Difference between revisions of "Polymorphous adenocarcinoma"

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'''Polymorphous_low-grade_adenocarcinoma''', abbreviated '''PLGA''', is a rare malignant [[salivary gland]] tumour.
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Polymorphous_low-grade_adenocarcinoma_high_mag.jpg
| Width      =
| Caption    = Polymorphous adenocarcinoma. [[H&E stain]].
| Synonyms  = polymorphmous low-grade adenocarcinoma (obsolete term)
| Micro      = low-grade cytology - nuclei ovoid & small, small nucleoli, powdery chromatin ([[papillary thyroid carcinoma]]-like appearance); eosinophilic cytoplasm; variable architecture - often small nests, classically has whorling ("eye-of-storm") pattern and single file pattern
| Subtypes  =
| LMDDx      = [[adenoid cystic carcinoma]], [[pleomorphic adenoma]], [[cribriform adenocarcinoma of the minor salivary gland]]
| Stains    =
| IHC        = S-100 +ve, CK7 +ve, p40 -ve, p63 +ve, vimentin +ve
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[oral cavity]], [[salivary gland]]
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = uncommon (varies by study)
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = good
| Other      =
| ClinDDx    =
}}
'''Polymorphous adenocarcinoma''', abbreviated '''PAC''', is a rare malignant [[salivary gland]] tumour.
 
It was previously known as '''polymorphous low-grade adenocarcinoma''', abbreviated '''PLGA'''.<ref name=pmid29761209>{{cite journal |authors=Vander Poorten V, Triantafyllou A, Skálová A, Stenman G, Bishop JA, Hauben E, Hunt JL, Hellquist H, Feys S, De Bree R, Mäkitie AA, Quer M, Strojan P, Guntinas-Lichius O, Rinaldo A, Ferlito A |title=Polymorphous adenocarcinoma of the salivary glands: reappraisal and update |journal=Eur Arch Otorhinolaryngol |volume=275 |issue=7 |pages=1681–1695 |date=July 2018 |pmid=29761209 |doi=10.1007/s00405-018-4985-5 |url=}}</ref>


==General==
==General==
*Almost exclusively in the oral cavity.
**Classically found in the palate -- 60% of PLGAs in palate.
*Tumour of the minor salivary glands.
*Always a low-grade tumour - by definition.
*Female:male ~= 2:1.
*Female:male ~= 2:1.
*Older people ~50-70 years old.
*Older people ~50-70 years old.
*Large variably in the prevalence reported - suggests a lacking of reproducibility in the [[diagnosis]].<ref name=pmid23320410/>
*Slow growing - metastases rare - prognosis good.<ref name=pmid18327037>{{Cite journal  | last1 = Paleri | first1 = V. | last2 = Robinson | first2 = M. | last3 = Bradley | first3 = P. | title = Polymorphous low-grade adenocarcinoma of the head and neck. | journal = Curr Opin Otolaryngol Head Neck Surg | volume = 16 | issue = 2 | pages = 163-9 | month = Apr | year = 2008 | doi = 10.1097/MOO.0b013e3282f70441 | PMID = 18327037 }}</ref><ref name=pmid23618791>{{Cite journal  | last1 = Fife | first1 = TA. | last2 = Smith | first2 = B. | last3 = Sullivan | first3 = CA. | last4 = Browne | first4 = JD. | last5 = Waltonen | first5 = JD. | title = Polymorphous low-grade adenocarcinoma: a 17 patient case series. | journal = Am J Otolaryngol | volume = 34 | issue = 5 | pages = 445-8 | month =  | year =  | doi = 10.1016/j.amjoto.2013.03.012 | PMID = 23618791 }}</ref>
==Gross==
*Tumour of the minor [[salivary gland]]s.<ref name=pmid24021444>{{Cite journal  | last1 = Andreu-Barasoain | first1 = M. | last2 = Vicente-Martín | first2 = FJ. | last3 = Gómez de la Fuente | first3 = E. | last4 = Salamanca-Santamaría | first4 = J. | last5 = Pampín-Franco | first5 = A. | last6 = López-Estebaranz | first6 = JL. | title = Polymorphous low-grade adenocarcinoma in the upper lip: a well-described but infrequently recognized tumor. | journal = Dermatol Online J | volume = 19 | issue = 8 | pages = 19265 | month =  | year = 2013 | doi =  | PMID = 24021444 }}</ref>
*Almost exclusively in the [[oral cavity]] - usually palate.<ref name=pmid18327037/>


==Microscopic==
==Microscopic==
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**Cytoplasm: eosinophilic.  
**Cytoplasm: eosinophilic.  
*Indistinct cell borders.  
*Indistinct cell borders.  
Note:
*''Pretty much always'' a low-grade tumour.


DDx:
DDx:
*[[Adenoid cystic carcinoma]].
*[[Adenoid cystic carcinoma]].
*[[Pleomorphic adenoma]].
*[[Pleomorphic adenoma]].
*Cribriform adenocarcinoma of the minor salivary gland.<ref name=pmid21716087>{{Cite journal | last1 = Skalova | first1 = A. | last2 = Sima | first2 = R. | last3 = Kaspirkova-Nemcova | first3 = J. | last4 = Simpson | first4 = RH. | last5 = Elmberger | first5 = G. | last6 = Leivo | first6 = I. | last7 = Di Palma | first7 = S. | last8 = Jirasek | first8 = T. | last9 = Gnepp | first9 = DR. | title = Cribriform adenocarcinoma of minor salivary gland origin principally affecting the tongue: characterization of new entity. | journal = Am J Surg Pathol | volume = 35 | issue = 8 | pages = 1168-76 | month = Aug | year = 2011 | doi = 10.1097/PAS.0b013e31821e1f54 | PMID = 21716087 }}
*[[Cribriform adenocarcinoma of the minor salivary gland]] - if one considers it a separate diagnosis.
</ref>
**The ''World Health Organization'' lumps ''[[cribriform adenocarcinoma of the minor salivary gland]]'' with ''polymorphous adenocarcinoma''.<ref name=pmid29761209>{{cite journal |authors=Vander Poorten V, Triantafyllou A, Skálová A, Stenman G, Bishop JA, Hauben E, Hunt JL, Hellquist H, Feys S, De Bree R, Mäkitie AA, Quer M, Strojan P, Guntinas-Lichius O, Rinaldo A, Ferlito A |title=Polymorphous adenocarcinoma of the salivary glands: reappraisal and update |journal=Eur Arch Otorhinolaryngol |volume=275 |issue=7 |pages=1681–1695 |date=July 2018 |pmid=29761209 |doi=10.1007/s00405-018-4985-5 |url=}}</ref>
 
===Images===
===Images===
<gallery>
<gallery>
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Image:Polymorphous_low-grade_adenocarcinoma_high_mag.jpg | PLGA - high mag. (WC/Nephron)
Image:Polymorphous_low-grade_adenocarcinoma_high_mag.jpg | PLGA - high mag. (WC/Nephron)
</gallery>
</gallery>
==IHC==
==IHC==
*S100 +ve, CK +ve, vimentin +ve.
Features:<ref name=pmid23320410>{{Cite journal  | last1 = de Araujo | first1 = VC. | last2 = Passador-Santos | first2 = F. | last3 = Turssi | first3 = C. | last4 = Soares | first4 = AB. | last5 = de Araujo | first5 = NS. | title = Polymorphous low-grade adenocarcinoma: an analysis of epidemiological studies and hints for pathologists. | journal = Diagn Pathol | volume = 8 | issue =  | pages = 6 | month =  | year = 2013 | doi = 10.1186/1746-1596-8-6 | PMID = 23320410 }}</ref>
*S-100 +ve.
*CK7 +ve.
*Vimentin +ve.
*p63 +ve (22 of 23 cases<ref name=pmid31653135/>).
*p40 -ve (22 of 23 cases<ref name=pmid31653135>{{cite journal |authors=Atiq A, Mushtaq S, Hassan U, Loya A, Hussain M, Akhter N |title=Utility of p63 and p40 in Distinguishing Polymorphous Adenocarcinoma and Adenoid Cystic Carcinoma |journal=Asian Pac J Cancer Prev |volume=20 |issue=10 |pages=2917–2921 |date=October 2019 |pmid=31653135 |pmc=6982655 |doi=10.31557/APJCP.2019.20.10.2917 |url=}}</ref>).
**Useful to differentiate from [[adenoid cystic carcinoma]] (AdCC); AdCC is usually p63 +ve/p40 +ve (36 of 47 cases) or p63 -ve/p40 -ve (10 of 47 cases).
***p63 +ve/p40 +ve is seen in pleomorphic adenoma and mucoepidermoid carcinoma (in addition to adenoid cystic carcinoma).<ref name=pmid34518135>{{cite journal |authors=Sivakumar N, Narwal A, Pandiar D, Devi A, Anand R, Bansal D, Kamboj M |title=Diagnostic utility of p63/p40 in the histologic differentiation of salivary gland tumors: A systematic review |journal=Oral Surg Oral Med Oral Pathol Oral Radiol |volume=133 |issue=2 |pages=189–198 |date=February 2022 |pmid=34518135 |doi=10.1016/j.oooo.2021.07.010 |url=}}</ref>
 
Others:
*GFAP +ve/-ve.
*GFAP +ve/-ve.
**One study suggests GFAP cleanly separates the PLGA (-ve) from [[pleomorphic adenoma]] (+ve).<ref name=pmid11174597>{{Cite journal  | last1 = Curran | first1 = AE. | last2 = White | first2 = DK. | last3 = Damm | first3 = DD. | last4 = Murrah | first4 = VA. | title = Polymorphous low-grade adenocarcinoma versus pleomorphic adenoma of minor salivary glands: resolution of a diagnostic dilemma by immunohistochemical analysis with glial fibrillary acidic protein. | journal = Oral Surg Oral Med Oral Pathol Oral Radiol Endod | volume = 91 | issue = 2 | pages = 194-9 | month = Feb | year = 2001 | doi = 10.1067/moe.2001.111306 | PMID = 11174597 }}</ref>
*BCL2 +ve/-ve.
*BCL2 +ve/-ve.
*Generally negative for myoepithelial markers (calponin, actin) - '''useful if negative'''.
*Generally negative for myoepithelial markers (calponin, actin) - '''useful if negative'''.
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==References==
==References==
{{Reflist|1}}
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Salivary gland]]
[[Category:Salivary gland]]

Latest revision as of 20:41, 2 June 2024

Polymorphous adenocarcinoma
Diagnosis in short

Polymorphous adenocarcinoma. H&E stain.
Synonyms polymorphmous low-grade adenocarcinoma (obsolete term)
LM low-grade cytology - nuclei ovoid & small, small nucleoli, powdery chromatin (papillary thyroid carcinoma-like appearance); eosinophilic cytoplasm; variable architecture - often small nests, classically has whorling ("eye-of-storm") pattern and single file pattern
LM DDx adenoid cystic carcinoma, pleomorphic adenoma, cribriform adenocarcinoma of the minor salivary gland
IHC S-100 +ve, CK7 +ve, p40 -ve, p63 +ve, vimentin +ve
Site oral cavity, salivary gland
Prevalence uncommon (varies by study)
Prognosis good

Polymorphous adenocarcinoma, abbreviated PAC, is a rare malignant salivary gland tumour.

It was previously known as polymorphous low-grade adenocarcinoma, abbreviated PLGA.[1]

General

  • Female:male ~= 2:1.
  • Older people ~50-70 years old.
  • Large variably in the prevalence reported - suggests a lacking of reproducibility in the diagnosis.[2]
  • Slow growing - metastases rare - prognosis good.[3][4]

Gross

Microscopic

Features:[6]

  • Architecture: often small nests, may be targetoid.
    • Classically has whorling with eye-of-storm & single file.
  • Cytologically monotonous (uniform) with variable architecture - key feature.
  • Indistinct cell borders.

Note:

  • Pretty much always a low-grade tumour.

DDx:

Images

  • PLGA - very low mag. (WC/Nephron)

  • PLGA - high mag. (WC/Nephron)

IHC

Features:[2]

  • S-100 +ve.
  • CK7 +ve.
  • Vimentin +ve.
  • p63 +ve (22 of 23 cases[7]).
  • p40 -ve (22 of 23 cases[7]).
    • Useful to differentiate from adenoid cystic carcinoma (AdCC); AdCC is usually p63 +ve/p40 +ve (36 of 47 cases) or p63 -ve/p40 -ve (10 of 47 cases).
      • p63 +ve/p40 +ve is seen in pleomorphic adenoma and mucoepidermoid carcinoma (in addition to adenoid cystic carcinoma).[8]

Others:

  • GFAP +ve/-ve.
  • BCL2 +ve/-ve.
  • Generally negative for myoepithelial markers (calponin, actin) - useful if negative.

See also

References

  1. ↑ 1.0 1.1 Vander Poorten V, Triantafyllou A, Skálová A, Stenman G, Bishop JA, Hauben E, Hunt JL, Hellquist H, Feys S, De Bree R, Mäkitie AA, Quer M, Strojan P, Guntinas-Lichius O, Rinaldo A, Ferlito A (July 2018). "Polymorphous adenocarcinoma of the salivary glands: reappraisal and update". Eur Arch Otorhinolaryngol 275 (7): 1681–1695. doi:10.1007/s00405-018-4985-5. PMID 29761209.
  2. ↑ 2.0 2.1 de Araujo, VC.; Passador-Santos, F.; Turssi, C.; Soares, AB.; de Araujo, NS. (2013). "Polymorphous low-grade adenocarcinoma: an analysis of epidemiological studies and hints for pathologists.". Diagn Pathol 8: 6. doi:10.1186/1746-1596-8-6. PMID 23320410.
  3. ↑ 3.0 3.1 Paleri, V.; Robinson, M.; Bradley, P. (Apr 2008). "Polymorphous low-grade adenocarcinoma of the head and neck.". Curr Opin Otolaryngol Head Neck Surg 16 (2): 163-9. doi:10.1097/MOO.0b013e3282f70441. PMID 18327037.
  4. ↑ Fife, TA.; Smith, B.; Sullivan, CA.; Browne, JD.; Waltonen, JD.. "Polymorphous low-grade adenocarcinoma: a 17 patient case series.". Am J Otolaryngol 34 (5): 445-8. doi:10.1016/j.amjoto.2013.03.012. PMID 23618791.
  5. ↑ Andreu-Barasoain, M.; Vicente-Martín, FJ.; Gómez de la Fuente, E.; Salamanca-Santamaría, J.; Pampín-Franco, A.; López-Estebaranz, JL. (2013). "Polymorphous low-grade adenocarcinoma in the upper lip: a well-described but infrequently recognized tumor.". Dermatol Online J 19 (8): 19265. PMID 24021444.
  6. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 74. ISBN 978-0781765275.
  7. ↑ 7.0 7.1 Atiq A, Mushtaq S, Hassan U, Loya A, Hussain M, Akhter N (October 2019). "Utility of p63 and p40 in Distinguishing Polymorphous Adenocarcinoma and Adenoid Cystic Carcinoma". Asian Pac J Cancer Prev 20 (10): 2917–2921. doi:10.31557/APJCP.2019.20.10.2917. PMC 6982655. PMID 31653135. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6982655/.
  8. ↑ Sivakumar N, Narwal A, Pandiar D, Devi A, Anand R, Bansal D, Kamboj M (February 2022). "Diagnostic utility of p63/p40 in the histologic differentiation of salivary gland tumors: A systematic review". Oral Surg Oral Med Oral Pathol Oral Radiol 133 (2): 189–198. doi:10.1016/j.oooo.2021.07.010. PMID 34518135.
  9. ↑ Curran, AE.; White, DK.; Damm, DD.; Murrah, VA. (Feb 2001). "Polymorphous low-grade adenocarcinoma versus pleomorphic adenoma of minor salivary glands: resolution of a diagnostic dilemma by immunohistochemical analysis with glial fibrillary acidic protein.". Oral Surg Oral Med Oral Pathol Oral Radiol Endod 91 (2): 194-9. doi:10.1067/moe.2001.111306. PMID 11174597.
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