Management of Ewing Family of Tumors Arising from Chest Wall in Children

Aim: Malignant chest wall tumors are rare in children. We aimed to study the management of the Ewing family of tumors (EFT) in the chest walls of children. Materials and Methods: The files of patients diagnosed with primitive neuro-ectodermal tumor and Ewing sarcoma of the chest wall in the prior 2 decades were retrospectively reviewed. Results: A total of 15 children with a median age of 10 (8-14) years were included. Their symptoms were palpable mass (n=6), chest pain (n=4), B symptoms (n=2), cough (n=2) and abdominal pain. Their history revealed delayed diagnoses in 1/3 of the patients due to a misinterpretation of the symptoms. All children except for one had tru-cut biopsy and upfront chemotherapy. The median greatest diameter of mass at diagnosis was 100 (67-148) mm and 51 (39-100) mm at preoperative imaging after chemotherapy. Pleural effusion (n=8), costal destruction (n=9), extension to neural foramina (n=3), pulmonary or diaphragmatic nodule (n=5) and distant bone metastasis (n=4) were present at diagnosis in some cases. More than one surgery was performed in 9 (60%) of the children in order to remove the primary tumor and metastases. Costal excision (n=11), diaphragmatic resection (n=6) and chest wall reconstruction with graft material were performed on 8 patients. All cases had radiotherapy postoperatively. Mortality occurred in 9 (60%) patients due to local recurrences or metastases. Only 3 children reached 5-year survival and were considered to be cured. The remaining 3 patients completed their first line oncologic treatments and were under surveillance without treatment. Conclusion: The EFT in the chest wall are aggressive tumors with poor prognosis despite multimodality treatment. Surgeons dealing with these patients should be familiar with complex chest wall reconstruction techniques.