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'''Adenoid cystic carcinoma''', abbreviated '''AdCC'', is a malignant tumour commonly seen in the [[salivary gland]]. | |||
==General== | |||
*Common malignant neoplasm of salivary gland. | |||
*AKA ''cylindroma''.<ref>Chest. May 1957. Vol. 31. No. 5. PP. 493-511. [http://www.chestjournal.org/content/31/5/493.abstract http://www.chestjournal.org/content/31/5/493.abstract]</ref> | |||
**Should ''not'' be confused with ''[[dermal cylindroma]]'' (a benign skin tumour). | |||
*Composed of ductal cells and myoepithelial cells; '''myoepithelial cells''' > ductal cells. | |||
==Microscopic== | |||
Features: | |||
*Cribriform architecture ''or'' pseudoglandular spaces (classic pattern) - '''important feature'''. | |||
**Other patterns: solid, cords, (bilayered) tubules. | |||
**Cystic spaces filled with basophilic material (that is PAS +ve) - '''key feature'''. | |||
*Scant cytoplasm in most cells (myoepithelial cells) - clear/eosinophilic. | |||
**Moderate eosinophilic cytoplasm in the (rare) ductal cells. | |||
*Nucleus - small. | |||
**May be angulated (carrot-shaped) - myoepithelial cells; round/ovoid in ductal cells. | |||
*Hyaline stroma. | |||
Memory device: | |||
*A'''d'''CC - mostly '''D'''NA (scant cytoplasm), distinct nucleus (carrot-shaped). | |||
Notes: | |||
*'''Squamous differentiation is extremely rare'''. It presence should prompt consideration of: | |||
**Basaloid [[squamous cell carcinoma]]. | |||
**[[Basal cell carcinoma]] (BCC). | |||
DDx: | |||
*[[Pleomorphic adenoma]], esp. if encapsulated. | |||
===Images=== | |||
<gallery> | |||
Image:Adenoid_cystic_carcinoma_-_intermed_mag.jpg | AdCC - intermed. mag. (WC/Nephron) | |||
Image:Adenoid_cystic_carcinoma_-_high_mag.jpg | AdCC - high mag. (WC/Nephron) | |||
Image:Adenoid_cystic_carcinoma_-a-_very_high_mag.jpg | AdCC - very high mag. (WC/Nephron) | |||
</gallery> | |||
www: | |||
*[http://www.nature.com/modpathol/journal/v16/n12/fig_tab/3880915f2.html Adenoid cystic carcinoma (nature.com)]. | |||
===Grading=== | |||
Based on solid component: | |||
*Low grade = tubules and cribriform structures only; no solid component. | |||
*Intermediate grade = solid component <30%. | |||
*High grade = solid component >=30% | |||
==Stains== | |||
Special stains: | |||
*PAS +ve material - cystic spaces.<ref name=pc_add>URL: [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970070-5 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970070-5]. Accessed on: 12 May 2011.</ref> | |||
==IHC== | |||
Features:<ref name=pmid19360297>{{Cite journal | last1 = Sequeiros-Santiago | first1 = G. | last2 = García-Carracedo | first2 = D. | last3 = Fresno | first3 = MF. | last4 = Suarez | first4 = C. | last5 = Rodrigo | first5 = JP. | last6 = Gonzalez | first6 = MV. | title = Oncogene amplification pattern in adenoid cystic carcinoma of the salivary glands. | journal = Oncol Rep | volume = 21 | issue = 5 | pages = 1215-22 | month = May | year = 2009 | doi = | PMID = 19360297 }}</ref> | |||
*CD117 +ve. | |||
*Cyclin D1 +ve. | |||
*Myoepithelial markers (e.g. calponin, actin) +ve. | |||
**Typically -ve in PLGA. | |||
==Molecular== | |||
Features:<ref>{{Cite journal | last1 = Mitani | first1 = Y. | last2 = Rao | first2 = PH. | last3 = Futreal | first3 = PA. | last4 = Roberts | first4 = D. | last5 = Stephens | first5 = P. | last6 = Zhao | first6 = YJ. | last7 = Zhang | first7 = L. | last8 = Mitani | first8 = M. | last9 = Weber | first9 = RS. | title = Novel Chromosomal Rearrangements and breakpoints at the t(6;9) in Salivary Adenoid Cystic Carcinoma: association with MYB-NFIB chimeric fusion, MYB expression, and clinical outcome. | journal = Clin Cancer Res | volume = | issue = | pages = | month = Oct | year = 2011 | doi = 10.1158/1078-0432.CCR-11-1870 | PMID = 21976542 }}</ref> | |||
*t(6;9) MYB-NFIB. | |||
**Seen in ~50% of cases. | |||
**Worse prognosis if present, esp. if fusion assoc. with transcription. | |||
==See also== | |||
*[[Salivary glands]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Head and neck pathology]] |
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