Peripheral Primitive Neuroectodermal Tumor of the Chest Wall in Childhood: Clinico- Pathological Significance, Management and Literature Review

Peripheral primitive neuroectodermal tumor of the chest wall is a rare malignant tumorusually occurring in children. The tumor shares a similar histology, immunohistology andcytogenetics to Ewing’s sarcoma. The location of the tumor in the chest wall poses a majorchallenge with respect to the diagnostic workup and treatment which involves multidisciplinarymanagement. Neoadjuvant chemotherapy is given initially and is followed by completesurgical resectioning of the mass followed by repeat chemotherapy with or without radiotherapy.We report a case of peripheral primitive neuroectodermal tumor of the chest wall ina 5-year-old boy and review the literature regarding its diagnosis and management.