Abstract

ROMERO, R. et al. Recurrence of peripheral primitive neuroectodermal tumor of the orbit with systemic metastases. Arch Soc Esp Oftalmol [online]. 2006, vol.81, n.10, pp.599-602. ISSN 0365-6691.

Case report: A six-year-old boy presented with proptosis of the right eye. Imaging studies detected a mass in the medial wall of the right orbit. This mass was biopsied revealing a histopathologic diagnosis of primitive neuroectodermal tumor, so chemotherapy treatment was given. After seven years in remission he presented with a recurrence of the orbital tumor and was found to also have systemic metastases. Treatment with chemotherapy, radiotherapy and orbital exenteration was unsuccessful. Discussion: The orbital occurrence of these tumors is extremely rare. Differentiation from other small round cell tumors requires immunohistochemical and ultrastructural techniques.

Keywords : Peripheral primitive neuroectodermal tumor; recurrence; orbit; proptosis; small round cell tumors.

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