| Popis: |
We report a series of 70 consecutive patients (pts) treated on the Adult Sarcoma Unit since 1980 for the Ewing's group of tumours. All pts had a diagnosis of Ewing's sarcoma, Askin's tumour or PNET confirmed after local histology review. Characteristics: median âge 24 yrs (14–65); 46 M, 24 F; 12 upper limb, 21 lower limb, 3 head and neck, 11 chest wall, 11 pelvis and 7 other primary sites. 5 pts presented with disseminated disease and no definite primary. Chemotherapy was predominantly IVAD3 (30 cases), VAC in 7 cases, and a dose intensive regimen of ifosfamide, etoposide and cyclophosphamide + G-CSF in 5 cases. Surgery and radiotherapy were used for local control. 5 pts had high dose chemotherapy with ABMT or PBSC rescue. 15 pts, were lost to follow up after a median of 7 months. Of the remainder 28 (51%) hâve died, 4 (7%) are alive with residuai disease, 3 (5%) are on treatment for relapsed disease and 6 (11%) have yet to complete induction therapy. 14 patients (25%) are alive and disease free with a median follow-up of 33 mths (10–102 months). The poor survival in this group is explained by tumour bulk > 100 ml in the majority of pts. There is clearly a need for further studies to determine the raie of dose intensification and high dose chemotherapy with PBSC rescue, which are currently underway. |
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