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.
AuthorsVickie Willoughby, Anuj Sonawala, Ada Werlang-Perurena, Ludvik R Donner
JournalApplied immunohistochemistry & molecular morphology : AIMM / official publication of the Society for Applied Immunohistochemistry (Appl Immunohistochem Mol Morphol) Vol. 16 Issue 4 Pg. 344-8 (Jul 2008) ISSN: 1533-4058 [Electronic] United States
PMID18528283 (Publication Type: Comparative Study, Journal Article)
Chemical References
  • Biomarkers, Tumor
  • Intermediate Filament Proteins
  • Membrane Glycoproteins
  • NES protein, human
  • Nerve Tissue Proteins
  • Nestin
  • PRPH protein, human
  • Peripherins
  • alpha-internexin
  • Biomarkers, Tumor (analysis)
  • Carcinoma, Small Cell (diagnosis, metabolism, pathology)
  • Child
  • Humans
  • Immunohistochemistry
  • Intermediate Filament Proteins (biosynthesis, metabolism)
  • Membrane Glycoproteins (biosynthesis, metabolism)
  • Nerve Tissue Proteins (biosynthesis, metabolism)
  • Nestin
  • Neuroblastoma (diagnosis, metabolism, pathology)
  • Peripherins

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