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“Pleomorphic Adenoma”, Victor McKusick, Mendelian Inheritance in Man, 1966. 多型性腺瘤。icd10=D11.0

 

 



Here I present: Pleomorphic Adenoma”, Victor McKusick, Mendelian Inheritance in Man’, 1966. 多型性腺瘤。icd10=D11.0

INTRODUCTION.

Pleomorphic adenoma is a common benign salivary gland neoplasm characterized by neoplastic proliferation of epithelial (ductal) cells along with myoepithelial components, having a malignant potentiality. It is the most common type of salivary gland tumor and the most common tumor of the parotid gland. It derives its name from the architectural pleomorphism (variable appearance) seen by light microscopy. It is also known as “Mixed salivary gland tumor“, which refers to its dual origin from epithelial and myoepithelial elements’ as opposed to its pleomorphic appearance.

There is evidence that pleomorphic adenoma is caused by mutation in the PLAGL1 encoded zinc finger DNA-binding transcription factor that can regulate apoptosis and cell cycle arrest.  The PLAG1 gene is encoded on cytogenetic location 6q24.2 and genomic coordinates 6:143,940,300-144,064,599.  The screenshot of the PLAG1  gene 124,300 bp (base pairs) of DNA sequence length is shown BELOW. Nine (9) other genes besides PLAG1 in the 6q24.2 cytogenetic location are listed BENEATH.

 

 




 

Coordinate  Symbol  Genomic Name
6:143,450,805  PEX3 Peroxisomal biogenesis factor-3
6:143,494,812  FUCA2 Fucosidase, alpha-L- 2, plasma
6:143,536,878  PHACTR2 Phosphatase and actin regulator 2
6:143,843,338  LTV1 LTV1 ribosome biogenesis factor
6:143,940,300  PLAGL1 Pleomorphic adenoma gene-like 1 
6:144,004,916  HYMAI Hydatidiform mole-associated and imprinted transcript
6:144,094,884  SF3B5 Splicing factor 3B, subunit 5
6:144,139,963  STX11 Syntaxin-11
6:144,285,335  UTRN Utrophin (homologous to dystrophin)
6:145,383,353  EPM2A EPM2A glucan phosphatase, laforin

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