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MANAGEMENT OF EWING FAMILY OF TUMORS ARISING FROM CHEST WALL IN CHILDREN
DL RANA USER, BURAK ARDILI, ARBAY ZDEN FT, BRAHM KARNAK, BERNA OUZ, MTHAT HALLOLU, TEZER KUTLUK, FERAH YILDIZ, DCLEHAN ORHAN, FLZ ZMCGL, SANYE EKNC
The Journal of Pediatric Research - 2024;11(2):118-122
Hacettepe University Faculty of Medicine Department of Pediatric Surgery, Ankara, Turkey

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.

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