Abstract
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.
MeSH terms
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Antibodies, Monoclonal, Humanized / administration & dosage
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Antineoplastic Combined Chemotherapy Protocols / therapeutic use
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Bevacizumab
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Cyclophosphamide / administration & dosage
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Doxorubicin / administration & dosage
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Eye Neoplasms / secondary*
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Eye Neoplasms / therapy
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Face / pathology
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Fatal Outcome
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Humans
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Infant, Newborn
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Male
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Neuroectodermal Tumors, Primitive / congenital*
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Neuroectodermal Tumors, Primitive / pathology*
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Neuroectodermal Tumors, Primitive / therapy
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Soft Tissue Neoplasms / congenital*
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Soft Tissue Neoplasms / pathology*
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Soft Tissue Neoplasms / therapy
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Topotecan / administration & dosage
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Vincristine / administration & dosage
Substances
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Antibodies, Monoclonal, Humanized
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Bevacizumab
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Vincristine
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Topotecan
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Doxorubicin
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Cyclophosphamide