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ZBTB16: A new biomarker for primitive neuroectodermal tumor element / Ewing sarcoma
Guang-Qian Xiao1, Andy E Sherrod1, Kyle M Hurth1
1Department of Pathology, Keck School of Medicine, University of Southern California, Los Angeles, CA, 90033, United States.
Pathology, Research and Practice
|July 22, 2019
Summary
ZBTB16 is a sensitive and specific biomarker for primitive neuroectodermal tumors (PNET), effectively distinguishing them from other small round blue cell tumors. This finding aids in the diagnosis of pPNET and gPNET/cPNET.
Area of Science:
- Oncology
- Molecular Pathology
- Cancer Biomarkers
Background:
- Primitive neuroectodermal tumors (PNET) encompass peripheral (pPNET) and central (cPNET) nervous system types, with cPNET including germ cell tumor-derived PNET (gPNET).
- Currently, specific diagnostic markers for gPNET are lacking.
- PNETs share morphological similarities with other small round blue cell tumors, complicating diagnosis.
Purpose of the Study:
- To investigate the expression of ZBTB16 in various PNET subtypes and other small round blue cell tumors.
- To evaluate the potential diagnostic utility of ZBTB16 as a biomarker for PNETs.
- To differentiate PNETs from mimics like rhabdomyosarcoma and nephroblastoma.
Main Methods:
- Immunohistochemical analysis of ZBTB16 expression.
- Study cohort included 27 PNETs (pPNET, cPNET, gPNET) and 38 non-PNET small round blue cell tumors.
- Comparative analysis of ZBTB16 reactivity across tumor types.
Main Results:
- ZBTB16 was expressed in a high percentage of pPNET (75% diffuse), gPNET (43% diffuse), and cPNET (25% diffuse).
- All 38 non-PNET small round blue cell tumors showed no reactivity for ZBTB16.
- ZBTB16 expression differentiated PNETs from other small round blue cell tumors, including germ cell tumor-derived malignancies.
Conclusions:
- ZBTB16 is a highly sensitive and specific biomarker for pPNET and gPNET/cPNET.
- ZBTB16 aids in distinguishing PNETs from common small round blue cell tumor mimics.
- Variable ZBTB16 expression in cPNET may reflect its inherent heterogeneity.
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