An Unusual Case of Congenital Primitive Neuroectodermal Tumor With Ocular Metastasis

Autor: Sara O. Vargas, Tobias Neff, Lindsay Frazier, Cristian M. Salgado, Deborah K. VanderVeen
Rok vydání: 2012
Předmět:
Zdroj: Journal of Pediatric Hematology/Oncology. 34:e69-e71
ISSN: 1077-4114
DOI: 10.1097/mph.0b013e318221ca9b
Popis: 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.
Databáze: OpenAIRE