Adenoid cystic carcinoma

Source: Wikipedia, the free encyclopedia.
Adenoid cystic carcinoma
Other namesAdenocyst, Malignant cylindroma, Adenocystic, Adenoidcystic
Micrograph of an adenoid cystic carcinoma of a salivary gland (right of image): Normal serous glands, typical of the parotid gland, are also seen (left of image), H&E stain.
SpecialtyOncology Edit this on Wikidata

Adenoid cystic carcinoma is a rare type of

trachea, and the paranasal sinuses
.

It is the third-most common malignant salivary gland tumor overall (after

polymorphous adenocarcinoma). It represents 28% of malignant submandibular gland tumors, making it the single most common malignant salivary gland tumor in this region. Patients may survive for years with metastases because this tumor is generally well-differentiated and slow growing. In a 1999 study[3] of a cohort of 160 ACC patients, disease-specific survival was 89% at 5 years, but only 40% at 15 years,[citation needed
] reflecting deaths from late-occurring metastatic disease.

Cause

Activation of the oncogenic transcription factor gene MYB is the key genomic event of ACC and seen in the vast majority of cases.[4][5] Most commonly, MYB is activated through gene fusion with the transcription factor encoding NFIB gene as a result of a t(6;9) translocation.[4] Alternatively, MYB is activated by copy number gain or by juxtaposition of enhancer elements in the vicinity of the MYB gene.[6] In a subset of ACCs, the closely related MYBL1 gene is fused to NFIB or to other fusion partners.[7][8]

MYB drives proliferation of ACC cells and regulates genes involved in cell cycle control, DNA replication and repair, and RNA processing.[9] Thus, the MYB oncogene is a potential diagnostic and therapeutic target in ACC.

ACC has a relatively quiet genome with few recurrent copy number alterations or point mutations,[6][10][11][12] consistent with the view that MYB and MYBL1 are the main oncogenic drivers of the disease.

Treatment

Primary treatment for this cancer, regardless of body site, is surgical removal with clean

radiotherapy is commonly given following surgery. For advanced major and minor salivary gland tumors that are inoperable, recurrent, or exhibit gross residual disease after surgery, fast neutron therapy is widely regarded as the most effective form of treatment.[13][14][15][16]
metastatic disease. Chemotherapy is considered on a case-by-case basis, as data on the positive effects of chemotherapy are limited. Clinical studies are ongoing, however.[citation needed
]

Images

  • Coronal MRI showing right parotid adenoid cystic carcinoma with perineural spread of tumor: The tumor originates in the right parotid gland and spreads along the trigeminal nerve via the auricuotemporal branch extending intracranially through the foramen ovale at the skull base towards Meckel's cave.
    Coronal MRI showing right parotid adenoid cystic carcinoma with perineural spread of tumor: The tumor originates in the right parotid gland and spreads along the trigeminal nerve via the auricuotemporal branch extending intracranially through the foramen ovale at the skull base towards Meckel's cave.
  • Coronal MRI showing right parotid adenoid cystic carcinoma with perineural spread of tumor along the facial nerve extending to the stylomastoid foramen
    Coronal MRI showing right parotid adenoid cystic carcinoma with perineural spread of tumor along the facial nerve extending to the stylomastoid foramen
  • Histopathological image of adenoid cystic carcinoma of the salivary gland infiltrating a nerve (center), H&E stain
    Histopathological image of adenoid cystic carcinoma of the salivary gland infiltrating a nerve (center), H&E stain
  • Histopathological image of adenoid cystic carcinoma of the salivary gland, immunostain for S-100 protein
    Histopathological image of adenoid cystic carcinoma of the salivary gland, immunostain for S-100 protein
  • Micrograph of adenoid cystic carcinoma, fine needle aspiration specimen, Pap stain
    Micrograph of adenoid cystic carcinoma,
    Pap stain
  • Adenoid cystic carcinoma with comedonecrosis
    Adenoid cystic carcinoma with comedonecrosis
  • Relative incidence of submandibular tumors, with adenoid cystic carcinoma being the second most common.[17]
    Relative incidence of submandibular tumors, with adenoid cystic carcinoma being the second most common.[17]
  • Relative incidence of parotid tumors, with adenoid cystic carcinoma being relatively rare.[17]
    Relative incidence of parotid tumors, with adenoid cystic carcinoma being relatively rare.[17]

See also

References

  1. PMID 20203221
    .
  2. S2CID 5935648
    .
  3. PMID 10037280
    .
  4. ^
    PMID 19841262
    .
  5. PMID 27101980
    .
  6. ^
    S2CID 25742114
    .
  7. PMID 26631070
    .
  8. PMID 26631609
    .
  9. PMID 28954282
    .
  10. PMID 23685749
    .
  11. PMID 23778141
    .
  12. PMID 26862087
    .
  13. PMID 3114190
    .
  14. PMID 8949762
    .
  15. PMID 10701733
    .
  16. PMID 10897760
    .
  17. ^ a b Lee SC (23 December 2021). "Salivary Gland Neoplasms". Medscape. Updated: Jan 13, 2021
    Diagrams by Mikael Häggström, MD

Further reading

  • Neville, Damm, Allen, Bouquot. Oral and Maxillofacial Pathology. 2nd edition.

External links

  • [1] Search for clinical trials at ClinicalTrials.gov
  • [2] Search for research literature at PubMed.gov
  • [3] Search for consumer health information at Medlineplus.gov
Retrieved from "https://en.wikipedia.org/w/index.php?title=Adenoid_cystic_carcinoma&oldid=1187329223"