Adolescent–adult nonmetastatic Ewing sarcoma—Experience from a large developing country

• Published in: Pediatric blood & cancer 2021-09, Vol.68 (9), p.e29081-n/a
• Main Authors: Bajpai, Jyoti, Panda, Goutam Santosh, Chandrasekharan, Arun, Bhargava, Prabhat, Srinivas, Sujay, Laskar, Siddhartha, Dandekar, Sonal, Mokal, Smruti, Rekhi, Bharat, Khanna, Nehal, Menon, Nandini, Patil, Vijay, Noronha, Vanita, Joshi, Amit, Prabhash, Kumar, Banavali, Shripad D., Gupta, Sudeep
• Format: Article
• Language: English
• Subjects: Adolescent; adolescent–adult; Adult; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Bone Neoplasms - drug therapy; Chemotherapy; Cyclophosphamide; Cyclophosphamide - therapeutic use; Developing Countries; Disease-Free Survival; Doxorubicin; Doxorubicin - therapeutic use; EFT‐2001; Etoposide; Etoposide - therapeutic use; Ewing sarcoma; Ewing's sarcoma; Ewings sarcoma; Female; Hematology; Humans; Ifosfamide; Ifosfamide - therapeutic use; low and middle‐income countries (LMICs); Male; Medical prognosis; Middle Aged; Neutropenia; nonmetastatic; Oncology; Patients; Pediatrics; Peripheral neuropathy; Sarcoma, Ewing - drug therapy; Survival; Toxicity; Tumors; Vincristine; Vincristine - therapeutic use; Young Adult
• ISSN: 1545-5009; 1545-5017
• DOI: 10.1002/pbc.29081

Background Outcome and toxicity data in adolescent–adult Ewing sarcoma (AA‐ES) patients are sparse and merits exploration. Methods Histopathologically confirmed, nonmetastatic AA‐ES patients, who received standard institutional combination chemotherapy regimen (Ewing's family of tumors‐2001 [EFT‐2001]) comprising of ifosfamide plus etoposide and vincristine, doxorubicin plus cyclophosphamide, lasting a total of 12 months between 2013 and 2018, were analyzed for treatment‐related toxicities, event‐free survival (EFS), and overall survival (OS). Results There were 235 patients (primary safety cohort [PSC]) with median age of 23 (15–61) years; 159 (67.7%) were males, 155 (65.9%) had skeletal primary and 114 (48.5%) had extremity tumors. One hundred ninety‐six (83.4%) were treatment naïve (primary efficacy cohort [PEC]) and of these 119 (60.7%) had surgery. In PEC, at a median follow‐up of 36.4 (interquartile range [IQR] 20–55) months, estimated 3‐year EFS and OS were 67.3% (95% CI 60.3–75.1%) and 91.1% (95% CI 86.7–95.7%), respectively. Of these, 158 (80.6%) complying with intended treatment, at a median follow‐up of 39 (IQR 26–57) months had an estimated 3‐year EFS of 68.2% (95% CI 60.3–76.1%). In multivariable analysis, good prognostic factors included longer symptom(s) duration (HR 0.93, 95% CI 0.86–0.994), ≥99% necrosis (HR 0.30, 95% CI 0.11–0.77), and treatment completion (HR 0.32, 95% CI 0.14–0.74). Among PSC, grade 3–4 toxicities were febrile neutropenia (119, 50.6%), anemia (130, 55.3%), peripheral neuropathy (37, 15.7%), with three (1.3%) chemo‐toxic deaths. Conclusions The outcomes of AA nonmetastatic ES patients treated with EFT‐2001 regimen were comparable to those reported by others, with acceptable toxicity. This regimen can be considered a standard of care in AA‐ES.