The authors present a case of primary peripheral primitive neuroectodermal tumor of the orbit in a 10-month-old girl, which is the first case in the Brazilian literature. Clinical, radiologic and histopathologic features are discussed. The diagnosis was confirmed by immunohistochemical analysis which is essential to the diagnosis of small round cell tumors in the orbit.
Neuroectodermal tumors; Orbit; Small round cell; Immunohistochemistry; Sarcoma; Ewing's; Case reports
