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{{ Infobox diagnosis | {{ Infobox diagnosis | ||
| Name = {{PAGENAME}} | | Name = {{PAGENAME}} | ||
| Image = | | Image = Carcinoma ex pleomorphic adenoma -- low mag.jpg | ||
| Width = | | Width = | ||
| Caption = | | Caption = Carcinoma ex pleomorphic adenoma. [[H&E stain]]. | ||
| Micro = | | Micro = epithelial or myoepithelial cells with cytologic features of malignancy, pleomorphic adenoma (required if no history) | ||
| Subtypes = | | Subtypes = | ||
| LMDDx = [[salivary duct carcinoma]], [[mucoepidermoid carcinoma]], [[adenoid cystic carcinoma]], [[pleomorphic adenoma]] | | LMDDx = [[salivary duct carcinoma]], [[mucoepidermoid carcinoma]], [[adenoid cystic carcinoma]], [[pleomorphic adenoma]] | ||
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| Assdx = | | Assdx = | ||
| Syndromes = | | Syndromes = | ||
| Clinicalhx = | | Clinicalhx = pleomorphic adenoma (required if not present in assoc. with carcinoma) | ||
| Signs = usu. | | Signs = usu. mass lesion, firm | ||
| Symptoms = | | Symptoms = | ||
| Prevalence = uncommon | | Prevalence = uncommon | ||
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| Rads = | | Rads = | ||
| Endoscopy = | | Endoscopy = | ||
| Prognosis = typically poor | | Prognosis = dependent on stage, typically poor | ||
| Other = | | Other = | ||
| ClinDDx = | | ClinDDx = | ||
}} | }} | ||
'''Carcinoma ex pleomorphic adenoma''', abbreviated '''ca ex PA''', is a rare malignant [[salivary gland]] tumour that | '''Carcinoma ex pleomorphic adenoma''', abbreviated '''ca ex PA''', is a rare malignant [[salivary gland]] tumour that arises from a [[pleomorphic adenoma]]. | ||
==General== | ==General== | ||
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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== | ==Gross== | ||
*Usually mass lesion - | *Usually mass lesion - typically parotid gland.<ref name=pmid21744105/> | ||
==Microscopic== | ==Microscopic== | ||
Features: | Features: | ||
*Cells with cytologic features of malignancy - '''key feature'''. | *Cells (epithelial or myoepithelial) with cytologic features of malignancy - '''key feature'''. | ||
*Architecture (any of the following): | *Architecture (any of the following): | ||
**Glands. | **Glands. | ||
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**Single cells (may be subtle). | **Single cells (may be subtle). | ||
Subtyping/patterns: | |||
*[[Ductal carcinoma]] NOS (arising from ductal cells) - most common pattern for Ca ex PA. | *Epithelial carcinoma (~75% of cases<ref name=pmid21744105/>): | ||
**[[Ductal carcinoma]] NOS (arising from ductal cells) - most common pattern for ca ex PA. | |||
**"Named carcinoma": | |||
***[[Salivary duct carcinoma]] - second most common pattern for Ca ex PA. | |||
***[[Mucoepidermoid carcinoma]]. | |||
***[[Adenoid cystic carcinoma]]. | |||
*Myoepithelial carcinoma NOS (arising from myoepithelial cells). | *Myoepithelial carcinoma NOS (arising from myoepithelial cells). | ||
Notes: | |||
*Myoepithelial cells may be clear cells. | *Myoepithelial cells may be clear cells. | ||
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===Images=== | ===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/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)]. | *[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== |
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