A comparative immunohistochemical analysis of small round cell tumors of childhood: utility of peripherin and alpha-internexin as markers for neuroblastomas.
Immunohistochemical study of neuroblastomas, Ewing sarcomas, rhabdomyosarcomas, and Wilms tumors demonstrate specific expression of peripherin and alpha-internexin in 20/22 and 6/22 cases of neuroblastomas, respectively. Microtubule-associated protein 1B (MAP 1B) was strongly and diffusely expressed in all 22 cases of neuroblastomas, but was also focally or multifocally expressed in 9/12 rhabdomyosarcomas and also in the blastema and stroma of 8/11 Wilms tumors. All rhabdomyosarcomas strongly and diffusely express nestin, but this marker was also expressed, multifocally, in 15/22 neuroblastomas and also in the blastema and stroma of all 11 Wilms tumors. NeuN, a neuron-specific nuclear protein, was expressed focally in 1 case of neuroblastoma and diffusely in 2 other cases (3/22). Surprisingly, it was also focally expressed in 2/12 rhabdomyosarcomas. In contrast, all 7 cases of Ewing sarcoma were negative for peripherin, MAP 1B, alpha-internexin, NeuN, and nestin. Thirteen neuroblastomas were also immunostained for neurofilaments, tyrosinase, and anaplastic lymphoma kinase 1 (ALK 1), and were found to be negative for these markers. Our results confirm that peripherin and alpha-internexin are neuroblastoma markers useful for the differential diagnostic work-up of small round cell tumors of childhood. Strong diffuse immunoreactivity for MAP 1B favors a diagnosis of neuroblastoma, whereas strong diffuse immunoreactivity for nestin favors a diagnosis of rhabdomyosarcoma.
Department of Pathology, Scott and White Memorial Hospital, Temple, TX 76508, USA.
Vickie
V
Willoughby
Anuj
A
Sonawala
Ada
A
Werlang-Perurena
Ludvik R
LR
Donner
D014408:Biomarkers, Tumor / Q000032:analysis*; D018288:Carcinoma, Small Cell / Q000175:diagnosis / Q000378:metabolism* / Q000473:pathology; D002648:Child; D006801:Humans; D007150:Immunohistochemistry; D007381:Intermediate Filament Proteins / Q000096:biosynthesis / Q000378:metabolism*; D008562:Membrane Glycoproteins / Q000096:biosynthesis / Q000378:metabolism*; D009419:Nerve Tissue Proteins / Q000096:biosynthesis / Q000378:metabolism*; D064231:Nestin; D009447:Neuroblastoma / Q000175:diagnosis / Q000378:metabolism* / Q000473:pathology; D064531:Peripherins
D014408:Biomarkers, Tumor; D007381:Intermediate Filament Proteins; D008562:Membrane Glycoproteins; C577484:NES protein, human; D009419:Nerve Tissue Proteins; D064231:Nestin; C578994:PRPH protein, human; D064531:Peripherins; C046643:alpha-internexin