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adenoid cystic carcinoma

Monday 9 August 2004

Digital cases

- JRC:6088 : Pulmonary adenoid cystic carcinoma.
- JRC:6089 : Pulmonary adenoid cystic carcinoma.
- JRC:6090 : Pulmonary adenoid cystic carcinoma.
- JRC:6092 : Pulmonary adenoid cystic carcinoma.
- JRC:6093 : Pulmonary adenoid cystic carcinoma.
- Adenoid Cystic Carcinoma of the Sphenoid Sinus by Ed Euthman.

Localization

- salivary adenoid cystic carcinoma
- pulmonary adenoid cystic carcinoma
- prostatic adenoid cystic carcinoma

Etiology

- somatic mutations in genes coding for components of the Wnt signaling pathway (35%) (15195113)

- The recurrent t(6;9)(q22-23;p23-24) translocation in adenoid cystic carcinoma results in a novel fusion of the MYB proto-oncogene with the transcription factor gene NFIB. (21572406)

  • MYB-NFIB fusion transcript is detected in 86% of adenoid cystic carcinoma tumor frozen samples and 44% formalin-fixed paraffin-embedded adenoid cystic carcinoma tumor specimens. (21572406)
  • The MYB-NFIB fusion is not expressed in non-adenoid cystic carcinoma neoplasms of the head and neck, confirming the high specificity of the MYB-NFIB fusion. (21572406)
  • Adenoid cystic carcinomas from various anatomic sites, including salivary gland, sinonasal cavity, tracheobronchial tree, larynx, breast, and vulva are repeatedly fusion-positive, indicating that adenoid cystic carcinomas located in different anatomic sites not only have important morphologic features in common, but also probably evolve through activation of the same molecular pathways. (21572406)
  • 89% of the tumors, including both fusion-positive and fusion-negative cases, overexpressed MYB RNA. (21572406)
  • 82% of adenoid cystic carcinomas stained positive for MYB protein, compared with 14% of non-adenoid cystic carcinoma neoplasms, indicating that MYB immunostaining may be useful for the diagnosis of adenoid cystic carcinoma, but that neoplasms sometimes in the differential diagnosis are also labeled. The latter are, however, fusion-negative. (21572406)
  • MYB activation through gene fusion or other mechanisms is a major oncogenic event in adenoid cystic carcinoma occurring at various anatomic sites.
  • In addition to being a diagnostically useful biomarker for adenoid cystic carcinoma, MYB and its downstream effectors are also novel potential therapeutic targets.

Links

- Adenoid Cystic Carcinoma Organization

See also

- adenoid cystic carcinoma

References

- Analysis of MYB expression and MYB-NFIB gene fusions in adenoid cystic carcinoma and other salivary neoplasms. Brill LB 2nd, Kanner WA, Fehr A, Andrén Y, Moskaluk CA, Löning T, Stenman G, Frierson HF Jr. Mod Pathol. 2011 May 13. PMID: 21572406