Abstract
Sarcomas account for 10% of cancers occurring in 15–24 year olds. Within this group there is considerable clinical and biological heterogeneity and incomplete understanding of optimal treatments.
Most clinical research attention has focused on the management of bone sarcomas, particularly osteosarcoma and Ewing’s tumours. Several factors have been studied which consistently identify patient groups with differing outcomes. Age at diagnosis appears to affect prognosis in Ewing’s tumours but less obviously in localised extremity osteosarcoma. Any underlying biological or treatment delivery variables which may explain these observations have yet to be elucidated. Whether different treatment approaches for bone sarcomas should be adopted for teenagers and young adults (TYA) is unclear and will require systematic prospective evaluation.
Soft tissue sarcomas affect all ages. The numerous histiotypes are not evenly distributed across all age ranges. In the progression from childhood through adolescence to adulthood, rhabdomyosarcoma is replaced as the commonest subtype by the many different subtypes recognised by adult oncologists. There is little guidance about appropriate management of ‘adult-type’ soft tissue sarcomas occurring in TYA and this group have not been systematically studied. Their representation within clinical trials may be biased towards those with adverse features. There is considerable variation in practice particularly regarding the use of adjuvant chemotherapy. Few studies address whether specific approaches to treatment are appropriate for TYA with soft tissue sarcoma.
In the future, biologists and clinicians familiar with sarcomas affecting TYA and adults need to work together to share understanding and to design rational treatment programmes aimed at improving outcomes for TYA.
Correspondence should be addressed to Professor Stefan Bielack, Olgahospital, Klinikum Stuttgart, Bismarkstrasse 8, D-70176 Stuttgart, Germany. Email: s.bielack@klinikum_stuttgart.de
