Long‐term outcome of patients with monostotic Ewing's sarcoma treated with combined modality

Abstract

One hundred and twenty-one consecutive patients with monostotic Ewing's sarcoma (ES) were treated according to three consecutive combined modality programs from 1974 to 1986. Their 3-year progression free survival (PFS) rate from diagnosis of 59% was identical to the event free survival (EFS) rate, since all the 50 events occurring within 3 years from diagnosis were tumor recurrences. Primary tumor was treated with radiotherapy in 75 cases, surgical resection plus radiotherapy in 38, and radical surgery in 8. Chemotherapy was given to all patients and each program included adriamycin, vincristine, and cyclophosphamide +/- dactinomycin. Median follow-up was 12 years, ranging from 6 to 19 years. The PFS rate decreased to 49% at 6 years and plateaued at 46% after the 7th year from diagnosis, even though some relapses were observed as late as 14 years from diagnosis. Second malignancies developed in 7 patients free from progressive ES and were represented by osteogenic sarcoma in previously irradiated bone in 4 cases and by breast carcinoma in 3. No other event but tumor relapse or second malignancy occurred in this series. EFS rate was 47% at 6 years and 39% at 12 years, further decreasing in the following years because of a number of late events. A continuous PFS longer than 7 years may be consistent with cure in the majority of patients with monostotic ES. However, these patients should be followed indefinitely because of risk of second malignancies.

Very late local recurrence of Ewing's sarcoma – can you ever say 'cured'? A report of two cases and literature review

“…When metastatic disease is present at diagnosis, the 5-year survival rate is less than 25%. 4,9 Many studies have reported the pattern of local recurrence after disease remission and its relation to the prognostic factors 2,[4][5][6]10 and in the majority of these studies, tumour relapse (local recurrence and/or metastases) occurred between 2-10 years after starting initial treatment. [4][5][6] Bacci et al 4 reported therapy and survival rates after recurrence of treated non-metastatic Ewing's sarcoma in 195 patients, the mean relapse time was 27.3 months with one patient developing local recurrence 222 months (18.5 years) after initial treatment.…”

“…Gasparini et al 10 described the long-term outcome in 121 patients with monostotic Ewing's sarcoma treated with combined modality therapy. The mean follow-up in this study was 12 years, with one patient developing local recurrence at 14 years.…”

“…This variability is present in all age groups and in both genders. However, as the method of local treatment (surgery and/or radiotherapy) has been reported to affect long-term outcome, 10 this may also be related to the duration of the remission state.…”

Long-Term Follow-Up and Prognostic Factors in Ewing’s Sarcoma

“…In 73 cases, the histologic specimens were considered adequate for subclassification by means of light microscopy; 54 thereof met the criteria Sex 22 Oncology 1998;55: [20][21][22][23][24][25][26] Aparicio/Muná rriz/Pastor/Vera/Castel/ Aparisi/Montalar/Badal/Gó mez-Codina/ Herranz of typical Ewing's sarcoma, while the remaining 19 (26%) were classified as atypical forms. Presence of rosettes, cellular pleomorphism, and increased number of mitoses (12/high-power field) were the most common features for inclusion in this subgroup.…”

“…Ninety-six patients received standard multiagent chemotherapy protocols (with the remaining 20 cases thus being excluded from the multivariate analysis). The median number of chemotherapy courses per patient was 10 (range, [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20].…”