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Adenoid cystic carcinoma

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Adenoid cystic carcinoma

  1. 1. Adenoid cystic carcinoma
  2. 2. Definition Adenoid cystic carcinoma is a slow growing and relentless salivary gland malignancy composed of epithelial and myoepithelial neoplastic cells that form various patterns, including tubular, cribriform , and solid forms.
  3. 3. Epidemiology • the female-to-male ratio is about (1.5:1) • there is no ethnic predilection • wide patient age range, but are most common in the sixth to eighth decades of life. • ACC accounts for < 1 % of all head and neck cancers and < 10% of all salivary gland neoplasms.
  4. 4. Localization • Occurs most frequently in the major salivary glands, but more than one third of cases occur in minor glands in the oral cavity, (rarely) other sites. • metastases occur in 40-60 % of patients.
  5. 5. Clinical features Patients usually present with: swelling or masses, and may have numbness, paraesthesia, or pain. Involvement of motor nerves can cause facial or tongue weakness. • Most adenoid cystic carcinomas are widely infiltrative at diagnosis, invade bone early, and characteristically show perineural infiltration.
  6. 6. Histopathology A system of three histological patterns has been shown to be of prognostic value; this is based on the relative proportions of tubular, cribriform and solid patterns. • the cribriform pattern: The most recognizable architectural form, characterized by nests of tumour cells interrupted by sharply punched-out spaces filled with basophilic matrix. • The tubular pattern: is composed of bilayered tubules with lumina. The tumour cells show scant cytoplasm and typically have small angulated and hyperchromatic nuclei. • The solid growth pattern: is characterized by sheets of tumour cells without lumen formation and may consist of epithelial or myoepithelial elements.
  7. 7. Grading: • Depends on a simple analysis of the morphological pattern of the tumour: 1. tubular (low grade) 2. cribriform (intermediate grade) 3. solid (high grade) Some high-grade tumours also show “dedifferentiation”.
  8. 8. Immunohistochemical • Adenoid cystic carcinoma expresses both ductal and myoepithelial/basal cell markers, such as CK7, CAM 5.2, calponin, SMA, SMMHC, p63, CD117, SOX10, and S100. • MYB and MYB-NFIB. • c-KIT expression • Ki-67 and p53 labelling
  9. 9. Genetic profile The adenoid cystic carcinoma-specific t(6;9) chromosomal translocation, resulting in a MYB- NFIB gene fusion. The key genomic alterations are at (6;9) chromosomal translocation or more rarely a t(8;9) translocation, resulting in fusions involving the MYB or MYBL1 oncogenes and the transcription factor gene NFIB.
  10. 10. In addition, over 80% of adenoid cystic carcinomas show a translocation involving the MYB oncogene and the transcription factor gene NFIB. The translocation can be detected by FISH and an antibody to the MYB-NFIB fusion protein has also been developed
  11. 11. Perineural invasion • and is a feature of most adenoid cystic carcinomas However, two studies have found it to be an independent factor in prognosis, and it is a means by which the tumour can grow along a plane of low tissue resistance. • Even anatomical barriers can be breached since all are pierced by neurovascular bundles. It is particularly important when it occurs outside the main tumour mass, or causes clinical manifestations. • Clinical evidence of nerve invasion is a poor prognostic indicator. Such evidence may become manifest as paralysis, paraesthesia, deafness, diplopia, pain or tic.
  12. 12. (Barrett & Speight, 2009) Perineural invasion in adenoid cystic carcinoma of the salivary glands: A valid prognostic indicator? • The answer to the question posed at the beginning of this article, ‘‘does histological evidence of PNI affect the patient’s prognosis?” • “Certainly ‘‘yes”. There is no consensus that PNI correlates with site, histological grade or other clinicopathological variables. However, the majority of studies show PNI to be an adverse factor for one or more outcome indicators.”
  13. 13. Prognosis and predictive factors • The 10-year survival rate is 50-70% and the local recurrence rate is highly variable. Lymph node involvement is uncommon, but is more frequent in solid varian. • Distant metastasis is reported in > 50% of cases, most commonly to the lungs, followed by bone, liver, and brain.
  14. 14. Factors that influence survival include tumour stage, node status, patient age, tumour site, large nerve perineural invasion, and surgical margins. Generally, tumours with tubular and cribriform growth patterns have a less aggressive clinical course than do tumours with a solid component constituting more than one third of the tumour.
  15. 15. References 1. Barrett, A. W., & Speight, P. M. (2009). Perineural invasion in adenoid cystic carcinoma of the salivary glands : A valid prognostic indicator ? Oral Oncology, 45(11), 936–940. https://doi.org/10.1016/j.oraloncology.2009.07.001 2. Khurram, S. A., Barrett, A. W., & Speight, P. M. (2017). Diagnostic difficulties in lesions of the minor salivary glands. Diagnostic Histopathology, 23(6), 250–259. https://doi.org/10.1016/j.mpdhp.2017.04.008 3. Simpson, R. H. W., Skálová, A., Di Palma, S., & Leivo, I. (2014). Recent advances in the diagnostic pathology of salivary carcinomas. Virchows Archiv, 465(4), 371–384. https://doi.org/10.1007/s00428-014- 1639-x 4. Speight, P. M., & Barrett, A. W. (2009). Leading article Prognostic factors in malignant tumours of the salivary glands, 47, 587–593. https://doi.org/10.1016/j.bjoms.2009.03.017 5. Zhu, S., Schuerch, C., & Hunt, J. (2005). Review and Updates of Immunohistochemistry in Selected Salivary Gland and Head and Neck Tumors. https://doi.org/10.5858/arpa.2014-0167-RA 6. WHO Classification of Head and Neck Tumours, 4ed, (2017) 7. Van Weert, S., Van Der Waal, I., Witte, B. I., René Leemans, C., & Bloemena, E. (2015). Histopathological grading of adenoid cystic carcinoma of the head and neck: Analysis of currently used grading systems and proposal for a simplified grading scheme. Oral Oncology, 51(1), 71–76. https://doi.org/10.1016/j.oraloncology.2014.10.007