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Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_III | Pages 463 - 463
1 Jul 2010
Bien E Kazanowska B Godzinski J Dantonello T Adamkiewicz-Drozynska E Balcerska A Madziara W Rybczynska A Nurzynska-Flak J Solarz E Kurylak A Zalewska-Szewczyk B Koscielniak E
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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:

Current WHO classification has placed EHE in MVTs, however it may behave similarly rather to IVTs than AS.

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.

In contrast to most studies, radicality of PE was not an independent prognostic factor for survival.

High rate of patients not responding to classic CHT and developing metastatic recurrences suggests an urgent need for modification of systemic therapy.

Problem of efficient therapy of childhood AS is the most appalling.