Oral etoposide in relapsed or refractory Ewing sarcoma: a monoinstitutional experience in children and adolescents



To assess the efficacy and toxicity of low-dose oral etoposide (VP) 16 in relapsing/refractory Ewing sarcoma.


The records of all patients treated at our department between 1989 and 2012 for relapsing/refractory Ewing sarcoma who received oral VP-16 were analyzed. The dose was 40 mg/m2 daily for 21 consecutive days in every 28. Response was assessed after 2/3 cycles according to Response Evaluation Criteria in Solid Tumors 1.0.


A total of 46 of 58 patients completed at least 2 cycles; 12 suspended the treatment earlier due to rapid disease progression. The patients’ median age at diagnosis was 14 years and 25/58 had metastatic disease. All patients received intensive polychemotherapy including VP-16 IV as first- (n = 53) or second-line (n = 5) treatment; 21/58 had myeloablative regimens with peripheral blood stem cell rescue, and 1 underwent allogeneic stem cell transplantation. Oral VP-16 was prescribed as 2nd-, 3rd-, and 4th-line treatment for 19, 27, and 12 patients, respectively. The cycles administered totaled 241 (median 3, mean 4 per patient; range 1-14). A total of 46 of 58 patients were evaluable: 11 responded (9 partial remission, 1 very good partial remission, 1 complete remission) and 10 were stable, the response lasting a mean of 8 months. Hematologic toxicity G3/G4 (in 164/241 evaluable cycles) occurred in 15%, 16%, and 11% of cycles for leukocytes, hemoglobin, and platelets, respectively. There were 5 cases of pneumonia. Two patients developed secondary leukemia after receiving 12 and 14 cycles.


Low-dose oral VP-16 may be suitable in a palliative setting with an acceptable toxicity. The risk of secondary leukemia is in line with reports in the literature.

Tumori 2016; 102(1): 84 - 88




Marta G. Podda, Roberto Luksch, Nadia Puma, Lorenza Gandola, Carlo Morosi, Monica Terenziani, Andrea Ferrari, Michela Casanova, Filippo Spreafico, Cristina Meazza, Serena Catania, Elisabetta Schiavello, Veronica Biassoni, Stefano Chiaravalli, Maura Massimino

Article History


Financial support: None.
Conflict of interest: None.

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  • Pediatric Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan - Italy
  • Pediatric Radiotherapy Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan - Italy
  • Radiology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan - Italy

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