Abstract
Liposarcoma (LPS) is among the most common soft tissue sarcoma (STS) in adults, accounting for > 10% of all STS. In children and adolescents, however, LPS are a rarity. Limited data about best treatment of pediatric LPS are derived from the scarce single-centre reports encompassing no more than a dozen patients.
Between 10/1980-6/2008, 18 of > 3,500 patients < 19 years with sufficient clinical data registered with CWS in Germany and Poland had a first diagnosis of LPS confirmed by reference pathologic review.
Median age was 14 years, median follow-up for survivors as of 2/2009 seven years. Sixteen patients had localized, two metastatic LPS at diagnosis. Lymphnodes were affected in a single case. The most frequent primary site were the limbs (n=11), the remaining seven were trunk tumours (abdomen n=4, thorax n=3). 10/18 primary tumours were > 5cm. Thirteen LPS were completely resected at best surgery, and microscopically residual disease remained in two more tumours. Six individuals received radiation with a median dose of 45Gy, including one of the two R1-resected patients. Nine patients received multiagent chemotherapy (only two of them since 1996 onwards). Response to induction treatment could be assessed in three of these nine individuals, but tumour volume regression occured in a single case only. Four patients died of disease, among them two of the three patients who did not achieve a CR with primary treatment. Two relapses (one combined, one metastatic), both involving the lungs, occurred one years after diagnosis and these patients were not salvaged. Actuarial 5-year EFS and OS survival rates were 69±23 and 81±20%, respectively.
LPS account for < 0.1% of childhood STS. The golden standard of treatment and key to cure is complete surgical excision. The role of radiation and/or chemotherapy remains unclear, but both modalities do not appear to be indicated in completely resected, localized tumours.
Correspondence should be addressed to Professor Stefan Bielack, Olgahospital, Klinikum Stuttgart, Bismarkstrasse 8, D-70176 Stuttgart, Germany. Email: s.bielack@klinikum_stuttgart.de
