Abstract
Objective: providing the rarity of malignant and intermediate vascular tumours (MVTs, IVTs) in children little is known about their clinical course, optimal treatment and variables predicting survival.
Material and methods: 32 children with MIVTs (14 angiosarcomas-AS, 5 epithelioid haemangioendotheliomas-EHE and 13 IVTs), registered in Polish and German Paediatric Soft Tissue Sarcomas (STS) Study Groups, treated with CWS-81, −86, −91 and −96 protocols.
Results: AS presented with advanced disease (84%), deep-seated T2 invasive tumours (71%), > 5cm in diameter (64%). Primary excision (PE) was incomplete in all and response to CHT/RTX disappointing. Nine/14 children entered CR; however all relapsed and, except one, died of disease. EHE and IVTs presented mainly in local stages (66,7%) and tumours > 5cm (72%). Complete PE was feasible in 30% and response to CHT/IFN poor in half. 16 patients entered CR, but six relapsed and, except one, died of disease.
In multivariate analysis male gender, AS histology, tumour size > 5cm, T2 invasiveness and lack of CR after Ist line therapy were independent predictors of poorer 5-year-OS, while AS histology and T2 invasiveness – of inferior 5-year-EFS. Radicality of PE was an independent prognostic factor for survival in univariate but not multivariate analysis.
Conclusions:
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Current WHO classification has placed EHE in MVTs, however it may behave similarly rather to IVTs than AS.
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Male gender, AS histology, tumour size > 5cm, T2 invasiveness and lack of remission after Ist line therapy were independent predictors of inferior 5-year-OS while AS histology and T2 invasiveness – of inferior 5-year-EFS.
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In contrast to most studies, radicality of PE was not an independent prognostic factor for survival.
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High rate of patients not responding to classic CHT and developing metastatic recurrences suggests an urgent need for modification of systemic therapy.
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Problem of efficient therapy of childhood AS is the most appalling.
Correspondence should be addressed to Professor Stefan Bielack, Olgahospital, Klinikum Stuttgart, Bismarkstrasse 8, D-70176 Stuttgart, Germany. Email: s.bielack@klinikum_stuttgart.de
