Ewing's sarcoma: long-term follow-up in 49 patients treated from 1967 to 1989

Review of long-term results of therapy for Ewing's sarcoma in terms of survival, local tumor control, distant failure and complications rates.

Retrospective review of the records of patients with Ewing's sarcoma of bone and soft tissues treated at The Prince of Wales Children's and Prince of Wales Hospitals, Sydney, between 1967 and 1989 and followed-up to July 1991.

There were 49 patients with median age 16 years (range 3-33 years) and average potential follow-up time 12.3 years (range 2-24 years). Forty patients presented with localized disease (three with regional lymph node involvement) and nine with distant metastases. Local therapy for the primary was by amputation in three patients, by resection and postoperative radiotherapy in five, and by definitive radiotherapy in 41 (median dose 50 Gy). Forty-four patients received adjuvant multi-agent chemotherapy. The overall actuarial survival rate was 33% (SE = 7%) at 5 years and 30% (SE = 7%) at 10, 15, and 20 years. The factors predictive of shorter survival were distant metastases at diagnosis (p = 0.036) and older age (p = 0.025). The actuarial local control rate for all 49 patients was 75% (SE = 8%) at 5, 10, 15, and 20 years. The only factor predictive of local failure was an inadequate target volume irradiated (p = 0.003). In 40 patients who presented with localized disease only, the actuarial rate of freedom from distant failure at 5 years was 44% (SE = 8%) and at 10, 15, and 20 years was 40% (SE = 8%). Seven patients experienced severe or fatal complications (defined as requiring investigation and treatment in hospital), namely stress fracture in two, fatal osteogenic sarcoma in one, fatal cardiotoxicity in one and severe hemorrhagic cystitis in three. The rate for severe or fatal complications at 5 years was 19% (SE = 8%), at 10 years was 29% (SE = 12%) and at 15 and 20 years was 53% (SE = 21%).

Survival to 5 years appears to confer probable cure and one third of our patients have achieved this. Long-term follow-up also reveals that an increasing number of patients experience treatment-related complications, the majority of which, however, can be corrected.