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Intra-abdominal polyphenotypic tumor.
Pediatr Pathol Lab Med. 1996 Jan-Feb; 16(1):161-9.PP

Abstract

The presence of t(11;22)(q24;q12) is often considered diagnostic of Ewing sarcoma and peripheral primitive neuroectodermal tumor (pPNET). We report a case of a polyphenotypic tumor that possessed this translocation as detected by reverse transcriptase polymerase chain reaction (RT-PCR). This tumor was positive for vimentin, desmin, low-molecular-weight keratin, neuron-specific enolase, S-100 protein, and CD57 by immunohistochemistry. Of note, the tumor was negative for MIC2. The tumor had double-minute chromosomes with > 100 copies of the MDM2 gene. Thus, the presence of the t(11;22)(q24;q12) translocation should not be considered diagnostic of Ewing sarcoma and pPNET in the absence of supporting histologic evidence such as positive staining for MIC2. The presence of this translocation in Ewing sarcoma and pPNET has been taken as evidence that these two tumors are related. Rather than extending this relationship to include some polyphenotypic tumors, other tumors may acquire this genetic change during tumor progression. Treatment regimens for tumors may be better based on phenotype rather than genotype when these two profiles are seemingly in conflict.

Authors+Show Affiliations

Department of Pathology, Hospital for Sick Children, Toronto, Ontario, Canada.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

8963628

Citation

Thorner, P. "Intra-abdominal Polyphenotypic Tumor." Pediatric Pathology & Laboratory Medicine : Journal of the Society for Pediatric Pathology, Affiliated With the International Paediatric Pathology Association, vol. 16, no. 1, 1996, pp. 161-9.
Thorner P. Intra-abdominal polyphenotypic tumor. Pediatr Pathol Lab Med. 1996;16(1):161-9.
Thorner, P. (1996). Intra-abdominal polyphenotypic tumor. Pediatric Pathology & Laboratory Medicine : Journal of the Society for Pediatric Pathology, Affiliated With the International Paediatric Pathology Association, 16(1), 161-9.
Thorner P. Intra-abdominal Polyphenotypic Tumor. Pediatr Pathol Lab Med. 1996 Jan-Feb;16(1):161-9. PubMed PMID: 8963628.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Intra-abdominal polyphenotypic tumor. A1 - Thorner,P, PY - 1996/1/1/pubmed PY - 1996/1/1/medline PY - 1996/1/1/entrez SP - 161 EP - 9 JF - Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association JO - Pediatr Pathol Lab Med VL - 16 IS - 1 N2 - The presence of t(11;22)(q24;q12) is often considered diagnostic of Ewing sarcoma and peripheral primitive neuroectodermal tumor (pPNET). We report a case of a polyphenotypic tumor that possessed this translocation as detected by reverse transcriptase polymerase chain reaction (RT-PCR). This tumor was positive for vimentin, desmin, low-molecular-weight keratin, neuron-specific enolase, S-100 protein, and CD57 by immunohistochemistry. Of note, the tumor was negative for MIC2. The tumor had double-minute chromosomes with > 100 copies of the MDM2 gene. Thus, the presence of the t(11;22)(q24;q12) translocation should not be considered diagnostic of Ewing sarcoma and pPNET in the absence of supporting histologic evidence such as positive staining for MIC2. The presence of this translocation in Ewing sarcoma and pPNET has been taken as evidence that these two tumors are related. Rather than extending this relationship to include some polyphenotypic tumors, other tumors may acquire this genetic change during tumor progression. Treatment regimens for tumors may be better based on phenotype rather than genotype when these two profiles are seemingly in conflict. SN - 1077-1042 UR - https://www.unboundmedicine.com/medline/citation/8963628/Intra_abdominal_polyphenotypic_tumor_ L2 - https://antibodies.cancer.gov/detail/CPTC-MDM2-1 DB - PRIME DP - Unbound Medicine ER -