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Sinonasal primitive neuroectodermal tumor arising in a long‐term survivor of heritable unilateral retinoblastoma
Author(s) -
Klein Edward A.,
Anzil Archinto P.,
Mezzacappa Peter,
Borderon Marie,
Ho Victor
Publication year - 1992
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19920715)70:2<423::aid-cncr2820700209>3.0.co;2-b
Subject(s) - primitive neuroectodermal tumor , synaptophysin , pathology , retinoblastoma , chromogranin a , enucleation , immunohistochemistry , neuroectodermal tumor , medicine , enolase , sarcoma , ewing's sarcoma , immunostaining , biology , biochemistry , surgery , gene
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, synapto‐physin, 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 immunohistochemi‐cal, 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. Cancer 1992; 70:423–431.