Challenges in the management of localized Ewing sarcoma in a developing country.

Abstract

Survival in pediatric Ewing sarcoma (ES) lags in low- and middle-income countries (LMICs). This study analyzed factors contributing to a lower outcome in an LMIC center. A retrospective case review of children with localized ES treated from January 2011 till December 2017 was performed. Neoadjuvant chemotherapy with alternating cycles of vincristine, doxorubicin, cyclophosphamide; and ifosfamide, etoposide was administered 3-weekly for 48 weeks. Reassessment was planned for week 12, followed by local therapy (surgery/radiotherapy or both) tailed by adjuvant chemotherapy. Forty-eight patients with mean age 8 years (range: 0.7-14) were evaluated. Extremity and central axis tumors were seen in 25 (52%) and 23 (48%) patients. Three patients died of neutropenic sepsis and five abandoned therapy. Local therapy included primary surgery, radiotherapy and a combination of surgery and radiotherapy in 7 (16%), 20 (45%) and 17 (39%) patients. The 3-year event-free survival (EFS) and disease-free survival (DFS) for the cohort were 47.7 ± 11% and 57.6 ± 11.2%. Time to local therapy >16 weeks was associated with inferior DFS vs. local therapy administered within 16 weeks [46.6 ± 12.4 vs. 63.9 ± 19.4, p=.046]. Older age, axial site, large size and incomplete surgical resection did not predict relapse/progression. Patients who received wide local excision, as local therapy, had 100% DFS. Coordinated efforts to ensure timely therapy can improve outcome in pediatric ES. Abandonment and treatment-related mortality (TRM) are additional challenges that need to be tackled in LMICs.

• Authors: Sidharth Totadri, Deepak Bansal, K L N Rao, Richa Jain, Akshay Kumar Saxena, Rakesh Kapoor, Ram Samujh, Amita Trehan
• Journal: Pediatric hematology and oncology (Pediatr Hematol Oncol) Vol. 37 Issue 7 Pg. 610-619 (Oct 2020) ISSN: 1521-0669 [Electronic] England
• PMID: 32558608 (Publication Type: Journal Article)

Chemical References

• Vincristine
• Etoposide
• Doxorubicin
• Cyclophosphamide
• Ifosfamide

Topics

• Adolescent
• Antineoplastic Combined Chemotherapy Protocols (administration & dosage)
• Bone Neoplasms (drug therapy, mortality)
• Child
• Child, Preschool
• Cyclophosphamide (administration & dosage)
• Developing Countries
• Disease-Free Survival
• Doxorubicin (administration & dosage)
• Etoposide (administration & dosage)
• Female
• Humans
• Ifosfamide (administration & dosage)
• Infant
• Male
• Patient Compliance
• Prognosis
• Retrospective Studies
• Sarcoma, Ewing (drug therapy, mortality)
• Sepsis (mortality)
• Survival Rate
• Vincristine (administration & dosage)