Vol.:(0123456789) 1 3 Cancer Chemotherapy and Pharmacology https://doi.org/10.1007/s00280-019-03789-3 ORIGINAL ARTICLE SCMCIE94: an intensifed pilot treatment protocol known to be associated with cure in CD 56-negative non-pelvic isolated Ewing sarcoma (EWS) is also associated with no early relapses in non- metastatic extremity EWS Ian Joseph Cohen 1,2  · Helen Toledano 1,2  · Jerry Stein 1,2  · Yehuda Kollender 2,3  · Eyal Fenig 2,4  · Osnat Konen 2,5  · Zvi Bar‑Sever 2,6  · Josephine Issakov 2,7  · Meora Feinmesser 2,8  · Smadar Avigad 2,9  · Shifra Ash 1,2 Received: 1 January 2019 / Accepted: 29 January 2019 © Springer-Verlag GmbH Germany, part of Springer Nature 2019 Abstract Purpose We report the unexpected absence of early relapse (before 30 months) in 24 consecutive patients with isolated limb primary Ewing sarcoma treated with an intensifed pilot protocol, SCMCIE94. Methods Clinical data for the study were collected retrospectively from the patient fles. The protocol included 6 courses of chemotherapy, split radiation, and limb salvage surgery. This SCMCIE94 protocol had been used in almost all the patients described in an earlier report, in whom those with non-pelvic isolated tumors and low/absent CD56 expression in Ewing sarcoma tumor cells were all long-term survivors. Results The 5-year (10-year) event-free survival rate for the patients with isolated limb primary Ewing sarcoma was 78.95 ± 8.3% (68.6 ± 10.0%) and the overall survival rate was 90.7 ± 6.2% (71.1 ± 11.2%). There were no relapses before 30 months in any of these patients. Conclusion The intensifed SCMCIE94 pilot protocol has been shown previously to cure patients with localized CD56- negative non-pelvic Ewing sarcoma. The present study shows that among all patients with localized extremity disease who were treated with this protocol, there were no cases of early relapse. Although our cohort was small, the diference in results from studies using other protocols is so striking, that it would seem reasonable to assume it is attributable to the changes made in the protocol itself rather than risk factors. Late relapses of isolated limb CD56-positive Ewing sarcoma suggest minimal residual disease warranting additional therapeutic approaches such as autologous stem cell rescue after Busulfan Melfelan. Keywords SCMCIE94 · Non-metastatic · Non-pelvic · Limb Ewing sarcoma · Treatment outcome · CD56 · Early relapse prevention * Ian Joseph Cohen icohen@tau.ac.il 1 The Rina Zaizov Hematology-Oncology Division, Schneider Children’s Medical Center of Israel, Petach Tikva, Israel 2 Sackler faculty of Medicine, Tel Aviv University, Tel Aviv, Israel 3 Unit of Orthopedic Oncology, Tel Aviv Medical Center, Tel Aviv, Israel 4 Department of Radiotherapy, Rabin Medical Center, Beilinson Hospital, Petach Tikva, Israel 5 Department of Pediatric Radiology, Schneider Children’s Medical Center of Israel, Petach Tikva, Israel 6 Department of Nuclear Medicine, Schneider Children’s Medical Center of Israel, Petach Tikva, Israel 7 Institute of Pathology, Sourasky Tel Aviv Medical Center, Tel Aviv, Israel 8 Institute of Pathology, Beilinson Hospital, Petach Tikva, Israel 9 Molecular Oncology, Felsenstein Medical Research Center, Beilinson Hospital, Petach Tikva, Israel