Abstract Introduction Multimodality treatment for Ewing sarcoma (chemotherapy, radiation, and high-dose conditioning with stem-cell rescue) has substantially improved long-term survival for many pediatric patients. However, these treatments can cause late toxicities, including radiation-induced pulmonary fibrosis and hepatic complications. Long-term follow-up studies of survivors of childhood Ewing sarcoma document late pulmonary and other organ complications, though progression to end-stage organ failure requiring transplantation is uncommon. Case A young male diagnosed at age 12 with metastatic Ewing sarcoma of the right femur with pulmonary metastases. His initial oncologic therapy included the AEWS0031 regimen, followed by high-dose therapy with autologous stem-cell rescue after conditioning with busulfan, melphalan, and topotecan, and adjuvant lung radiation complete remission achieved one year later. Over ensuing years he developed progressive radiation-associated pulmonary fibrosis and pulmonary arterial hypertension, and non-cirrhotic portal hypertension with recurrent esophageal variceal bleeding requiring endoscopic band ligation and TIPS placement. Multidisciplinary review concluded he was an appropriate candidate for combined lung & liver transplantation. He was admitted to Montefiore Einstein with acute hypoxemic hypercapnic respiratory failure and altered mental status requiring veno-venous extracorporeal membrane oxygenation (VV-ECMO) as a bridge to transplant. A suitable donor became available, and he underwent combined double-lung and liver transplant. Complex post transplant course included failed extubation requiring tracheostomy placement. He was discharged to inpatient reb and is now decannulated at home. Discussion Survivors of childhood Ewing sarcoma are known to experience late treatment-related toxicities — radiation and certain chemotherapeutic agents can cause progressive pulmonary injury (including pulmonary fibrosis) and other organ dysfunction. Longitudinal cohorts and survivorship studies document pulmonary and other late effects among Ewing sarcoma survivors, particularly in those who received chest or lung radiation and high-dose or cumulative pulmonary-toxic chemotherapy. Combined lung-liver transplantation is a rare procedure performed for patients with concurrent end-stage lung and liver disease. Key considerations for transplant candidacy after prior malignancy include: length of remission, tumor biology, risk of recurrence, and risk of donor-derived or recurrent disease under post-transplant immunosuppression. Conclusion We present a rare case of combined double-lung and liver transplantation following curative treatment of metastatic Ewing sarcoma. This case underscores the potential for severe late organ toxicity after curative childhood cancer therapy, the feasibility of combined multiorgan transplantation after prior malignancy, and the need for long-term survivorship surveillance and multidisciplinary planning. This abstract is funded by: None
Gazineo et al. (Fri,) studied this question.