Abstract
We reviewed the treatment and clinical outcome of 32 consecutive patients with Ewing’s sarcoma who presented with or developed pathological fracture after biopsy between 1984 and 2004. The minimum follow-up was 18 months. The mean age at diagnosis was 20 years (5 – 51). There were 18 males and 14 females. All patients were newly diagnosed and had localized disease at the time of diagnosis. 21 patients presented with pathological fracture while 11 patients developed fracture during the course of chemotherapy. The femur was the most common location in 15 patients.
All the patients had chemotherapy according to the protocol current at the time of treatment. 6 patients had radiotherapy alone while 26 patients underwent surgical excision and reconstruction. Of the patients who had surgery, 7 patients had adjuvant radiotherapy. Fracture healing was the norm after pre-operative chemotherapy. Surgical margins were wide in 17 patients, marginal in 4 and intralesional in 3 patients.
Local recurrence developed in one patient (3%). Metastases occurred in 12 patients (37%). At the time of review 16 patients were free of disease, 3 were alive with disease and 13 patients had died of disease. The cumulative 5 year metastases free and overall survival in all the patients was 58% and 61 % respectively and similar to patients with Ewing’s sarcoma without fracture treated at our centre. The prognosis of patients who presented with fracture was exactly similar to those who developed fracture in the course of treatment.
We conclude that limb preserving surgery is perfectly safe in patients with Ewing’s sarcoma who have associated pathological fracture and survival is not in any way compromised. Survival of patients who present with fracture is similar to those who develop fracture in the course of treatment. The exact role of adjuvant radiotherapy in these patients needs to be clarified.
Correspondence should be addressed to Ms Larissa Welti, Scientific Secretary, EFORT Central Office, Technoparkstrasse 1, CH-8005 Zürich, Switzerland