Unusual sinonasal small-cell neoplasms following radiotherapy for bilateral retinoblastomas

Journal Article · Wed Nov 01 00:00:00 EST 1989 · American Journal of Surgical Pathology; (USA)

DOI:https://doi.org/10.1097/00000478-198911000-00005· OSTI ID:5224453

Frierson, Jr, H F; Ross, G W; Stewart, F M; Newman, S A; Kelly, M D ^[1]

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1. Univ. of Virginia Health Sciences Center, Charlottesville (USA)

Two patients developed sinonasal small-cell neoplasms that arose 22 years and 37 years, respectively, following radiotherapy for bilateral retinoblastomas. The tumors were composed of small cells with scant cytoplasm and had a few scattered Homer-Wright rosettes. Immunohistochemically, one tumor was positive for keratin (CAM 5.2 and AE1/AE3), epithelial membrane antigen, and neuron-specific enolase. The other neoplasm was immunoreactive for keratin (CAM 5.2 only) and neuron-specific enolase; it also had focal immunopositivity for S-100 protein, desmin, and muscle-specific actin. Both were negative for CEA, vimentin, melanocyte-specific antigen (HMB45), chromogranin A, synaptophysin, Leu-7, 200 kd neurofilament, and retinal S-antigen. Despite aggressive multimodal therapy, the patients died of metastatic tumor 7 months and 10 months following their initial diagnosis, respectively. Although osteosarcoma is the most frequent second cancer following bilateral retinoblastomas, some patients develop clinically aggressive sinonasal small-cell tumors that are difficult to place into conventional classifications. Both of our cases showed evidence of multidirectional differentiation; one tumor labeled with epithelial and neural markers, and the other expressed epithelial, neural, and myogenous antigens.49 references.

• OSTI ID: 5224453
• Journal Information: American Journal of Surgical Pathology; (USA), Vol. 13:11; ISSN 0147-5185
• Country of Publication: United States
• Language: English

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