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A study of docetaxel and irinotecan in children and young adults with recurrent or refractory Ewing sarcoma family of tumors

Jong Hyung Yoon1, Mi Mi Kwon1, Hyeon Jin Park1, Seog Yun Park12, Kun Young Lim3, Jungnam Joo4 and Byung-Kiu Park1*

Author Affiliations

1 Center for Pediatric Oncology, National Cancer Center, 323 Ilsan-ro, Ilsandong-gu, Goyang-si, Gyeonggi-do 410-769, Korea

2 Department of Pathology, National Cancer Center, Goyang, Korea

3 Department of Radiology, National Cancer Center, Goyang, Korea

4 Biometric Research Branch, National Cancer Center, Goyang, Korea

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BMC Cancer 2014, 14:622  doi:10.1186/1471-2407-14-622

Published: 28 August 2014



Patients with Ewing sarcoma family of tumors (ESFT) who are resistant even to salvage chemotherapy, have dismal prognoses and few therapeutic options. Because the docetaxel/irinotecan (DI) combination has not been previously evaluated in ESFT, we prospectively evaluated its use in patients with recurrent or refractory ESFT.


Patients aged <30 years with ESFT, who failed ≥ third-line therapy, were eligible. They received docetaxel 100 mg/m2 intravenously on day 1, and irinotecan 80 mg/m2 on days 1 and 8, of a 21-day cycle up to 15 cycles or until disease progressed. The primary objective was objective response rate (ORR); secondary objectives were progression-free survival (PFS) and safety.


We enrolled nine patients (median age: 13 years); four were male. Two patients had recurrent disease and seven had progressive disease. This group had undergone a median of four prior chemotherapy regimens (range: 3-6), and received a total of 51 DI cycles (median: three cycles/per person; range: 1-15 cycles). The nine patients showed one complete response (CR), two partial responses (PRs), one stable disease, and five progressive diseases, for an ORR (CR + PR) of 3/9 (33.3%). Two patients with PR achieved CR with subsequent surgery. Overall median PFS was 2.2 months (range: 0.5-16.9 months). All nine patients had grade 4 neutropenia (100%); grade 3 diarrhea or grade 2/3 neuropathy each occurred in two patients (22%). All toxicities were manageable without serious morbidities or treatment-related mortality.


The DI combination may be effective and tolerable for patients with heavily pre-treated ESFT.

Trial registration

NCT01380275. Registered June 21, 2011.

Docetaxel; Irinotecan; Recurrent; Refractory; Ewing sarcoma family of tumors