Katsutsugu Umeda1, Takako Miyamura2, Kenji Yamada3, Hideki Sano4, Ako Hosono5, Minako Sumi6, Hajime Okita7, Takuya Kamio8, Naoko Maeda9, Hiroyuki Fujisaki10, Ryoji Jyoko11, Atsuko Watanabe12, Yosuke Hosoya13, Daiichiro Hasegawa14, Satoshi Takenaka15, Shunsuke Nakagawa16, Motoaki Chin17, Toshifumi Ozaki11. 1. Department of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto, Japan. 2. Department of Pediatrics, Osaka University Graduate School of Medicine, Suita, Japan. 3. Department of Orthopedic Surgery, Okazaki City Hospital, Okazaki, Japan. 4. Department of Pediatric Oncology, National Cancer Center Hospital East, Kashiwa, Japan. 5. Department of Pediatric Oncology, Fukushima Medical University Hospital, Fukushima, Japan. 6. Department of Radiation Oncology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan. 7. Department of Pathology, Keio University School of Medicine, Tokyo, Japan. 8. Department of Pediatrics, Hirosaki University Hospital, Hirosaki, Japan. 9. Department of Pediatrics, National Hospital Organization Nagoya Medical Center, Nagoya, Japan. 10. Department of Pediatric Hematology/Oncology, Osaka City General Hospital, Osaka, Japan. 11. Department of Orthopedic Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan. 12. Division of Pediatric Oncology, Comprehensive Cancer Center, International Medical Center, Saitama Medical University, Saitama, Japan. 13. Department of Pediatrics, St. Luke's International Hospital, Tokyo, Japan. 14. Department of Hematology and Oncology, Children's Cancer Center, Kobe Children's Hospital, Kobe, Japan. 15. Department of Orthopedic Surgery, Osaka University Graduate School of Medicine, Suita, Japan. 16. Department of Pediatrics, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan. 17. Department of Pediatrics and Child Health, Nihon University Itabashi Hospital, Tokyo, Japan.
Abstract
BACKGROUND: The prognosis of patients with metastatic Ewing sarcoma family of tumors (ESFT) remains poor. PROCEDURE: We retrospectively analyzed 57 patients diagnosed with metastatic ESFT between 2000 and 2018 to identify prognostic and therapeutic factors affecting the clinical outcome. RESULTS: The 3-year overall survival (OS) rate of the entire cohort was 46.8% (95% confidence interval [CI], 33.0-59.4%). Treatment-related death was not observed. Multivariate analysis identified stem cell transplantation (SCT), response to first-line chemotherapy, and bone metastasis as independent risk factors for OS. Objective response rate to first-line chemotherapy was 65.1% in the 43 evaluable patients. There was no significant difference in the response to different types of first-line chemotherapy. Among patients with lung metastasis alone, the 3-year OS rate was higher in 13 patients who received local treatment than in four who did not, although the difference was not significant. CONCLUSIONS: One possible reason for the high OS rates was the absence of treatment-related mortality even in patients receiving SCT, which could be attributed to advances in the management of post-SCT complications. Novel first-line chemotherapy strategies need to be established to improve the disease status prior to SCT in a higher proportion of patients.
BACKGROUND: The prognosis of patients with metastatic Ewing sarcoma family of tumors (ESFT) remains poor. PROCEDURE: We retrospectively analyzed 57 patients diagnosed with metastatic ESFT between 2000 and 2018 to identify prognostic and therapeutic factors affecting the clinical outcome. RESULTS: The 3-year overall survival (OS) rate of the entire cohort was 46.8% (95% confidence interval [CI], 33.0-59.4%). Treatment-related death was not observed. Multivariate analysis identified stem cell transplantation (SCT), response to first-line chemotherapy, and bone metastasis as independent risk factors for OS. Objective response rate to first-line chemotherapy was 65.1% in the 43 evaluable patients. There was no significant difference in the response to different types of first-line chemotherapy. Among patients with lung metastasis alone, the 3-year OS rate was higher in 13 patients who received local treatment than in four who did not, although the difference was not significant. CONCLUSIONS: One possible reason for the high OS rates was the absence of treatment-related mortality even in patients receiving SCT, which could be attributed to advances in the management of post-SCT complications. Novel first-line chemotherapy strategies need to be established to improve the disease status prior to SCT in a higher proportion of patients.
Authors: Paulina Jagodzińska-Mucha; Paweł Sobczuk; Michał Mikuła; Anna Raciborska; Anna Dawidowska; Maria Kulecka; Katarzyna Bilska; Anna Szumera-Ciećkiewicz; Anna Kluska; Magdalena Piątkowska; Anna Bałabas; Piotr Rutkowski; Iwona Ługowska Journal: Contemp Oncol (Pozn) Date: 2021-12-29