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NKX2.2 is a useful immunohistochemical marker for Ewing sarcoma.
- Source :
-
The American journal of surgical pathology [Am J Surg Pathol] 2012 Jul; Vol. 36 (7), pp. 993-9. - Publication Year :
- 2012
-
Abstract
- Ewing sarcoma is a high-grade round cell sarcoma that affects bones and soft tissues in children and young adults. Its diagnosis can be challenging, and the differential diagnoses include a wide variety of small round cell tumors. CD99 and FLI-1 are the currently accepted immunohistochemical markers for Ewing sarcoma, but their accuracy has been controversial. NKX2.2 is a homeodomain-containing transcription factor that plays a critical role in neuroendocrine/glial differentiation. The NKX2.2 gene was recently identified as a target of EWS-FLI-1, the fusion protein specific to Ewing sarcoma, and was shown to be differentially upregulated in Ewing sarcoma on the basis of array-based gene expression analysis. However, the immunohistochemical diagnostic potential of this marker has not been tested. We immunostained representative sections of 30 genetically confirmed Ewing sarcomas and 130 non-Ewing small round cell tumors by using an antibody to NKX2.2. Nuclear staining in at least 5% of the cells was deemed positive. Twenty-eight (93%) of the 30 Ewing sarcomas were positive for NKX2.2. The staining was diffuse (>50%) in all the positive cases and was moderate or strong in intensity for most cases (25 of 28). NKX2.2 was also positive in 14 non-Ewing tumors, including all the olfactory neuroblastomas and a minor subset of small cell carcinomas, synovial sarcomas, mesenchymal chondrosarcomas, and malignant melanomas. All the other non-Ewing tumors tested were negative for this marker. NKX2.2 is a valuable marker for Ewing sarcoma, with a sensitivity of 93% and a specificity of 89%, and aids in the differential diagnosis of small round cell tumors.
- Subjects :
- Biomarkers, Tumor genetics
Bone Neoplasms genetics
Bone Neoplasms pathology
Diagnosis, Differential
Gene Rearrangement
Homeobox Protein Nkx-2.2
Humans
Japan
Nuclear Proteins
Predictive Value of Tests
RNA-Binding Protein EWS genetics
Sarcoma, Ewing genetics
Sarcoma, Ewing pathology
Sensitivity and Specificity
Zebrafish Proteins
Biomarkers, Tumor analysis
Bone Neoplasms chemistry
Homeodomain Proteins analysis
Immunohistochemistry
Sarcoma, Ewing chemistry
Transcription Factors analysis
Subjects
Details
- Language :
- English
- ISSN :
- 1532-0979
- Volume :
- 36
- Issue :
- 7
- Database :
- MEDLINE
- Journal :
- The American journal of surgical pathology
- Publication Type :
- Academic Journal
- Accession number :
- 22446943
- Full Text :
- https://doi.org/10.1097/PAS.0b013e31824ee43c