Making a correct diagnosis when dealing with a small round blue cell tumor (SRBCT) of children and adolescents may be relatively straightforward if the tumor arises in the typical clinical setting and the classic pathologic features are all recognizable. However it is widely known that diagnostic difficulties may arise because of: (i) many tumors share overlapping morphological and/or immunohistochemical features; (ii) considerable clinical, pathologic, and immunohistochemical variations do exist; (iii) the increasing use of small biopsies in daily practice makes the diagnosis of these neoplasms more challenging. Accordingly, immunohistochemical analyses are currently mandatory in establishing the correct diagnosis. In this regard there is the need to identify more sensitive and specific immunomarkers useful in the distinction of the several tumor entities. Over the last decades, several markers, such as CD99, WT1 protein, desmin, myogenin, NB84, and INI1 have been identified, providing a considerable help in recognition of the most common solid tumors (ESW/pPNET, rhabdomyosarcoma, neuroblastoma, Wilms' tumor, desmoplastic small round cell tumor; malignant rhabdoid tumor) in children and adolescents. However, at the same time, their unusual, unexpected expression can result in a misinterpretation of the immunohistochemical results, especially by pathologists who are not familiar with oncologic pediatric pathology. Therefore the present review focuses on the potential immunohistochemical pitfalls which should be kept in mind by pathologists to prevent diagnostic errors when dealing with SRBCTs.
Keywords: Adolescents; Children; Immunohistochemistry; Review; Small round blue cell tumors.
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