Difference between revisions of "Adenoid cystic carcinoma"
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| Width = | | Width = | ||
| Caption = Adenoid cystic carcinoma. [[H&E stain]]. | | 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 | | 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 = | | Subtypes = | ||
| LMDDx = [[pleomorphic adenoma]] | | LMDDx = [[pleomorphic adenoma]], [[epithelial-myoepithelial carcinoma]], [[intraductal carcinoma of the salivary gland|intraductal carcinoma]] | ||
| Stains = | | Stains = | ||
| IHC = | | IHC = | ||
| Line 14: | Line 14: | ||
| Gross = | | Gross = | ||
| Grossing = | | Grossing = | ||
| Site = [[salivary gland]], [[breast]], [[lung]] | | Site = [[salivary gland]], [[breast]], [[lung]], [[skin]], [[trachea]], others | ||
| Assdx = | | Assdx = | ||
| Syndromes = | | Syndromes = | ||
| Line 20: | Line 20: | ||
| Signs = mass | | Signs = mass | ||
| Symptoms = | | Symptoms = | ||
| Prevalence = | | Prevalence = relatively common malignant salivary gland tumour | ||
| Bloodwork = | | Bloodwork = | ||
| Rads = | | Rads = | ||
| Line 27: | Line 27: | ||
| Other = | | Other = | ||
| ClinDDx = | | ClinDDx = | ||
| Tx = surgical excision | |||
}} | }} | ||
'''Adenoid cystic carcinoma''', abbreviated '''AdCC''', is a malignant tumour commonly seen in the [[salivary gland]]. | '''Adenoid cystic carcinoma''', abbreviated '''AdCC''', is a malignant tumour commonly seen in the [[salivary gland]]. | ||
==General== | ==General== | ||
*Common malignant neoplasm of salivary gland. | *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> | *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). | **Should ''not'' be confused with ''[[dermal cylindroma]]'' (a benign skin tumour). | ||
| Line 38: | Line 40: | ||
==Microscopic== | ==Microscopic== | ||
Features: | Features: | ||
*Cribriform architecture ''or'' pseudoglandular spaces (classic pattern) - '''important feature'''. | *[[Cribriform architecture]] ''or'' pseudoglandular spaces (classic pattern) - '''important feature'''. | ||
**Other patterns: solid, cords, (bilayered) tubules. | **Other patterns: solid, cords, (bilayered) tubules. | ||
**Cystic spaces filled with basophilic material (that is PAS +ve) - '''key feature'''. | **Cystic spaces filled with basophilic material (that is PAS +ve) - '''key feature'''. | ||
| Line 54: | Line 56: | ||
**Basaloid [[squamous cell carcinoma]]. | **Basaloid [[squamous cell carcinoma]]. | ||
**[[Basal cell carcinoma]] (BCC). | **[[Basal cell carcinoma]] (BCC). | ||
*[[Perineural invasion]] is usually present. | |||
**If it is ''not'' one should consider other diagnoses. | |||
DDx: | DDx: | ||
*[[Pleomorphic adenoma]], esp. if encapsulated. | *[[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=== | ===Images=== | ||
<gallery> | <gallery> | ||
Image:Adenoid cystic carcinoma - low mag.jpg | AdCC - low mag. (WC/Nephron) | 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_-_intermed_mag.jpg | AdCC - intermed. mag. (WC/Nephron) | ||
| Line 66: | Line 72: | ||
Image:Adenoid cystic carcinoma - very high mag.jpg | AdCC - very 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) | 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> | </gallery> | ||
===Grading=== | ===Grading=== | ||
Based on solid component: | Based on solid component: | ||
| Line 82: | Line 93: | ||
==IHC== | ==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> | 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. | *[[CD117]] +ve. | ||
*Cyclin D1 +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. | *Myoepithelial markers (e.g. calponin, actin) +ve. | ||
**Typically -ve in PLGA. | **Typically -ve in [[PLGA]]. | ||
==Molecular== | ==Molecular== | ||
| Line 92: | Line 112: | ||
**Seen in ~50% of cases. | **Seen in ~50% of cases. | ||
**Worse prognosis if present, esp. if fusion assoc. with transcription. | **Worse prognosis if present, esp. if fusion assoc. with transcription. | ||
==Sign out== | |||
===Micro=== | |||
The sections show a tumour with a cribriform architecture. The cystic spaces have basophilic material. The tumour cells are carrot-like and have scant/modest cytoplasm. | |||
==See also== | ==See also== | ||
*[[Salivary glands]]. | *[[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== | ==References== | ||
| Line 101: | Line 129: | ||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Head and neck pathology]] | [[Category:Head and neck pathology]] | ||
[[Category:Salivary gland]] | |||
Latest revision as of 15:42, 20 December 2023
| 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]
- 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:
- AdCC - mostly DNA (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
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]
- 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:[7]
- t(6;9) MYB-NFIB.
- Seen in ~50% of cases.
- Worse prognosis if present, esp. if fusion assoc. with transcription.
Sign out
Micro
The sections show a tumour with a cribriform architecture. The cystic spaces have basophilic material. The tumour cells are carrot-like and have scant/modest cytoplasm.
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
- ↑ 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.
- ↑ 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.
- ↑ Chest. May 1957. Vol. 31. No. 5. PP. 493-511. http://www.chestjournal.org/content/31/5/493.abstract
- ↑ URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970070-5. Accessed on: 12 May 2011.
- ↑ 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.
- ↑ 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.
- ↑ 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.