Since 2000, centers across Central America have shared treatment guidelines for Wilms tumor, using histology (anaplasia present or absent) and tumor stage to stratify patients into low, intermediate, and high-risk groups. Weekly virtual tumor board meetings involving local and international experts were held to ensure consistent treatment assignments. We analyzed data from 367 children with unilateral tumor treated per these guidelines. Five-year abandonment-sensitive event-free and overall survival estimates were: low-risk 82% ±3.8% and 86% ±3.6%, intermediate-risk 50% ±3.4% and 60% ±3.4%, and high-risk 36% ±7.6% and 45% ±7.9%. Survival outcomes were suboptimal, primarily due to advanced disease in fragile children at presentation and abandonment of treatment.

BACKGROUND AND AIMS: There is scarce information about pediatric lymphoblastic lymphoma (LLy) in low and middle-income countries. We describe the clinical characteristics, treatment and outcome of a cohort of children and adolescents with LLy in Latin America (LA). METHODS: Retrospective study analyzing pediatric patients with LLy in 10 institutions of the St. Jude Global Alliance from nine LA countries between 2007 and 2017. RESULTS: One-hundred and twenty-six patients were included. Sixty (47.6%) had T-LLy, 49 (38.9%) B-LLy and in 17 (13.5%) the immunophenotype was unknown. Ninety-seven (77%) presented with stage III/IV disease, and 42 (33.3%) in critical conditions. In 30 cases (23.8%), the results of pathology diagnosis exceeded 15 days from biopsy, and 23 patients (18%) required a review at another institution. The EFS and OS at 5 years were 73% (SE 0.047) and 78% (SE 0.0435), respectively. Five-year abandonment-sensitive EFS and OS were 65% (SE 0.0477) and 70% (SE 0.0459), respectively. Events included relapse/progression (n=22), refractory disease (n =1) abandonment (n=11), induction death (n=4), death in complete remission (n=4), and second malignancies (n=1). CONCLUSIONS: A balanced proportion of LLy-T and B phenotypes was observed. Diagnosis was a challenge. Most of the patients presented with high-risk disease, and many in critical conditions. Toxic deaths and abandonment represented nearly half of the events. Improvements in diagnosis, supportive measures and follow up are imperative to improve the outcomes of pediatric LLy in Latin America.

Background/Objectives: High-risk Hodgkin lymphoma (HRHL) in children is a curable with combined modality therapy. The AHOPCA is a consortium of cancer centers from Central America. In 2004, AHOPCA implemented a guideline with a short course of chemotherapy (mStanfordV), strict diagnostics and radiation guidelines, aimed at reducing abandonment and improving outcomes. Methods: Newly diagnosed children less than 18 years of age with high-risk HL (Ann Arbor stages: IIB, IIIB, IV) from AHOPCA centers were staged with chest X-ray, and ultrasound or CT. Therapy was a modified StanfordV (mStanfordV) substituting cyclophosphamide for mechlorethamine and involved field radiation. Results: Of 219 patients with HRHL, 181 patients were eligible and evaluable;146 (81%) were boys, 22% being less than 6 years; 43 were stage IIB, 84 IIIB and 54 IV. Thirty-one (17%) abandoned therapy, 28 (15%) progressed, 30 (17%) relapsed and 8 (4%) died of toxicity. Radiation guidelines were not followed. Five-year abandonment-sensitive event-free survival and overall survival (AS-EFS, AS-OS±SE) for the cohort were 46±4% and 56±4%; 5-year AS-OS for stages IIB, IIIB and IV was 76±7%, 59±7%, and 35±7% (p=0.0006). Conclusion: Despite instituting a short treatment guideline, it did not improve the abandonment rate (17%) and did not achieve the reported outcomes of StanfordV. The cyclophosphamide dose used to replace merchlorethamine was inadequate. Despite strict guidelines, the radiation therapy application was inaccuarate. Weekly chemotherapy may have adversely affected abandonment of therapy by increasing the burden of travel-time. Based on these results, AHOPCA established a new abandonment strategy and a new guideline.