Difference between revisions of "Adenoid cystic carcinoma"

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#redirect [[Salivary_glands#Adenoid_cystic_carcinoma]]
{{ 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]]

Revision as of 17:07, 9 May 2018

Adenoid cystic carcinoma
Diagnosis in short

Adenoid cystic carcinoma. H&E stain.
LM cribriform architecture (other patterns: solid, cords, (bilayered) tubules), cystic spaces filled with basophilic material, scant cytoplasm in most cells, nucleus - small, hyaline stroma
LM DDx pleomorphic adenoma, epithelial-myoepithelial carcinoma, intraductal carcinoma
Site salivary gland, breast, lung, skin, trachea, others
Signs mass
Prevalence relatively common malignant salivary gland tumour
Treatment surgical excision

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

General

  • Common malignant neoplasm of salivary gland.[1]
  • May occur in the skin.[2]
  • AKA cylindroma.[3]
  • 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 - low mag. (WC/Nephron)

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

  • AdCC - high mag. (WC/Nephron)

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

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

  • AdCC of breast - solid type (WC/Sarahkayb)

  • AdCC trachea - low mag.

  • AdCC trachea - intermed. mag.

  • AdCC trachea - high mag.

  • AdCC trachea - very high mag.

  • AdCC trachea - very high mag.

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.[4]

IHC

Features:[5]

  • CD117 +ve.
  • Cyclin D1 +ve/-ve.

Others:[6]

Note:

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

Molecular

Features:[7]

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

See also

References

  1. ↑ Krüll, A.; Schwarz, R.; Engenhart, R.; Huber, P.; Lessel, A.; Koppe, H.; Favre, A.; Breteau, N. et al. (1996). "European results in neutron therapy of malignant salivary gland tumors.". Bull Cancer Radiother 83 Suppl: 125-9s. PMID 8949764.
  2. ↑ Wick, MR.; Swanson, PE. (Feb 1986). "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.". Am J Dermatopathol 8 (1): 2-13. PMID 3010759.
  3. ↑ Chest. May 1957. Vol. 31. No. 5. PP. 493-511. http://www.chestjournal.org/content/31/5/493.abstract
  4. ↑ URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970070-5. Accessed on: 12 May 2011.
  5. ↑ 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.
  6. ↑ Thompson, LD.; Penner, C.; Ho, NJ.; Foss, RD.; Miettinen, M.; Wieneke, JA.; Moskaluk, CA.; Stelow, EB. (Sep 2013). "Sinonasal Tract and Nasopharyngeal Adenoid Cystic Carcinoma: A Clinicopathologic and Immunophenotypic Study of 86 Cases.". Head Neck Pathol. doi:10.1007/s12105-013-0487-3. PMID 24037641.
  7. ↑ 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.
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