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'''Carcinoma ex pleomorphic adenoma''', abbreviated ''ca ex PA'', is a rare malignant [[salivary gland]] tumour that | {{ Infobox diagnosis | ||
| Name = {{PAGENAME}} | |||
| Image = Carcinoma ex pleomorphic adenoma -- low mag.jpg | |||
| Width = | |||
| Caption = Carcinoma ex pleomorphic adenoma. [[H&E stain]]. | |||
| Micro = epithelial or myoepithelial cells with cytologic features of malignancy, pleomorphic adenoma (required if no history) | |||
| Subtypes = | |||
| LMDDx = [[salivary duct carcinoma]], [[mucoepidermoid carcinoma]], [[adenoid cystic carcinoma]], [[pleomorphic adenoma]] | |||
| Stains = | |||
| IHC = | |||
| EM = | |||
| Molecular = | |||
| IF = | |||
| Gross = firm | |||
| Grossing = | |||
| Site = [[salivary gland]] - usu. parotid gland | |||
| Assdx = | |||
| Syndromes = | |||
| Clinicalhx = pleomorphic adenoma (required if not present in assoc. with carcinoma) | |||
| Signs = usu. mass lesion, firm | |||
| Symptoms = | |||
| Prevalence = uncommon | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = dependent on stage, typically poor | |||
| Other = | |||
| ClinDDx = | |||
}} | |||
'''Carcinoma ex pleomorphic adenoma''', abbreviated '''ca ex PA''', is a rare malignant [[salivary gland]] tumour that arises from a [[pleomorphic adenoma]]. | |||
==General== | ==General== | ||
Definition: | Definition: | ||
* | *Malignancy arising from a ''[[pleomorphic adenoma]]''.<ref name=pmid21744105>{{Cite journal | last1 = Antony | first1 = J. | last2 = Gopalan | first2 = V. | last3 = Smith | first3 = RA. | last4 = Lam | first4 = AK. | title = Carcinoma ex pleomorphic adenoma: a comprehensive review of clinical, pathological and molecular data. | journal = Head Neck Pathol | volume = 6 | issue = 1 | pages = 1-9 | month = Mar | year = 2012 | doi = 10.1007/s12105-011-0281-z | PMID = 21744105 }}</ref> | ||
Diagnosis (either 1 or 2): | Diagnosis (either 1 or 2): | ||
#History of a pleomorphic adenoma at the same site. | #History of a pleomorphic adenoma at the same site (~25% of cases<ref name=pmid21744105/>). | ||
#Features of a pleomorphic adenoma and a carcinoma. | #Features of a pleomorphic adenoma and a carcinoma. | ||
Epidemiology: | Epidemiology: | ||
*Rare. | *Rare. | ||
*Prognosis dependent on stage: | |||
**Good for early lesions - like PA.<ref name=pmid23821206/> | |||
**Poor for advanced lesions.<ref name=pmid21744105/> | |||
==Gross== | |||
*Usually mass lesion - typically parotid gland.<ref name=pmid21744105/> | |||
==Microscopic== | ==Microscopic== | ||
Features: | Features: | ||
*Cells with cytologic features of malignancy. | *Cells (epithelial or myoepithelial) with cytologic features of malignancy - '''key feature'''. | ||
*Architecture (any of the following): | *Architecture (any of the following): | ||
**Glands. | **Glands. | ||
| Line 20: | Line 55: | ||
**Single cells (may be subtle). | **Single cells (may be subtle). | ||
Subtyping/patterns: | |||
*Ductal carcinoma NOS (arising from ductal cells) - most common pattern for | *Epithelial carcinoma (~75% of cases<ref name=pmid21744105/>): | ||
* | **[[Ductal carcinoma]] NOS (arising from ductal cells) - most common pattern for ca ex PA. | ||
*"Named carcinoma": | **"Named carcinoma": | ||
**Salivary duct carcinoma - second most common pattern for Ca ex PA. | ***[[Salivary duct carcinoma]] - second most common pattern for Ca ex PA. | ||
**Mucoepidermoid carcinoma. | ***[[Mucoepidermoid carcinoma]]. | ||
**Adenoid cystic carcinoma. | ***[[Adenoid cystic carcinoma]]. | ||
*Myoepithelial carcinoma NOS (arising from myoepithelial cells). | |||
Notes: | |||
*Myoepithelial cells may be clear cells. | *Myoepithelial cells may be clear cells. | ||
DDx: | |||
*[[Salivary duct carcinoma]]. | |||
*[[Mucoepidermoid carcinoma]]. | |||
*[[Adenoid cystic carcinoma]]. | |||
*[[Pleomorphic adenoma]]. | |||
===Subclassification=== | ===Subclassification=== | ||
| Line 37: | Line 78: | ||
#Minimally invasive <=1.5 mm beyond the capsule. | #Minimally invasive <=1.5 mm beyond the capsule. | ||
#Widely invasive >1.5 mm beyond the capsule. | #Widely invasive >1.5 mm beyond the capsule. | ||
===Images=== | |||
<gallery> | |||
Image: Carcinoma ex pleomorphic adenoma -- very low mag.jpg | Ca ex PA - very low mag. | |||
Image: Carcinoma ex pleomorphic adenoma -- low mag.jpg | Ca ex PA - low mag. | |||
Image: Carcinoma ex pleomorphic adenoma - a1 -- low mag.jpg | Ca ex PA - low mag. | |||
Image: Carcinoma ex pleomorphic adenoma - a2 -- low mag.jpg | Ca ex PA - low mag. | |||
Image: Carcinoma ex pleomorphic adenoma -- intermed mag.jpg | Ca ex PA - intermed. mag. | |||
Image: Carcinoma ex pleomorphic adenoma - a1 -- intermed mag.jpg | Ca ex PA - intermed. mag. | |||
Image: Carcinoma ex pleomorphic adenoma -- high mag.jpg | Ca ex PA - high mag. | |||
Image: Carcinoma ex pleomorphic adenoma -- very high mag.jpg | Ca ex PA - very high mag. | |||
</gallery> | |||
www: | |||
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3311945/figure/Fig1/ Ca ex PA - showing ca and PA areas (nih.gov)].<ref name=pmid21744105/> | |||
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3311945/figure/Fig2/ Ca ex PA - showing capsule invasion (nih.gov)]. | |||
==IHC== | |||
Features - ca ex PA versus PA:<ref name=pmid23821206>{{Cite journal | last1 = Di Palma | first1 = S. | title = Carcinoma ex pleomorphic adenoma, with particular emphasis on early lesions. | journal = Head Neck Pathol | volume = 7 Suppl 1 | issue = | pages = S68-76 | month = Jul | year = 2013 | doi = 10.1007/s12105-013-0454-z | PMID = 23821206 }}</ref> | |||
*Ki-67 >35% of cells. | |||
*HER2 +ve membrane staining (3+ for high-grade, 2+ or less for low-grade). | |||
*p53 +ve. | |||
*AR +ve. | |||
Others:<ref name=pmid23821206/> | |||
*CK7 +ve. | |||
*AE1/AE3 +ve. | |||
==Sign out== | |||
<pre> | |||
PAROTID GLAND (MASS), LEFT, EXCISION: | |||
- CARCINOMA EX PLEOMORPHIC ADENOMA, LOW-GRADE, INTRACAPSULAR. | |||
-- MARGINS NEGATIVE. | |||
-- SEE TUMOUR SUMMARY AND COMMENTS. | |||
- THREE BENIGN LYMPH NODES. | |||
</pre> | |||
===Micro=== | |||
The sections show a lesion with spindled (myoepithelial) cells and an epithelial component, | |||
on a background of a chondromyxoid stroma. The lesion is encapsulated by a thin layer of | |||
fibrous tissue. | |||
Focally, within the lesion, nuclear atypia and mitotic activity are identified, including an atypical mitotic figure. The atypical region stains with p53. Approximately 45% of the atypical cells stain with Ki-67. There is no appreciable HER2 staining. | |||
Unremarkable parotid gland and lymph nodes are present. | |||
==See also== | ==See also== | ||