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Mucoepidermoid carcinoma (view source)

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#redirect [[Salivary_glands#Mucoepidermoid_carcinoma]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Mucoepidermoid_carcinoma_-_2a_-_very_high_mag.jpg
| Width      =
| Caption    = Mucoepidermoid carcinoma. [[H&E stain]].
| Micro      = mucous cells (abundant fluffy cytoplasm and large mucin vacuoles - nucleus distorted by mucin vacuole, cells may be scarce); epidermoid cells (non-keratinized, polygonal squamoid cell with clear or oncocytic cytoplasm); architecture - cystic (low grade) or solid (high grade)
| Subtypes  =
| LMDDx      = [[squamous cell carcinoma of the head and neck]], [[adenosquamous carcinoma]], [[pleomorphic adenoma]]
| Stains    = mucous cells: [[alcian blue stain]] +ve, [[mucicarmine stain]] +ve
| IHC        =
| EM        =
| Molecular  = t(11;19)(q21;p13)
| IF        =
| Gross      = solid, cystic or both
| Grossing  =
| Site      = [[salivary gland]], classically parotid gland
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      = mass lesion
| Symptoms  =
| Prevalence = most common malignant salivary gland tumour, generally uncommon
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    =
}}
'''Mucoepidermoid carcinoma''', abbreviated '''MEC''', the is the most common malignant neoplasm of the [[salivary gland]].
 
==General==
*Most common malignant neoplasm of salivary gland in all age groups.<ref>URL: [http://path.upmc.edu/cases/case715/dx.html http://path.upmc.edu/cases/case715/dx.html]. Accessed on: 2 February 2012.</ref><ref name=pmid16003616>{{Cite journal  | last1 = Bell | first1 = RB. | last2 = Dierks | first2 = EJ. | last3 = Homer | first3 = L. | last4 = Potter | first4 = BE. | title = Management and outcome of patients with malignant salivary gland tumors. | journal = J Oral Maxillofac Surg | volume = 63 | issue = 7 | pages = 917-28 | month = Jul | year = 2005 | doi =  | PMID = 16003616 }}</ref>
*Female:male ~= 3:2.
*Site: parotid > submandibular.
 
==Gross==
*Cystic or solid, usually a mix of both.
 
Note:
*May mimic a benign cystic lesion grossly.
*Unicystic mucoepidermoid carcinoma is rare.<ref name=pmid22843640>{{cite journal |authors=Qannam A, Bello IO, Al-Kindi M, Al-Hindi M |title=Unicystic mucoepidermoid carcinoma presenting as a salivary duct cyst |journal=Int J Surg Pathol |volume=21 |issue=2 |pages=181–5 |date=April 2013 |pmid=22843640 |doi=10.1177/1066896912454918 |url=}}</ref>
 
==Microscopic==
Features:
*Architecture:<ref>URL: [http://moon.ouhsc.edu/kfung/jty1/opaq/PathQuiz/D2A001-PQ01-M.htm http://moon.ouhsc.edu/kfung/jty1/opaq/PathQuiz/D2A001-PQ01-M.htm]. Accessed on: 19 October 2010.</ref>
**Cystic (low grade).
**Solid (high grade).
*Mucous cells with abundant fluffy cytoplasm and large mucin vacuoles - '''key feature'''.
**Nucleus distorted by mucin vacuole.
**Mucous cell may be scarce - more difficult to diagnose.
*Epidermoid cells:
**Non-keratinized, polygonal squamoid cell with clear or oncocytic cytoplasm.
***Clear cells contain glycogen ([[PAS]] +ve, [[PAS-D]] -ve).
 
Notes:
*The classic description - composed of 3 cell types: epidermoid, intermediate, and mucin producing.<ref>{{Cite journal  | last1 = Lennerz | first1 = JK. | last2 = Perry | first2 = A. | last3 = Mills | first3 = JC. | last4 = Huettner | first4 = PC. | last5 = Pfeifer | first5 = JD. | title = Mucoepidermoid carcinoma of the cervix: another tumor with the t(11;19)-associated CRTC1-MAML2 gene fusion. | journal = Am J Surg Pathol | volume = 33 | issue = 6 | pages = 835-43 | month = Jun | year = 2009 | doi = 10.1097/PAS.0b013e318190cf5b | PMID = 19092631 }}</ref>
**"Intermediate cells" are described in textbooks. Weinreb thinks they are a pretty much a myth.<ref name=IW_10jan2011>IW. 10 January 2011.</ref>
*Mucin vacuoles may be rare; in a superficial glance -- it may mimic [[squamous cell carcinoma]].
*The thought of high-grade MEC should prompt consideration of squamous cell carcinoma.
 
DDx:<ref name=pmid22262946>{{Cite journal  | last1 = Mokhtari | first1 = S. | last2 = Mokhtari | first2 = S. | title = Clinical features and differential diagnoses in laryngeal mucoepidermoid carcinoma. | journal = Clin Med Insights Pathol | volume = 5 | issue =  | pages = 1-6 | month =  | year = 2012 | doi = 10.4137/CPath.S8435 | PMID = 22262946 }}</ref>
*[[Squamous cell carcinoma of the skin]] - 75% of metastases to the parotid region are from the skin.<ref name=pmid2694753>{{Cite journal  | last1 = Zanetti | first1 = D. | last2 = Renaldini | first2 = G. | last3 = Peretti | first3 = G. | last4 = Antonelli | first4 = AR. | title = [Intra-parotid lymph node metastasis of malignant skin neoplasms of the head]. | journal = Acta Otorhinolaryngol Ital | volume = 9 | issue = 4 | pages = 381-90 | month =  | year =  | doi =  | PMID = 2694753 }}</ref>
*[[Squamous cell carcinoma of the head and neck]].
*[[Adenosquamous carcinoma]].
*[[Pleomorphic adenoma]].<ref name=pmid15754364>{{Cite journal  | last1 = Siddiqui | first1 = NH. | last2 = Wu | first2 = SJ. | title = Fine-needle aspiration biopsy of cystic pleomorphic adenoma with adnexa-like differentiation mimicking mucoepidermoid carcinoma: a case report. | journal = Diagn Cytopathol | volume = 32 | issue = 4 | pages = 229-32 | month = Apr | year = 2005 | doi = 10.1002/dc.20215 | PMID = 15754364 }}</ref>
 
===Images===
<gallery>
Image:Mucoepidermoid_carcinoma_%282%29_HE_stain.jpg | Mucoepidermoid carcinoma 2. (WC)
Image:Mucoepidermoid_carcinoma_%283%29_HE_stain.jpg | Mucoepidermoid carcinoma 3. (WC)
Image:Mucoepidermoid_carcinoma_-_2_-_intermed_mag.jpg | Mucoepidermoid carcinoma - 2 - intermed. mag. (WC/Nephron)
Image:Mucoepidermoid_carcinoma_-_2_-_high_mag.jpg | Mucoepidermoid carcinoma - 2 - high mag. (WC/Nephron)
Image:Mucoepidermoid_carcinoma_-_2a_-_very_high_mag.jpg | Mucoepidermoid carcinoma - 2 - very high mag. (WC/Nephron)
</gallery>
www:
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3256999/figure/f2-cpath-5-2012-001/ Low-grade MEC (nih.gov)].<ref name=pmid22262946>{{Cite journal  | last1 = Mokhtari | first1 = S. | last2 = Mokhtari | first2 = S. | title = Clinical features and differential diagnoses in laryngeal mucoepidermoid carcinoma. | journal = Clin Med Insights Pathol | volume = 5 | issue =  | pages = 1-6 | month =  | year = 2012 | doi = 10.4137/CPath.S8435 | PMID = 22262946 }}</ref>
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3256999/figure/f3-cpath-5-2012-001/ Intermed. grade MEC (nih.gov)].
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3256999/figure/f4-cpath-5-2012-001/ High-grade MEC (nih.gov)].
 
[[File: 5_20985699291686_sl 1.png|Mucoepidermoid carcinoma of parotid, low grade]]
[[File: 5_20985699291686_sl 2.png|Mucoepidermoid carcinoma of parotid, low grade]]
[[File: 5_20985699291686_sl 3.png|Mucoepidermoid carcinoma of parotid, low grade]]
[[File: 5_20985699291686_sl 4.png|Mucoepidermoid carcinoma of parotid, low grade]]
 
Low grade mucoepidermoid carcinoma in left parotid of 51 year old woman. A. Tumor shows blue squamoud differentiation with cystic dilatation. B This focus shows more obvious cyst formation. C. Squamous component with round to spindled cancer cells; note that currently squamous pearls and high grade squamous cancer elements render the tumor a squamous carcinoma, according to Barnes. D. Glandular cell component.
 
===Subtypes===
*Conventional.
*Oncocytic.
**Definition: composed of 50% oncocytes.
**Good outcome.<ref name=pmid18971778>{{cite journal |author=Weinreb I, Seethala RR, Perez-Ordoñez B, Chetty R, Hoschar AP, Hunt JL |title=Oncocytic mucoepidermoid carcinoma: clinicopathologic description in a series of 12 cases |journal=Am. J. Surg. Pathol. |volume=33 |issue=3 |pages=409–16 |year=2009 |month=March |pmid=18971778 |doi=10.1097/PAS.0b013e318184b36d |url=}}</ref>
*Clear cell.
*Unicystic (cystadenocarcinoma).
**Based on the gross. (???)
*Sclerosing MEC +/- eosinophilia.
**Rare.
 
===Grading===
General:
*Two competing system exist:
**AFIP.<ref name=pmid9529011>{{cite journal |author=Goode RK, Auclair PL, Ellis GL |title=Mucoepidermoid carcinoma of the major salivary glands: clinical and histopathologic analysis of 234 cases with evaluation of grading criteria |journal=Cancer |volume=82 |issue=7 |pages=1217–24 |year=1998 |month=April |pmid=9529011 |doi= |url=}}</ref>
**Brandwein.<ref name=pmid11420454>{{cite journal |author=Brandwein MS, Ivanov K, Wallace DI, ''et al.'' |title=Mucoepidermoid carcinoma: a clinicopathologic study of 80 patients with special reference to histological grading |journal=Am. J. Surg. Pathol. |volume=25 |issue=7 |pages=835–45 |year=2001 |month=July |pmid=11420454 |doi= |url=}}</ref>
 
Notes:
*Both systems have their pros and cons.
*Weinreb uses the AFIP system with a slight modification.
 
====AFIP====
#Low cystic component (<20%) - 2 points.
#[[Perineural invasion]] - 2 points.
#[[Necrosis]] - 3 points.
#Mitoses > 4 per 10 HPFs (HPF not defined in paper - see [[HPFitis]]) - 3 points.
#Anaplasia - 4 points.
 
Scoring:
*Low grade = 0-4 points.
*Intermediate grade = 5-6 points.
*High grade = 7+ points.
 
=====Weinreb modification=====
Weinreb looks for the following:
*Tumour invades in small nests/islands - 2 points.
**If applicable, the two points are added to the AFIP score.
**The tumour is graded using the AFIP (scoring) cut points -- see above.
 
Notes:
*It seems pointless to memorize this but it is occasionally asked on exams.
**How to remember: think of the Nottingham grading system (architecture, mitoses, nuclear grade) + necrosis + LVI.
 
==Stains==
Mucous cells:
*Alcian blue +ve.
*Mucicarmine +ve.
 
Notes:
*Above useful for MEC versus [[squamous cell carcinoma]].<ref name=pmid23960993>{{Cite journal  | last1 = Jastrzebski | first1 = A. | last2 = Brownstein | first2 = S. | last3 = Jordan | first3 = DR. | last4 = Gilberg | first4 = SM. | title = Histochemical analysis and immunohistochemical profile of mucoepidermoid carcinoma of the conjunctiva. | journal = Saudi J Ophthalmol | volume = 26 | issue = 2 | pages = 205-10 | month = Apr | year = 2012 | doi = 10.1016/j.sjopt.2012.01.004 | PMID = 23960993 }}</ref>
 
==IHC==
*CK7 +ve.
*CK20 -ve.
*CEA +ve.<ref>{{Cite journal  | last1 = Hassanin | first1 = MB. | last2 = Ghosh | first2 = L. | last3 = Das | first3 = AK. | last4 = Waterhouse | first4 = JP. | title = Immunohistochemical and fluorescent microscopic study of histogenesis of salivary mucoepidermoid carcinoma. | journal = J Oral Pathol Med | volume = 18 | issue = 5 | pages = 291-8 | month = May | year = 1989 | doi =  | PMID = 2475619 }}</ref>
 
==Sign out==
<pre>
A. Lymph Node, Deep Lobe of Left Parotid Gland, Excision:
    - One lymph node NEGATIVE for malignancy (0/1).
 
B. Parotid Gland - Left Deep Lobe, Excision:
    - MUCOEPIDERMOID CARCINOMA, low (AFIP) grade.
    -- Margins clear.
    -- Please see synoptic report.   
</pre>
 
==Molecular==
*t(11;19)(q21;p13) -- MECT1-MAML2 fusion.<ref name=pmid12539049>{{cite journal |author=Tonon G, Modi S, Wu L, ''et al.'' |title=t(11;19)(q21;p13) translocation in mucoepidermoid carcinoma creates a novel fusion product that disrupts a Notch signaling pathway |journal=Nat. Genet. |volume=33 |issue=2 |pages=208–13 |year=2003 |month=February |pmid=12539049 |doi=10.1038/ng1083 |url=}}</ref><ref name=pmid20588178>{{cite journal |author=Seethala RR, Dacic S, Cieply K, Kelly LM, Nikiforova MN |title=A reappraisal of the MECT1/MAML2 translocation in salivary mucoepidermoid carcinomas |journal=Am. J. Surg. Pathol. |volume=34 |issue=8 |pages=1106–21 |year=2010 |month=August |pmid=20588178 |doi=10.1097/PAS.0b013e3181de3021 |url=}}</ref>
**Present in ~65% of MECs.
**Presence assoc. with low-grade MEC (vs. high-grade MEC) & favourable prognosis.
**Not seen in tumours that are in the DDx of MEC.
 
==See also==
*[[Salivary glands]].
*[[Squamous cell carcinoma]].
*[[Sclerosing mucoepidermoid carcinoma with eosinophilia]].
 
==References==
{{Reflist|2}}


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