RESUMO

Primitive neuroectodermal tumor (PNET) is most common in the second decade of life. Congenital PNET is very rare. Ocular metastasis of PNET is likewise exceedingly rare; with only 5 previously published cases. We report an unusual congenital PNET of the face, which metastasized to subcutis, eyes, and brain. The primary tumor responded to chemotherapy (vincristine/doxorubicin/cyclophosphamide) with metachronous progression of ocular lesions. A therapeutic trial of intraocular bevacizumab showed no efficacy on intraocular lesions. Eventually the patient developed cerebral metastasis, and second line therapy with topotecan/cyclophosphamide was initiated. The tumor progressed and the patient died after acute herniation.

Assuntos

Neoplasias Oculares/secundário , Tumores Neuroectodérmicos Primitivos/congênito , Tumores Neuroectodérmicos Primitivos/patologia , Neoplasias de Tecidos Moles/congênito , Neoplasias de Tecidos Moles/patologia , Anticorpos Monoclonais Humanizados/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Bevacizumab , Ciclofosfamida/administração & dosagem , Doxorrubicina/administração & dosagem , Neoplasias Oculares/terapia , Face/patologia , Evolução Fatal , Humanos , Recém-Nascido , Masculino , Tumores Neuroectodérmicos Primitivos/terapia , Neoplasias de Tecidos Moles/terapia , Topotecan/administração & dosagem , Vincristina/administração & dosagem