of the Polish and German Cooperative Paediatric Soft Tissue Sarcoma Study Groups and Review of the Literature

  • Pediatric Oncology
  • Published:
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Abstract

Background

The rarity of malignant and intermediate vascular tumors in children means that little is known about their clinical course, optimal treatment, and variables predicting survival.

Methods

A total of 32 children with malignant vascular tumors (14 angiosarcomas [AS], 5 epithelioid hemangioendotheliomas, and 13 intermediate vascular tumors, including other hemangioendotheliomas plus adult-type hemangiopericytomas), registered in the German and Polish Paediatric Soft Tissue Sarcomas Study Groups, were treated following the Cooperative Weichteilsarkom Studiengruppe (CWS)-81, -86, -91, and -96 protocols.

Results

Male sex, AS histology, tumor size >5 cm, and T2 invasiveness were independent predictors of inferior 5-year overall survival, while AS histology and T2 invasiveness were predictors of inferior 5-year event-free survival. AS histology was the most important negative prognostic factor for overall survival and event-free survival. Completeness of primary tumor excision was a good prognostic factor for survival in univariate, but not multivariate, analysis. Local therapy (radiotherapy and delayed surgery) were provided to the minority of patients (28% and 38%, respectively) late in the course of disease (after a mean of 9 and 6 months, respectively) and did not prevent local relapses. Response to systemic treatment was poor (44%) and did not prevent local and distant relapses.

Conclusions

The clinical course and outcome in childhood epithelioid HE seems to be similar to intravascular tumors and less aggressive than AS. RTX and delayed surgery should be performed more frequently and earlier in the disease course. An urgent need for modification of systemic therapy is needed because of the development of many metastatic and/or combined relapses and poor response to classic chemotherapy. The problem of effective therapy for childhood AS is the most appaling: 13 of 14 patients died of progression despite multimodal treatment.

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Authors and Affiliations

  1. Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdansk, Gdansk, Poland

    Ewa Bien MD, PhD, Elzbieta Adamkiewicz-Drozynska MD, PhD, Anna Balcerska MD, PhD & Malgorzata Krawczyk MD

  2. Department of Bone Marrow Transplantation, Oncology and Haematology, Medical University of Wroclaw, Wroclaw, Poland

    Bernarda Kazanowska MD, PhD

  3. Paediatrics 5 (Oncology, Haematology, and Immunology), Olgahospital, Stuttgart, Germany

    Tobias Dantonello MD & Ewa Koscielniak MD, PhD

  4. Department of Paediatric Surgery, Medical University of Katowice, Katowice, Poland

    Wojciech Madziara MD, PhD

  5. Department of Paediatric Surgery, Medical University of Poznan, Poznan, Poland

    Aleksandra Rybczynska MD, PhD

  6. Department of Oncology and Haematology, Medical University of Lublin, Lublin, Poland

    Joanna Nurzynska-Flak MD, PhD

  7. Department of Oncology and Haematology, Medical University of Bialystok, Bialystok, Poland

    Elzbieta Solarz MD, PhD

  8. Department of Oncology and Haematology, Medical University of Bydgoszcz, Bydgoszcz, Poland

    Andrzej Kurylak MD, PhD

  9. Department of Paediatrics, Haematology, Oncology and Diabetology, Medical University of Lodz, Lodz, Poland

    Beata Zalewska-Szewczyk MD, PhD

  10. Department of Patomorphology, Medical University of Gdansk, Gdansk, Poland

    Ewa Izycka-Swieszewska MD, PhD

  11. Department of Paediatric Surgery, Marciniak Hospital, Wroclaw, Poland

    Malgorzata Rapala

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  1. Ewa Bien MD, PhD
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Correspondence to Ewa Bien MD, PhD.

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Bien, E., Kazanowska, B., Dantonello, T. et al. Factors Predicting Survival in Childhood Malignant and Intermediate Vascular Tumors. Ann Surg Oncol 17, 1878–1889 (2010). https://doi.org/10.1245/s10434-010-0991-6

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