Reviewers: Kenneth Blank, MD
Source: Journal of Clinical Oncology, December 1998, Vol 16, No. 12, p 3736
Ewing's sarcoma is a bone cancer, which typically afflicts teenagers. The median age at diagnosis is about 16 years of age. However, unlike other childhood tumors such as neuroblastoma that occur almost exclusively in children, Ewing's sarcoma can occur in adults. Depending on the age at which one chooses to define an adult, as many as 50% of the cases of Ewing's sarcoma occur in adults.
Reviews of adult cases of Ewing's sarcoma have shown that the disease carries a worse prognosis when compared to childhood Ewing's sarcoma. The reasons for this are varied and include the ability to treat children more aggressively and the propensity for children to present before the sarcoma has spread. Because of its relative rarity, data on the natural history of Ewing's sarcoma is difficult to obtain. In the December issue of the Journal of Clinical Oncology investigators from the Institute Gustave-Roussy in Paris report on their experience treating adult patients with Ewing's sarcoma.
The case records of one hundred and eighty two consecutive patients fifteen years or older with Ewing's sarcoma treated at the Institute Gustave-Roussy between 1982 and 1992 are reported. Patient characteristics revealed a male predominance (1.6:1 male: female ratio), a median age of 21.5years old, with the oldest patient being 55 years old. The two most common sites of disease were the bones of the legs and pelvis, accounting for greater than two-thirds of all cases. The primary tumor measured greater than 10cm in 59% and invaded out of the bone into the adjacent soft tissue in the large majority. Distant metastasis was present at presentation in 53 patients (29%) with lung and bone being the two most common sites.
Treatment varied over time and according to time of referral to Institute Gustave-Roussy. Surgical extirpation was performed in 86 cases and one patient underwent amputation of an extremity. Surgical removal of metastasis was not performed. All but two patients received chemotherapy, often as the first component of therapy. Chemotherapy was anthracycline based. Radiotherapy was administered to 81% of patients with total dose being less than 50Gray in 30% and greater than 60Gray in 40%.
Analysis of survival and prognostic factors was performed with a median follow-up time of 66 months. For all patients the five year overall survival was 41%. Univariate analysis revealed several patient and tumor characteristics to portend better survival and these include lack of distant metastasis, lack of invasion into surrounding soft tissue, tumor site in the extremities and a tumor size less than 10cm. In patients with localized disease (no metastasis) the five year overall survival was 54%. Multivariate analysis determined metastasis at presentation, pelvic primary and tumor size greater than 10cm to be independent poor prognostic factors. Those patients presenting with distant metastasis had a five year overall survival of 9%. Of note, late relapses occurring after five years occurred in 10%. Based on the information reported the authors conclude that the natural history and prognosis of Ewing's sarcoma in adults does not differ from that in children.