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NKX2.2 is a useful immunohistochemical marker for Ewing sarcoma
Akihiko Yoshida1, Shigeki Sekine, Koji Tsuta
1Pathology and Clinical Laboratory Division, National Cancer Center Hospital, Tokyo, Japan. akyoshid@ncc.go.jp
The American Journal of Surgical Pathology
|March 27, 2012
Summary
NKX2.2 is a valuable new marker for diagnosing Ewing sarcoma, a rare bone cancer. This study shows NKX2.2 has high sensitivity and specificity, aiding in differentiating it from other small round cell tumors.
Area of Science:
- Oncology
- Pathology
- Molecular Biology
Background:
- Ewing sarcoma is a challenging pediatric and young adult bone cancer.
- Current markers like CD99 and FLI-1 have diagnostic limitations.
- NKX2.2, a target of the EWS-FLI-1 fusion protein, is upregulated in Ewing sarcoma.
Purpose of the Study:
- To evaluate the diagnostic utility of NKX2.2 immunohistochemistry in Ewing sarcoma.
- To determine the sensitivity and specificity of NKX2.2.
- To assess NKX2.2's role in the differential diagnosis of small round cell tumors.
Main Methods:
- Immunohistochemical staining for NKX2.2 was performed on 30 Ewing sarcomas and 130 non-Ewing small round cell tumors.
- Positive staining was defined as nuclear staining in at least 5% of tumor cells.
- Staining intensity and distribution were also evaluated.
Main Results:
- NKX2.2 was positive in 93% (28/30) of Ewing sarcoma cases.
- Staining was typically diffuse and moderate to strong in intensity.
- NKX2.2 showed positivity in 14 non-Ewing tumors, including olfactory neuroblastomas and small cell carcinomas, with 89% specificity.
Conclusions:
- NKX2.2 is a sensitive and specific marker for Ewing sarcoma.
- It aids in the differential diagnosis of small round cell tumors.
- NKX2.2 represents a valuable addition to the diagnostic immunohistochemical panel for Ewing sarcoma.
