RESUMO
Primitive neuroectodermal tumor is a small round cell malignancy which rarely involves the orbit. We report a case of a two-year old male child presenting as unilateral eccentric proptosis with extraconal and intraconal mass, diagnosed as primary peripheral primitive neuroectodermal tumor (pPPNET) on histopathology and immunohistochemistry. There is no defined consensus in the management of these tumors due to its rare presentation. We describe its distinguishing features with emphasis on multimodal and aggressive treatment approach which ensures appropriate management of these cases.
Assuntos
Antineoplásicos/uso terapêutico , Biópsia , Pré-Escolar , Seguimentos , Humanos , Imageamento por Ressonância Magnética , Masculino , Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico , Tumores Neuroectodérmicos Primitivos Periféricos/tratamento farmacológico , Tumores Neuroectodérmicos Primitivos Periféricos/radioterapia , Neoplasias Orbitárias/diagnóstico , Neoplasias Orbitárias/tratamento farmacológico , Neoplasias Orbitárias/radioterapia , Radioterapia Adjuvante , Tomografia Computadorizada por Raios XRESUMO
Peripheral neuroepithelioma [PN] is a rare highly malignant tumor, classified in the group of small round cell tumors. We report eight cases of PN, based on histologic appearance and immunohistochemical staining for neuron-specific enolase [NSE]. Electron microscopy was also performed in three cases. The tumors exhibited rapid local growth, leading to limb amputation in five cases. Histologically the presence of Homer-Wright rosettes is a characteristic of this neoplasm. Tumoral cells are generally positive for NSE. The relationship of PN with Ewing's sarcoma is discussed