Sinonasal primitive neuroectodermal tumor arising in a long-term survivor of heritable unilateral retinoblastoma.
BACKGROUND
Patients who survive retinoblastoma (RB) are at risk for having second nonocular tumors, usually osteosarcomas, which often are fatal. Such patients almost always have bilateral RB.


METHODS
This article reports a woman who, at the age of 1 year had been cured of a unilateral RB by radiation therapy and enucleation. Eighteen years later, she had a sinonasal small cell tumor that rapidly recurred and proved fatal 2 months after surgical debulking. The tumor was studied by immunohistochemistry and electron microscopic (EM) examination.


RESULTS
It showed diffuse neuron-specific enolase staining, focal weak staining for chromogranin, synaptophysin, and Leu-7 monoclonal antibodies in paraffin-embedded, B5-fixed tissue (Great Lakes Diagnostics, Troy, MI). EM study showed an undifferentiated primitive neuroectodermal tumor with many polyribosomes, simple cell junctions, few microtubules, and rare dense core granules.


CONCLUSIONS
The combined immunohistochemical, ultrastructural, and clinical features of the tumor were interpreted as a sinonasal primitive neuroectodermal tumor with early neuronal differentiation. The tumor was pathologically indistinguishable from poorly differentiated olfactory neuroblastoma (ONB) and Ewing sarcoma.
Klein
E A
EA
Department of Pathology, Staten Island University Hospital, NY 10305.
Anzil
A P
AP
Mezzacappa
P
P
Borderon
M
M
Ho
V
V
D002864:Chromogranins / Q000378:metabolism; D005134:Eye Neoplasms / Q000235:genetics / Q000473:pathology* / Q000532:radiotherapy; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D009373:Neoplasms, Germ Cell and Embryonal / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D016609:Neoplasms, Second Primary / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000473:pathology; D010255:Paranasal Sinus Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D010751:Phosphopyruvate Hydratase / Q000378:metabolism; D012175:Retinoblastoma / Q000235:genetics / Q000473:pathology* / Q000532:radiotherapy; D012512:Sarcoma, Ewing / Q000473:pathology; D016708:Synaptophysin / Q000378:metabolism
D002864:Chromogranins; D016708:Synaptophysin; D010751:Phosphopyruvate Hydratase