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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Adenoid cystic carcinoma - high mag.jpg | |||
| Width = | |||
| Caption = Adenoid cystic carcinoma. [[H&E stain]]. | |||
| Micro = [[cribriform architecture]] (other patterns: solid, cords, (bilayered) tubules), cystic spaces filled with basophilic material, scant cytoplasm in most cells, nucleus - small, hyaline stroma | |||
| Subtypes = | |||
| LMDDx = [[pleomorphic adenoma]], [[epithelial-myoepithelial carcinoma]], [[intraductal carcinoma of the salivary gland|intraductal carcinoma]] | |||
| Stains = | |||
| IHC = | |||
| EM = | |||
| Molecular = | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Site = [[salivary gland]], [[breast]], [[lung]], [[skin]], [[trachea]], others | |||
| Assdx = | |||
| Syndromes = | |||
| Clinicalhx = | |||
| Signs = mass | |||
| Symptoms = | |||
| Prevalence = relatively common malignant salivary gland tumour | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = | |||
| Other = | |||
| ClinDDx = | |||
| Tx = surgical excision | |||
}} | |||
'''Adenoid cystic carcinoma''', abbreviated '''AdCC''', is a malignant tumour commonly seen in the [[salivary gland]]. | |||
==General== | |||
*Common malignant neoplasm of salivary gland.<ref name=pmid8949764>{{Cite journal | last1 = Krüll | first1 = A. | last2 = Schwarz | first2 = R. | last3 = Engenhart | first3 = R. | last4 = Huber | first4 = P. | last5 = Lessel | first5 = A. | last6 = Koppe | first6 = H. | last7 = Favre | first7 = A. | last8 = Breteau | first8 = N. | last9 = Auberger | first9 = T. | title = European results in neutron therapy of malignant salivary gland tumors. | journal = Bull Cancer Radiother | volume = 83 Suppl | issue = | pages = 125-9s | month = | year = 1996 | doi = | PMID = 8949764 }}</ref> | |||
*May occur in the skin.<ref name=pmid3010759>{{Cite journal | last1 = Wick | first1 = MR. | last2 = Swanson | first2 = PE. | title = Primary adenoid cystic carcinoma of the skin. A clinical, histological, and immunocytochemical comparison with adenoid cystic carcinoma of salivary glands and adenoid basal cell carcinoma. | journal = Am J Dermatopathol | volume = 8 | issue = 1 | pages = 2-13 | month = Feb | year = 1986 | doi = | PMID = 3010759 }}</ref> | |||
*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). | |||
*[[Perineural invasion]] is usually present. | |||
**If it is ''not'' one should consider other diagnoses. | |||
DDx: | |||
*[[Pleomorphic adenoma]], esp. if encapsulated. | |||
*[[Epithelial-myoepithelial carcinoma]] - esp. for AdCC tubular variant. | |||
*[[Human papillomavirus-related multiphenotypic sinonasal carcinoma]] (previously known as [[human papillomavirus-related carcinoma with adenoid cystic-like features]]). | |||
*[[Intraductal carcinoma of the salivary gland]]. | |||
===Images=== | |||
<gallery> | |||
Image:Adenoid cystic carcinoma - low mag.jpg | AdCC - low mag. (WC/Nephron) | |||
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 - very high mag.jpg | AdCC - very high mag. (WC/Nephron) | |||
Image:Adenoid_cystic_carcinoma_-a-_very_high_mag.jpg | AdCC - very high mag. (WC/Nephron) | |||
File:Breast_AdenoidCysticCarcinoma_SolidType_14BR***.jpg | AdCC of breast - solid type (WC/Sarahkayb) | |||
</gallery> | |||
<gallery> | |||
Image: Adenoid cystic carcinoma of the trachea -- low mag.jpg | AdCC [[trachea]] - low mag. | |||
Image: Adenoid cystic carcinoma of the trachea -- intermed mag.jpg | AdCC trachea - intermed. mag. | |||
Image: Adenoid cystic carcinoma of the trachea -- high mag.jpg | AdCC trachea - high mag. | |||
Image: Adenoid cystic carcinoma of the trachea -- very high mag.jpg | AdCC trachea - very high mag. | |||
Image: Adenoid cystic carcinoma of the trachea - alt -- very high mag.jpg | AdCC trachea - very high mag. | |||
</gallery> | |||
===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/-ve. | |||
Others:<ref name=pmid24037641>{{Cite journal | last1 = Thompson | first1 = LD. | last2 = Penner | first2 = C. | last3 = Ho | first3 = NJ. | last4 = Foss | first4 = RD. | last5 = Miettinen | first5 = M. | last6 = Wieneke | first6 = JA. | last7 = Moskaluk | first7 = CA. | last8 = Stelow | first8 = EB. | title = Sinonasal Tract and Nasopharyngeal Adenoid Cystic Carcinoma: A Clinicopathologic and Immunophenotypic Study of 86 Cases. | journal = Head Neck Pathol | volume = | issue = | pages = | month = Sep | year = 2013 | doi = 10.1007/s12105-013-0487-3 | PMID = 24037641 }}</ref> | |||
*[[CK7]] +ve. | |||
*[[Pankeratin]] +ve. | |||
*[[p63]] +ve. | |||
*Calponin +ve. | |||
*S-100 +ve. | |||
Note: | |||
*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]]. | |||
*[[Adenoid cystic carcinoma of the breast]]. | |||
*[[Adenoid cystic/basal cell carcinoma of the prostate]]. | |||
*[[Head and neck pathology]]. | |||
*[[Head and neck cytopathology]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Diagnosis]] | |||
[[Category:Head and neck pathology]] | |||
[[Category:Salivary gland]] |
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