Difference between revisions of "Adenoid cystic carcinoma"

From Libre Pathology
Jump to navigation Jump to search
(+cat.)
(split out)
Line 1: Line 1:
#redirect [[Salivary_glands#Adenoid_cystic_carcinoma]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      =
| Width      =
| Caption    =
| Micro      =
| Subtypes  =
| LMDDx      =
| Stains    =
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[salivary gland]], [[breast]], [[lung]]
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence =
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    =
}}
'''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]]

Revision as of 21:07, 17 July 2013

Adenoid cystic carcinoma
Diagnosis in short
Site salivary gland, breast, lung

'Adenoid cystic carcinoma, abbreviated AdCC, is a malignant tumour commonly seen in the salivary gland.

General

  • Common malignant neoplasm of salivary gland.
  • AKA cylindroma.[1]
  • 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:

  • AdCC - mostly DNA (scant cytoplasm), distinct nucleus (carrot-shaped).

Notes:

DDx:

Images

  • AdCC - intermed. mag. (WC/Nephron)

  • AdCC - high mag. (WC/Nephron)

  • AdCC - very high mag. (WC/Nephron)

www:

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.[2]

IHC

Features:[3]

  • CD117 +ve.
  • Cyclin D1 +ve.
  • Myoepithelial markers (e.g. calponin, actin) +ve.
    • Typically -ve in PLGA.

Molecular

Features:[4]

  • t(6;9) MYB-NFIB.
    • Seen in ~50% of cases.
    • Worse prognosis if present, esp. if fusion assoc. with transcription.

See also

References

  1. ↑ Chest. May 1957. Vol. 31. No. 5. PP. 493-511. http://www.chestjournal.org/content/31/5/493.abstract
  2. ↑ URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970070-5. Accessed on: 12 May 2011.
  3. ↑ Sequeiros-Santiago, G.; García-Carracedo, D.; Fresno, MF.; Suarez, C.; Rodrigo, JP.; Gonzalez, MV. (May 2009). "Oncogene amplification pattern in adenoid cystic carcinoma of the salivary glands.". Oncol Rep 21 (5): 1215-22. PMID 19360297.
  4. ↑ Mitani, Y.; Rao, PH.; Futreal, PA.; Roberts, D.; Stephens, P.; Zhao, YJ.; Zhang, L.; Mitani, M. et al. (Oct 2011). "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.". Clin Cancer Res. doi:10.1158/1078-0432.CCR-11-1870. PMID 21976542.
Retrieved from "https://librepathology.org/w/index.php?title=Adenoid_cystic_carcinoma&oldid=23122"