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

INTRODUCTION: Malignant chest wall tumors are rare in children. We aim to study management of Ewing family of tumors in chest wall of children.
METHODS: Patients files diagnosed with primitive neuroectodermal tumor and Ewing sarcoma of chest wall in the last 2 decades was retrospectively reviewed.
RESULTS: Total of 15 children with the median age of 10 (8-14) years were included. Symptoms were palpable mass (n=6), chest pain (n=4), B symptoms (n=2), cough (n=2) and abdominal pain. History revealed delayed diagnosis in 1/3 of patients due to misinterpretation of symptoms. All children except one had tru-cut biopsy and upfront chemotherapy. Median greatest diameter of mass at diagnosis was 100 (67-148) mm and 51 (39-100) mm at the 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) was present at diagnosis. More than one surgery was performed in 9 (60%) children to remove primary tumor and metastases. Costal excision (n=11), diaphragmatic resection (n=6) and chest wall reconstruction with graft material were done in (n=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 cured. Other 3 patients completed first line oncologic treatments and under surveillance without treatment.
DISCUSSION AND CONCLUSION: Ewing family of tumors in 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.