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 tumor usually occurring in children. The tumor shares a similar histology, immunohistology and cytogenetics to Ewing's sarcoma. The location of the tumor in the chest wall poses a major challenge with respect to the diagnostic workup and treatment which involves multidisciplinary management. Neoadjuvant chemotherapy is given initially and is followed by complete surgical 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 in a 5-year-old boy and review the literature regarding its diagnosis and management.