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Difference between revisions of "Autosomal dominant polycystic kidney disease"
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Autosomal dominant polycystic kidney disease (view source)
Revision as of 19:01, 11 September 2016
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| Molecular = mutation in PKD1 gene or PKD2 gene | | Molecular = mutation in PKD1 gene or PKD2 gene | ||
| IF = | | IF = | ||
| Gross = enlarged kidney | | Gross = enlarged kidney with "too many cysts to count" | ||
| Grossing = | | Grossing = | ||
| Staging = | | Staging = | ||
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==General== | ==General== | ||
*Incidence of renal cell carcinoma (RCC) is increased in ADPKD in relation to the general population; however, the increase may to be due to the higher incidence of RCC in patients with (advanced) chronic kidney disease rather than ADPKD.<ref name=pmid19446940>{{Cite journal | last1 = Nishimura | first1 = H. | last2 = Ubara | first2 = Y. | last3 = Nakamura | first3 = M. | last4 = Nakanishi | first4 = S. | last5 = Sawa | first5 = N. | last6 = Hoshino | first6 = J. | last7 = Suwabe | first7 = T. | last8 = Takemoto | first8 = F. | last9 = Nakagawa | first9 = M. | title = Renal cell carcinoma in autosomal dominant polycystic kidney disease. | journal = Am J Kidney Dis | volume = 54 | issue = 1 | pages = 165-8 | month = Jul | year = 2009 | doi = 10.1053/j.ajkd.2009.01.270 | PMID = 19446940 }}</ref> | |||
**In a series of 510 patients evaluated clinically, 10 were found to have renal cell carcinoma. Eight of the ten were seen on imaging and two were detected by pathology.<ref name=pmid19446940>{{Cite journal | last1 = Nishimura | first1 = H. | last2 = Ubara | first2 = Y. | last3 = Nakamura | first3 = M. | last4 = Nakanishi | first4 = S. | last5 = Sawa | first5 = N. | last6 = Hoshino | first6 = J. | last7 = Suwabe | first7 = T. | last8 = Takemoto | first8 = F. | last9 = Nakagawa | first9 = M. | title = Renal cell carcinoma in autosomal dominant polycystic kidney disease. | journal = Am J Kidney Dis | volume = 54 | issue = 1 | pages = 165-8 | month = Jul | year = 2009 | doi = 10.1053/j.ajkd.2009.01.270 | PMID = 19446940 }}</ref> | |||
*In a series 301 renal surgeries on 240 ADPKD patients, 16 malignant lesions were seen.<ref name=pmid23752029>{{Cite journal | last1 = Jilg | first1 = CA. | last2 = Drendel | first2 = V. | last3 = Bacher | first3 = J. | last4 = Pisarski | first4 = P. | last5 = Neeff | first5 = H. | last6 = Drognitz | first6 = O. | last7 = Schwardt | first7 = M. | last8 = Gläsker | first8 = S. | last9 = Malinoc | first9 = A. | title = Autosomal dominant polycystic kidney disease: prevalence of renal neoplasias in surgical kidney specimens. | journal = Nephron Clin Pract | volume = 123 | issue = 1-2 | pages = 13-21 | month = | year = 2013 | doi = 10.1159/000351049 | PMID = 23752029 }}</ref> | |||
**10 [[papillary RCC]]s. | |||
**5 [[clear cell RCC]]s. | |||
**1 urothelial carcinoma. | |||
===Etiology=== | ===Etiology=== | ||
*Mutation in ''PKD1'' gene or ''PKD2'' gene. | *Mutation in ''PKD1'' gene or ''PKD2'' gene. | ||
| Line 90: | Line 97: | ||
**Morphologically similar to acquired renal cystic disease.<ref name=rj_oct2010>RJ. 20 October 2010.</ref> | **Morphologically similar to acquired renal cystic disease.<ref name=rj_oct2010>RJ. 20 October 2010.</ref> | ||
**It is said that "... if you can count 'em it's simple cysts; if you can't it's polycystic (renal disease)."<ref name=rj_oct2010>RJ. 20 October 2010.</ref><ref>{{cite book |author= Barbaric, Zoran L. |title=[http://books.google.com/books?id=uABgezxjhYwC&pg=PA87&lpg=PA87&dq=cystic+renal+disease,+%22if+you+can+count+them%22&source=bl&ots=GYyIdR9YmS&sig=DBYzT8LO2MltLA-wwW-0QwnQ6Lk&hl=en&ei=0hF-Taz0IsG3twe5yfG6BQ&sa=X&oi=book_result&ct=result&resnum=1&ved=0CBoQ6AEwAA#v=onepage&q=cystic%20renal%20disease%2C%20%22if%20you%20can%20count%20them%22&f=false Principles of Genitourinary Radiology] |publisher=Thieme |location= |year=1994 |pages= 87 |edition=2nd |isbn=978-0865774933 |oclc= |doi= |accessdate=}}</ref> | **It is said that "... if you can count 'em it's simple cysts; if you can't it's polycystic (renal disease)."<ref name=rj_oct2010>RJ. 20 October 2010.</ref><ref>{{cite book |author= Barbaric, Zoran L. |title=[http://books.google.com/books?id=uABgezxjhYwC&pg=PA87&lpg=PA87&dq=cystic+renal+disease,+%22if+you+can+count+them%22&source=bl&ots=GYyIdR9YmS&sig=DBYzT8LO2MltLA-wwW-0QwnQ6Lk&hl=en&ei=0hF-Taz0IsG3twe5yfG6BQ&sa=X&oi=book_result&ct=result&resnum=1&ved=0CBoQ6AEwAA#v=onepage&q=cystic%20renal%20disease%2C%20%22if%20you%20can%20count%20them%22&f=false Principles of Genitourinary Radiology] |publisher=Thieme |location= |year=1994 |pages= 87 |edition=2nd |isbn=978-0865774933 |oclc= |doi= |accessdate=}}</ref> | ||
===Images=== | |||
<gallery> | |||
Image: Polycystic kidney disease -- extremely low mag.jpg | PKD - extremely low mag. (WC) | |||
Image: Polycystic kidney disease -- very low mag.jpg | PKD - very low mag. (WC) | |||
Image: Polycystic kidney disease -- low mag.jpg | PKD - low mag. (WC) | |||
Image: Polycystic kidney disease -- intermed mag.jpg | PKD - intermed. mag. (WC) | |||
</gallery> | |||
==Sign out== | ==Sign out== | ||
<pre> | |||
Kidney, Right, Nephrectomy: | |||
- Polycystic kidney with chronic pyelonephritis and changes of chronic | |||
renal failure, consistent with polycystic kidney disease. | |||
- NEGATIVE for malignancy. | |||
</pre> | |||
===Alternate=== | |||
<pre> | <pre> | ||
Left Kidney, Nephrectomy: | Left Kidney, Nephrectomy: | ||
| Line 98: | Line 121: | ||
- NEGATIVE for malignancy. | - NEGATIVE for malignancy. | ||
</pre> | </pre> | ||
===Micro=== | |||
The sections show kidney with innumerable cysts and focal benign calcifications. Some atypical cysts with papillations are present. These have bland epithelial cells and do not have apparent proliferative activity. No mass lesion is present. No significant atypia is present. | |||
==See also== | ==See also== | ||