Synovial sarcoma (SS) of childhood is considered chemoresponsive, what frequently leads to conservative surgical approach. Aim of the report is to assess whether that approach is sufficient and what are other clinical factors influencing the outcome. Patients: 23 children (aged 3 months – 17 years) treated in 5 cooperating centres for nonmetastatic SS located in the limbs(Fu>
36 months). Treatment: primary or secondary resections, chemotherapy and radiotherapy (in case of resection R1 or R2 or relapse). Locations of tumours: thigh/6, shank/1, popliteal fossa/4, cubital fossa/3, axilla/2, forearm/2, foot/3, arm/1, hand/1. Primary R0 achieved in 3, R1/5, R2/3. Twelve after initial biopsy and 3 after primary R2 were submitted to chemotherapy and secondary surgery. 12/15 those resections were R0, 3/15 R1. Two of them underwent mutilating resections. Results: 11 pts are in CR. 12 relapsed (7 local, 5 metastatic). Initial locations of the relapsing tumours were around joints/4, foot/hand 4, thigh 3, arm 1. Seven of them died despite aggressive re-treatment, 2 are alive with disease, 3 are in second CR. Quality of resections (primary or secondary) in 7 locally relapsing pts were R0 in 3, R1 in 3 and biopsy only /1 (CR after CHT alone, refused local treatment). Of 2 submitted to mutilating resections, 1 relapsed in the lungs (2nd CR after re-treatment and metastasectomy).
R0 doesn’t prevent from local relapse (3 of 7 pts relapsed after R0 vs 4/7 with R1). Mutilating resections (amputations) were unfrequent (2) and were not followed by local relapses. One metastatic relapse occurred and was finally cured. Locations at risk seemed joints and distal parts of limbs (8/12 of the relapsing pts). Relapsed implied weak chance for long-term second CR (3/12).
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.
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.
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.
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.
