Cystic diseases of the

kidney

Presented By
Prof. Dr.
Nabil Tadros Mikhail
MBBS, MS Pathol., PhD Pathol.

Prof. of Pathology
Alexandria University - Egypt

Consultant & Chief Pathologist

King Fahad Central Hospital
Gizan - KSA
 Cystic diseases of the kidney
• Cystic diseases of the kidney are a
heterogeneous group inherited in most cases.

• They are important because:

1. They are common.
2. They can be confused with malignant
tumors.
3. Some forms as adult polycystic kidney
are the major cause of CRF.
1-Simple cysts :
• Occur single or multiple.
• Commonly they are 1-5 cm in diameter.
• It is translucent, lined by smooth
membrane and filled with clear fluid.
1-Simple cysts :
Microscopically

• The membrane is composed of single

layer of flattened or atrophic epithelium.
• The cyst is often confined to the cortex.
• It is usually asymptomatic ,or may
produce pain or hemorrhage.
1-Simple cysts :

• It can be differentiated from renal

tumor in three aspects:
1. Renal cyst has smooth contour.
2. Almost Avascular
3. It gives fluid rather than solid signal
on ultrasonography
Simple single renal cyst
Simple single renal cyst
Multiple simple renal cysts
 2-Dialysis associated cysts
• Cysts occur in patients with end stage
renal disease who have undergone
dialysis for long time.
• They are found in both renal cortex and
renal medulla. They may bleed.
• Occasionally adenoma or
adenocarcinoma arise in their walls .
 Cystic changes of chronic renal dialysis.
kidneys are normal in size.
Cysts are small in size and not exceed 2 cm in size.
 3-autosomal Dominant
Adult Polycystic Kidney Disease
(APKD)
• APKD is characterized by multiple
expanding cysts of both kidney that
destroy intervening parenchyma .
• It account for 10% of cases of chronic
renal failure.
• It is caused by inheritance of autosomal
dominant genes.
 3-autosomal Dominant
Adult Polycystic Kidney Disease
(APKD)

• In 90% of cases the defective gene is on

the short arm of chromosome 16.
• In the remaining 10% the defective gene
reside on chromosome 4.
 APKD ….
Morphology:
• The kidney may reach
large or huge size
• Become palpable abdominally.
 APKD ….
• Macroscopically:
• The kidney is composed of many
cysts of variable size up to 3-4 cm in
diameter with no intervening
parenchyma.
• The cysts are filled with fluid which
may be clear, turbid or hemorrhagic.
• at autopsy here to
reveal markedly
bilaterally enlarged
kidneys in the
retroperitoneum in an
adult who died from
complications of
chronic renal failure.
• This patient had
dominant polycystic
kidney disease
(DPKD).
 APKD;
the kidney is markedly
enlarged
and the cysts is large in size
APKD: bisected surface
(many large cysts are seen).
• This is a multicystic
dysplastic kidney.
• This condition must be
distinguished from RPKD
because it occurs only
sporadically and not with a
defined inheritance
pattern, though it is more
common than RPKD.
• The cysts are larger and
variably sized.
• Often, it is unilateral.
• If bilateral, it is often
asymmetric.
The microscopic appearance of multicystic dysplastic kidney
is characterized by large cysts lined by flattened cuboidal epithelium and
an intervening parenchyma that is fibrotic with islands of bluish cartilage
and rare glomeruli.
 APKD …

• Microscopic examination:
• It reveal some normal parenchyma
dispersed among cysts .
• Cysts may be found at any site of
nephron from tubules to collecting ducts
and has atrophic lining.
 APKD …
• Microscopic examination:…
• There is ischemic atrophy of intervening
renal tissue .
• Superimposed hypertension or infection is
common
 APKD cont..
• Clinical picture:
• APKD usually does not produce symptoms
until the fourth decade by which time the
kidney is large.
• The most common symptom is pain ,
which may be sever if hemorrhage occur.
• There is palpable abdominal mass .
• Intermittent hematuria is common.
 APKD cotin..

• Complication:
• The most important complications are
1. Hypertension
2. Urinary tract infection.
• These complications affect the renal
function
 APKD cotin..

• Complication:…
• End stage renal failure occur at about
age of 50 year.
• Death usually result from uremia or
hypertension .
• Renal transplantation is the only
hope .
 4-Autosomal recessive
(childhood) polycystic kidney

• This is a rare anomaly, that has

autosomal recessive inheritance.
• It results from mutation in gene at
chromosome 6.
• It is usually presented at birth or young
infants.
 4-Autosomal recessive
(childhood) polycystic kidney

• Kidney show numerous cysts in cortex

and medulla (sponge like
appearance).
• There is also multiple cysts in the liver
which may lead to congenital hepatic
fibrosis .
• It is fatal disorder.
• This child died soon after
premature birth at 23 weeks
gestation resulted from
markedly diminished fetal
urine output as a
consequence of polycystic
kidney disease.
• Note the bilaterally enlarged
kidneys that nearly fill the
abdomen below the liver.
• The histological appearance
in this case, coupled with the
gross appearance., was
consistent with recessive
polycystic kidney disease
(RPKD).
• Here is a cut section of a
kidney with recessive
polycystic kidney disease
(RPKD).
• Note that the cysts are
fairly small but uniformly
distributed throughout the
parenchyma so that the
disease is usually
symmetrical in
appearance,
• with both kidneys
markedly enlarged.
Microscopic examination of recessive polycystic kidney disease:
the cysts fill most parenchyma and is hard to find glomeruli. (sponge
like)