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Difference between revisions of "Polymorphous adenocarcinoma"
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{{ 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== | |||
*Female:male ~= 2:1. | |||
*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== | |||
Features:<ref name=Ref_WMSP74>{{Ref WMSP|74}} <!-- Mike S. --></ref> | |||
*Architecture: often small nests, may be targetoid. | |||
**Classically has whorling with '''eye-of-storm''' & '''single file'''. | |||
*Cytologically monotonous (uniform) with variable architecture - '''key feature'''. | |||
**Nucleus: ovoid & small, small nucleoli, powdery chromatin. | |||
***'''[[Papillary thyroid carcinoma]]-like appearance'''. | |||
**Cytoplasm: eosinophilic. | |||
*Indistinct cell borders. | |||
Note: | |||
*''Pretty much always'' a low-grade tumour. | |||
DDx: | |||
*[[Adenoid cystic carcinoma]]. | |||
*[[Pleomorphic adenoma]]. | |||
*[[Cribriform adenocarcinoma of the minor salivary gland]] - if one considers it a separate diagnosis. | |||
**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=== | |||
<gallery> | |||
Image:Polymorphous_low-grade_adenocarcinoma_-_very_low_mag.jpg | PLGA - very low mag. (WC/Nephron) | |||
Image:Polymorphous_low-grade_adenocarcinoma_high_mag.jpg | PLGA - high mag. (WC/Nephron) | |||
</gallery> | |||
==IHC== | |||
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 or 47 cases) or p63 -ve/p40 -ve (10 of 47 cases). | |||
Others: | |||
*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. | |||
*Generally negative for myoepithelial markers (calponin, actin) - '''useful if negative'''. | |||
==See also== | |||
*[[Salivary glands]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Diagnosis]] | |||
[[Category:Salivary gland]] | |||