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Head and Neck: Salivary gland tumors: Adenoid cystic carcinoma with t(6;9)(q23;p24) MYB/PDCD1LG2

Written2013-12Yoshitsugu Mitani, Adel K El-Naggar
Departments of Pathology, The University of Texas MD Anderson Cancer Center, Houston, Texas, United States of America.

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Identity

Atlas_Id 6403
Phylum Phylum; Salivary gland: Adenoid cystic carcinoma.

Clinics and Pathology

Disease Adenoid cystic carcinoma
Note The tumor is characterized by a relatively stable genome and indolent and progressive clinical behavior. Significant number of ACC manifest t(6;9) translocation, which may lead to fusions of the MYB-NFIB genes. We identified a different gene fusion involving MYB/PDCD1LG2 in an ACC with t(6:9).
Clinics Adenoid cystic carcinoma (ACC) is second most common malignancy of human salivary glands. ACC is slow progressing malignancy with a lethal outcome. The primary treatment for patients with ACC is surgical resection with and without post-operative radiotherapy. Patients with recurrent and distant metastatic disease have limited therapeutic options.
Pathology Histopathologically, ACC manifests three distinctive morphologic patterns that include cribriform, tubular and solid forms. The organization of the tubular and cribiform patterns is attributed to the presence of myoepithelial cells while the evolution of the solid form is coincident with the loss of these cells.

Cytogenetics

Cytogenetics
Morphological
t(6;9)(q23;p24) translocation results in a fusion transcript consisting of MYB and NFIB 6 the most common finding. A novel fusion between the MYB gene and the PDCD1LG2 gene was recently identified in an ACC (Mitani et al., 2010; Mitani et al., 2011).

Genes involved and Proteins

Gene Name MYB
Location 6q22-q23
Dna / Rna The nucleotide sequences and genomic organization of MYB (accession number NM_001130173) was obtained from the National Center for Biotechnology Information (NCBI; http://www.ncbi.nlm.nih.gov)

Gene Name PDCD1LG2
Location 9p24.2
Dna / Rna The nucleotide sequences and genomic organization of PDCD1LG2 (accession number NM_025239) was obtained from the National Center for Biotechnology Information (NCBI; http://www.ncbi.nlm.nih.gov)

Result of the chromosomal anomaly

Hybrid Gene
 
MYB-PDCD1LG2 hybrid gene
Description Inverted intron 3 of PDCD1LG2 gene was fused with MYB gene on exon 15. One of 102 patients with ACC was identified this fusion by 3'RACE technique.
Detection 3'RACE was performed to identify the unknown gene fusion of MYB and described by Mitani Y et al., 2010.
Fusion Protein
Note No evidence for fusion protein is documented.
Anti-MYB antibody recognized N-terminal of MYB confirmed the presence of truncated MYB protein by western blotting. The tumor is MYB positive by immunohistochemical analysis using the same anti-MyB anti-body.
  

Bibliography

PMID 21976542
 

Citation

This paper should be referenced as such :
Mitani Y, El-Naggar AK
Head and Neck: Salivary gland tumors: Adenoid cystic carcinoma with t(6;9)(q23;p24) MYB/PDCD1LG
Atlas Genet Cytogenet Oncol Haematol. in press
On line version : http://AtlasGeneticsOncology.org/Tumors/t0609CarcSalivGlandID6403.html


Translocations implicated (Data extracted from papers in the Atlas)

 t(6;9)(q23;p24) MYB/PDCD1LG2

External links

Mitelman database t(6;9)(q23;p24) MYB/PDCD1LG2 [Case List]    t(6;9)(q23;p24) MYB/PDCD1LG2 [Association List] Mitelman database (CGAP - NCBI)
arrayMap arrayMap ((UZH-SIB Zurich)   [auto + random 100 samples .. if exist ]   [tabulated segments]
 
Mitelman databaseMYB/PDCD1LG2[MCList]    MYB (6q23.3) PDCD1LG2 (9p24.1)   t(6;9)(q23;p24)
 
Disease databaseHead and Neck: Salivary gland tumors: Adenoid cystic carcinoma with t(6;9)(q23;p24) MYB/PDCD1LG2
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed


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