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'''Hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cell carcinoma''' is a malignant epithelial tumour of the [[kidney]] associated with the [[hereditary leiomyomatosis and renal cell carcinoma syndrome]]. | '''Hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cell carcinoma''' is a malignant epithelial tumour of the [[kidney]] associated with the [[hereditary leiomyomatosis and renal cell carcinoma syndrome]]. | ||
==Microscopic== | |||
Features - renal cell carcinoma:<ref name=pmid24441663/> | |||
*Large eosinophilic nucleolus with perinucleolar clearing - proposed hallmark - '''important'''. | |||
**May be focal. | |||
*Variable architecture: | |||
**Papillary - classic description. | |||
***Hyaline material within the fibrovascular cores - '''characteristic'''. | |||
**Tubulopapillary. | |||
**Tubular. | |||
**Solid. | |||
**[[Sieve-like pattern]]/[[cribriform]]. | |||
Notes: | |||
*Not common: [[psammoma bodies]], foamy macrophages.<ref name=pmid11248088>{{Cite journal | last1 = Launonen | first1 = V. | last2 = Vierimaa | first2 = O. | last3 = Kiuru | first3 = M. | last4 = Isola | first4 = J. | last5 = Roth | first5 = S. | last6 = Pukkala | first6 = E. | last7 = Sistonen | first7 = P. | last8 = Herva | first8 = R. | last9 = Aaltonen | first9 = LA. | title = Inherited susceptibility to uterine leiomyomas and renal cell cancer. | journal = Proc Natl Acad Sci U S A | volume = 98 | issue = 6 | pages = 3387-92 | month = Mar | year = 2001 | doi = 10.1073/pnas.051633798 | PMID = 11248088 }}</ref> | |||
DDx: | |||
*[[Papillary renal cell carcinoma]] (type 2). | |||
*[[Tubulocystic carcinoma of the kidney]]. | |||
*[[Collecting duct carcinoma]].<ref name=pmid16597677/> | |||
*[[Renal medullary carcinoma]] - cells also have a [[prominent nucleoli|prominent nucleolus]]. | |||
===Images=== | |||
====RCC==== | |||
<gallery> | |||
Image: Hereditary leiomyomatosis and renal cell carcinoma associated RCC -- low mag.jpg | HLRCC - low mag. (WC) | |||
Image: Hereditary leiomyomatosis and renal cell carcinoma associated RCC -- intermed mag.jpg | HLRCC - intermed mag. (WC) | |||
Image: Hereditary leiomyomatosis and renal cell carcinoma associated RCC -- high mag.jpg | HLRCC - high mag. (WC) | |||
Image: Hereditary leiomyomatosis and renal cell carcinoma associated RCC - alt -- high mag.jpg | HLRCC - high mag. (WC) | |||
Image: Hereditary leiomyomatosis and renal cell carcinoma associated RCC -- very high mag.jpg | HLRCC - very high mag. (WC) | |||
Image: Hereditary leiomyomatosis and renal cell carcinoma associated RCC - alt -- very high mag.jpg | HLRCC - very high mag. (WC) | |||
</gallery> | |||
<gallery> | |||
Image: Hereditary leiomyomatosis and renal cell carcinoma associated RCC - TC-like -- low mag.jpg | HLRCC - [[tubulocystic carcinoma of the kidney|TC-like]] - low mag. | |||
Image: Hereditary leiomyomatosis and renal cell carcinoma associated RCC - TC-like -- intermed mag.jpg | HLRCC - TC-like - intermed mag. | |||
Image: Hereditary leiomyomatosis and renal cell carcinoma associated RCC - TC-like -- high mag.jpg | HLRCC - TC-like - high mag. | |||
Image: Hereditary leiomyomatosis and renal cell carcinoma associated RCC - TC-like -- very high mag.jpg | HLRCC - TC-like - very high mag. | |||
</gallery> | |||
====www==== | |||
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC30663/figure/F3/ RCC in HLRCC (nih.gov)].<ref name=pmid11248088/> | |||
==See also== | ==See also== |
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