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Cystic Renal Disease
Jud Gash, MD
Cystic Renal Disease


Overview











Simple Cyst
 complicated
 hyperdense
 Bosniak classification
Cysts and cancer
 ACKD, TS & VHL
Multicystic dysplastic kidneys
Autosomal Dominant and Recessive Polycystic
Medullary Sponge Kidney
Medullary Cystic Disease
Localized Cystic Renal Disease
Renal Sinus Cysts
Simple Cyst
Most common adult renal mass (50% > 50yo)
 Cortical; straw colored fluid
 usually asymptomatic
 BIB them

Simple Cyst


IVP (historical)
homogeneous, lucent
 thin walls, smooth
interface
 “beak sign” - implies
indolent process
 Cannot dx on IVP; must
confirm (US or CT)

Simple Cyst


Ultrasound




anechoic
thin, smooth wall
increased thru transmission
Simple Cyst


CT and MRI


near water density (< 10 HU)









Check HU’s of other water

thin, smooth wall
homogeneous
No enhancement
MRI>CT
Veritas tip – test yourself
before ROI
Pitfall – beware of Papillary
RCC
Simple Cyst


Too small to characterize (subcentimeter) renal
hypodensity


2 points:
OK to say very likely cyst and BIB it
 Although cant get water HU’s




A 5 mm cyst is more hypodense to your eye than 5 mm solid
lesion
Cyst-makers vs Stranders
Complicated Renal Cyst




Simple cysts may become infected or
hemorrhage/trauma
US/CT/MRI




Thick walls; calcifications, septations, debris or increased
attenuation
And so, overlaps the appearance of cystic RCC
 And therefore Bosniak Classification
56 y/o with renal mass

Hemorrhagic cyst
at surgery
74 with flank pain
Hyperdense Cyst



Benign cyst containing blood products (clotted, old not just acute)
50 -90 HU (hyperdense to water or kidney)







if no NCCT, can be isodense or hypodense to kidney on CCT
50% appear cystic( a few echoes), but may not

US
CT Criteria





< 3cm
smooth wall (must extend beyond margin to evaluate)
rounded; sharp margins
**** NO SIGNIFICANT ENHANCEMENT ****
Hyperdense Cyst
Hyperdense Cyst
Hyperdense Cyst


Must distinguish from RCC
if strict CT criteria or US, then
some stop (II) or some follow
(IIF)
 if comes very close (intrarenal,
3.5 cm, elderly) could follow
(IIF)
 if doesnt meet criteria, “cannot
RO RCC”




New Data helpful Too


>70HU noncontrast
Bosniak Classification





Most problematic renal masses are cystic
Bosniak created a classification scheme to guide
proper management
Classification
I - simple cyst
 II - minimally complicated (1 or 2 thin,
“nonenhancing”septi, delicate Ca++ in septi or wall;
hyperdense)
 III - thick walls or Ca++, thick septi, nodules
 IV - definitely malignant (necrotic masses, etc.)

Bosniak I
Bosniak II
Bosniak II-F
Bosniak III
Bosniak IV
Bosniak Classification


Management
I - stop
 II - stop or follow Up (IIf)
 III & IV - surgery






Although most III will be benign, 30% will be cancer

Problems
Category II lesions (at least one study showed 4/5
malignant)
 Interobserver variability between II and III

Cysts and Cancer


3 main areas
Acquired cystic kidney disease (ACKD)
 Tuberous Sclerosis (TS)
 Von-Hipple Lindau (VHL)

Acquired Cystic Renal Disease




Development of cysts and occasional neoplasms in
patients with CRF
Can occur without dialysis (10%) but very common in
patients with dialysis (either hemo or peritoneal)





? prolongs lifespan so cysts develop
50% at 5 yrs, 90% at 10 years

Impact of transplant




controversial
cysts - some regress, some dont
neoplasms - again controversial
Acquired Cystic Renal Disease


Findings


Cysts






Neoplasms






small early; enlarge
and multiply in time;
can mimic ADPCKD
commonly Ca++,
hemorrhage
definite increased risk
with CRF, again esp.
dialysis
often indolent in
nature (papillary RCC)

kidneys small;
atrophic; hyperechoic;
no other cysts
Tuberous Sclerosis


Renal manifestations
occur in majority


Cysts – 15-50%





AML’s – 80%
Neoplasm (RCC)





When occur tend to be in
young children

Controversial whether
increased incidence
predisposition suggested by
younger age and bilateral
nature

No extrarenal cysts
Renal cysts in a 10-month-old with TS.
Von Hippel-Lindau




Renal manifestations
also common
Findings


Cysts – 50-75%




RCC - 35%





BL; Mulitple
bl and multiple
Aggressive behavior

pancreatic cysts
Multicystic Dysplastic Kidney





Congenital, non-hereditary dysplasia
characterized by renal parenchyma replaced by
multiple cysts
due to dysplastic ureteral bud
Clinical
May present as abdominal mass in child or incidental in
adult
 ** 10-30% incidence of contralateral anomaly, esp UPJ
and reflux**

Multicystic Dysplastic Kidney


Types
pelvoinfundibular atresia
(most common) - atretic
renal pelvis and ureter;
cystic renal mass
 Hydronephrotic type
(rare) - ureteral atresia
but dilated pelvis
 segmental (rare) - in
duplicated system

Multicystic Dysplastic Kidney


Findings
No function
 multiple noncommunicating cysts replacing kidney
 Usually no large central cystic area (UPJ)
 large in child
 Small to large, often Ca++ in adult (fails to grow)




MUST evaluate contra-lateral side
Multicystic Dysplastic Kidney

Mercado-Deane M et al. Radiographics 2002;22:1429-1438
Multicystic Dysplastic Kidney
23 week fetus with MCDK

Huppert B J et al. Radiographics 1999;19:S215-S227
Autosomal Dominant Polycystic
Kidney Disease


Clinically
May present prenatally to elderly
 Classically presents with CRF in 30-40 yo
 Problems








Renal Failure - often, but not always, progressive
HTN, UTI’s and pain
SAH

Autosomal Dominant condition
Autosomal Dominant Polycystic
Kidney Disease


Manifestation







Renal Cysts
 start small but enlarge over time
 enlarge the kidney
 often complicated with Ca++, hemorrhage
 usually bl, symetric involvement
Extrarenal cysts
 liver (40-90%)
 pancreas (10%)
 Spleen (5%)
Cerebral aneurysms (15-40%)
No increased risk of RCC
Autosomal Dominant Polycystic
Kidney Disease


IVP
Bilateral enlarged lobulated kidneys
 swiss cheese nephrogram (multiple lucencies on
nephrogram)
 arachnoid calyces (elongated, stretched calyces)




US & CT/MRI


bilateral large kidneys with cysts




often with Ca++, hyperdense

Extrarenal cysts
Autosomal Dominant Polycystic
Kidney Disease
Autosomal Dominant Polycystic
Kidney Disease
Autosomal Recessive Polycystic
Kidney Disease


Genetic condition two primary abnormalities
Kidneys - cystic dilatation (1-8mm) of the collecting ducts
-> renal failure
 Biliary - biliary ectasia/periportal fibrosis -> portal
hypertension; liver failure

Autosomal Recessive Polycystic
Kidney Disease


Clinically
Classically classified as perinatal, neonatal, infantile and
juvenile; now felt to be too rigid
 Spectrum of expression with predominately renal and
minimal hepatic disease when presentation in perinatal
period; and predominately hepatic and milder renal
disease when presenting in the older child
 An individual can present anywhere along the spectrum

Autosomal Recessive Polycystic
Kidney Disease


Imaging


Perinatal form (most common)









massive nephromegaly usually
without discrete cysts dominates
the imaging
Prenatal US - enlarged
hyperechoic kidneys; decreased
or absent bladder;
oligohydramnios
PF - flank masses; pulm.
hypoplasia with ptx
US - enlarged echogenic kidneys
with loss of CMJ
CT - nehpromegaly; striated
prolonged nephrogram
Liver US - usually normal or
slightly hyperechoic
Autosomal Recessive Polycystic
Kidney Disease
Autosomal Recessive Polycystic
Kidney Disease
Autosomal Recessive Polycystic
Kidney Disease


Imaging


Older child




The kidneys appear
normal or similar to
perinatal findings, only
milder; occ. discrete cysts
Liver US - hyperechoic;
may see cystic dilatation
of the biliary tree; changes
of portal HTN (HSM;
varices)
Medullary Sponge Kidney


Pathology




saccular 1-8mm dilatation of
the distal collecting ducts
with sluggish flow and
predisposition to stone
formation

Clinical





Young adults
usually asymptomatic except
stones
non-hereditary; nonprogressive
assoc. with Caroli’s,
ARPCKD; hemihypertrophy
Medullary Sponge Kidney


Imaging (PF, US and CT)


Medullary nephrocalcinosis



bilateral, unilateral or focal
“growing calculus sign”

Urolithiasis
 Discrete linear collections in papilla on IVP




should be distinguished papillary blush - a nondiscrete blush
of contrast within the papillary which can be normal
Medullary Sponge Kidney
Medullary Sponge Kidney
Medullary Sponge Kidney
Medullary Sponge Kidney
Medullary Cystic Disease


Clinicopathologic







Rare inherited disease
Chronic failure in early
adulthood
Medullary cysts
two types

Imaging






Medullar cysts
small, smooth kidneys with
poor function
Occasionally see dilated,
contrast filled tubules in
medulla (mimics MSK)
No ca++
Localized Cystic Disease






Multiple
conglomerated
cysts with
intervening
normal (or
compressed) renal
tissue
Uncommon and
benign
Unilateral (other
kidney normal)
Part or all of
kidney

AJR 2001; 176:843-849
Localized Cystic Disease


DDX



Abscess
MCDK




MLCN and CysticRCC




Kidney functions
normally
Normal interneving tissue
and lack of capsule

Should Follow


Can Grow

Multilocular cystic RCC – capsule
with enhancing tumorous septa

AJR 2001; 176:843-849
Renal Sinus Cysts


2 types


parapelvic cyst




peripelvic cyst




simple parenchymal cyst which projects into the sinus
extraparenchymal cyst arising in sinus (?origin; ?
congenital and lymphatic etiology)

Imaging (peripelvic)
May be multiple and bilateral, unilocular or
multilocular; usually small and insinuating
 smooth splaying of collecting system
 rarely results in obstruction and hydronephrosis

Renal Sinus Cyst
(parapelvic)
Renal Sinus Cysts


Imaging


US








cyst in renal
sinus
sinus cyst
(delays needed
to exclude
hydro)

CT

DDX hydronephrosis
Renal Sinus Cysts
Renal Sinus Cysts
Cystic Renal Disease


Review











Simple Cyst
 complicated
 hyperdense
 Bosniak classification
Cysts and cancer
 ACKD, TS & VHL
Multicystic dysplastic kidneys
Autosomal Dominant and Recessive Polycystic
Medullary Sponge Kidney
Medullary Cystic Disease
Localized Cystic Disease
Renal Sinus Cysts

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Cystic Renal Disease

  • 2. Cystic Renal Disease  Overview         Simple Cyst  complicated  hyperdense  Bosniak classification Cysts and cancer  ACKD, TS & VHL Multicystic dysplastic kidneys Autosomal Dominant and Recessive Polycystic Medullary Sponge Kidney Medullary Cystic Disease Localized Cystic Renal Disease Renal Sinus Cysts
  • 3. Simple Cyst Most common adult renal mass (50% > 50yo)  Cortical; straw colored fluid  usually asymptomatic  BIB them 
  • 4. Simple Cyst  IVP (historical) homogeneous, lucent  thin walls, smooth interface  “beak sign” - implies indolent process  Cannot dx on IVP; must confirm (US or CT) 
  • 6. Simple Cyst  CT and MRI  near water density (< 10 HU)        Check HU’s of other water thin, smooth wall homogeneous No enhancement MRI>CT Veritas tip – test yourself before ROI Pitfall – beware of Papillary RCC
  • 7. Simple Cyst  Too small to characterize (subcentimeter) renal hypodensity  2 points: OK to say very likely cyst and BIB it  Although cant get water HU’s   A 5 mm cyst is more hypodense to your eye than 5 mm solid lesion
  • 9. Complicated Renal Cyst   Simple cysts may become infected or hemorrhage/trauma US/CT/MRI   Thick walls; calcifications, septations, debris or increased attenuation And so, overlaps the appearance of cystic RCC  And therefore Bosniak Classification
  • 10. 56 y/o with renal mass Hemorrhagic cyst at surgery
  • 12. Hyperdense Cyst   Benign cyst containing blood products (clotted, old not just acute) 50 -90 HU (hyperdense to water or kidney)     if no NCCT, can be isodense or hypodense to kidney on CCT 50% appear cystic( a few echoes), but may not US CT Criteria     < 3cm smooth wall (must extend beyond margin to evaluate) rounded; sharp margins **** NO SIGNIFICANT ENHANCEMENT ****
  • 15. Hyperdense Cyst  Must distinguish from RCC if strict CT criteria or US, then some stop (II) or some follow (IIF)  if comes very close (intrarenal, 3.5 cm, elderly) could follow (IIF)  if doesnt meet criteria, “cannot RO RCC”   New Data helpful Too  >70HU noncontrast
  • 16. Bosniak Classification    Most problematic renal masses are cystic Bosniak created a classification scheme to guide proper management Classification I - simple cyst  II - minimally complicated (1 or 2 thin, “nonenhancing”septi, delicate Ca++ in septi or wall; hyperdense)  III - thick walls or Ca++, thick septi, nodules  IV - definitely malignant (necrotic masses, etc.) 
  • 22. Bosniak Classification  Management I - stop  II - stop or follow Up (IIf)  III & IV - surgery    Although most III will be benign, 30% will be cancer Problems Category II lesions (at least one study showed 4/5 malignant)  Interobserver variability between II and III 
  • 23. Cysts and Cancer  3 main areas Acquired cystic kidney disease (ACKD)  Tuberous Sclerosis (TS)  Von-Hipple Lindau (VHL) 
  • 24. Acquired Cystic Renal Disease   Development of cysts and occasional neoplasms in patients with CRF Can occur without dialysis (10%) but very common in patients with dialysis (either hemo or peritoneal)    ? prolongs lifespan so cysts develop 50% at 5 yrs, 90% at 10 years Impact of transplant    controversial cysts - some regress, some dont neoplasms - again controversial
  • 25. Acquired Cystic Renal Disease  Findings  Cysts    Neoplasms    small early; enlarge and multiply in time; can mimic ADPCKD commonly Ca++, hemorrhage definite increased risk with CRF, again esp. dialysis often indolent in nature (papillary RCC) kidneys small; atrophic; hyperechoic; no other cysts
  • 26. Tuberous Sclerosis  Renal manifestations occur in majority  Cysts – 15-50%    AML’s – 80% Neoplasm (RCC)    When occur tend to be in young children Controversial whether increased incidence predisposition suggested by younger age and bilateral nature No extrarenal cysts Renal cysts in a 10-month-old with TS.
  • 27. Von Hippel-Lindau   Renal manifestations also common Findings  Cysts – 50-75%   RCC - 35%    BL; Mulitple bl and multiple Aggressive behavior pancreatic cysts
  • 28. Multicystic Dysplastic Kidney    Congenital, non-hereditary dysplasia characterized by renal parenchyma replaced by multiple cysts due to dysplastic ureteral bud Clinical May present as abdominal mass in child or incidental in adult  ** 10-30% incidence of contralateral anomaly, esp UPJ and reflux** 
  • 29. Multicystic Dysplastic Kidney  Types pelvoinfundibular atresia (most common) - atretic renal pelvis and ureter; cystic renal mass  Hydronephrotic type (rare) - ureteral atresia but dilated pelvis  segmental (rare) - in duplicated system 
  • 30. Multicystic Dysplastic Kidney  Findings No function  multiple noncommunicating cysts replacing kidney  Usually no large central cystic area (UPJ)  large in child  Small to large, often Ca++ in adult (fails to grow)   MUST evaluate contra-lateral side
  • 31. Multicystic Dysplastic Kidney Mercado-Deane M et al. Radiographics 2002;22:1429-1438
  • 33. 23 week fetus with MCDK Huppert B J et al. Radiographics 1999;19:S215-S227
  • 34. Autosomal Dominant Polycystic Kidney Disease  Clinically May present prenatally to elderly  Classically presents with CRF in 30-40 yo  Problems      Renal Failure - often, but not always, progressive HTN, UTI’s and pain SAH Autosomal Dominant condition
  • 35. Autosomal Dominant Polycystic Kidney Disease  Manifestation     Renal Cysts  start small but enlarge over time  enlarge the kidney  often complicated with Ca++, hemorrhage  usually bl, symetric involvement Extrarenal cysts  liver (40-90%)  pancreas (10%)  Spleen (5%) Cerebral aneurysms (15-40%) No increased risk of RCC
  • 36. Autosomal Dominant Polycystic Kidney Disease  IVP Bilateral enlarged lobulated kidneys  swiss cheese nephrogram (multiple lucencies on nephrogram)  arachnoid calyces (elongated, stretched calyces)   US & CT/MRI  bilateral large kidneys with cysts   often with Ca++, hyperdense Extrarenal cysts
  • 39. Autosomal Recessive Polycystic Kidney Disease  Genetic condition two primary abnormalities Kidneys - cystic dilatation (1-8mm) of the collecting ducts -> renal failure  Biliary - biliary ectasia/periportal fibrosis -> portal hypertension; liver failure 
  • 40. Autosomal Recessive Polycystic Kidney Disease  Clinically Classically classified as perinatal, neonatal, infantile and juvenile; now felt to be too rigid  Spectrum of expression with predominately renal and minimal hepatic disease when presentation in perinatal period; and predominately hepatic and milder renal disease when presenting in the older child  An individual can present anywhere along the spectrum 
  • 41. Autosomal Recessive Polycystic Kidney Disease  Imaging  Perinatal form (most common)       massive nephromegaly usually without discrete cysts dominates the imaging Prenatal US - enlarged hyperechoic kidneys; decreased or absent bladder; oligohydramnios PF - flank masses; pulm. hypoplasia with ptx US - enlarged echogenic kidneys with loss of CMJ CT - nehpromegaly; striated prolonged nephrogram Liver US - usually normal or slightly hyperechoic
  • 44. Autosomal Recessive Polycystic Kidney Disease  Imaging  Older child   The kidneys appear normal or similar to perinatal findings, only milder; occ. discrete cysts Liver US - hyperechoic; may see cystic dilatation of the biliary tree; changes of portal HTN (HSM; varices)
  • 45. Medullary Sponge Kidney  Pathology   saccular 1-8mm dilatation of the distal collecting ducts with sluggish flow and predisposition to stone formation Clinical     Young adults usually asymptomatic except stones non-hereditary; nonprogressive assoc. with Caroli’s, ARPCKD; hemihypertrophy
  • 46. Medullary Sponge Kidney  Imaging (PF, US and CT)  Medullary nephrocalcinosis   bilateral, unilateral or focal “growing calculus sign” Urolithiasis  Discrete linear collections in papilla on IVP   should be distinguished papillary blush - a nondiscrete blush of contrast within the papillary which can be normal
  • 51. Medullary Cystic Disease  Clinicopathologic      Rare inherited disease Chronic failure in early adulthood Medullary cysts two types Imaging     Medullar cysts small, smooth kidneys with poor function Occasionally see dilated, contrast filled tubules in medulla (mimics MSK) No ca++
  • 52. Localized Cystic Disease     Multiple conglomerated cysts with intervening normal (or compressed) renal tissue Uncommon and benign Unilateral (other kidney normal) Part or all of kidney AJR 2001; 176:843-849
  • 53. Localized Cystic Disease  DDX   Abscess MCDK   MLCN and CysticRCC   Kidney functions normally Normal interneving tissue and lack of capsule Should Follow  Can Grow Multilocular cystic RCC – capsule with enhancing tumorous septa AJR 2001; 176:843-849
  • 54. Renal Sinus Cysts  2 types  parapelvic cyst   peripelvic cyst   simple parenchymal cyst which projects into the sinus extraparenchymal cyst arising in sinus (?origin; ? congenital and lymphatic etiology) Imaging (peripelvic) May be multiple and bilateral, unilocular or multilocular; usually small and insinuating  smooth splaying of collecting system  rarely results in obstruction and hydronephrosis 
  • 56. Renal Sinus Cysts  Imaging  US     cyst in renal sinus sinus cyst (delays needed to exclude hydro) CT DDX hydronephrosis
  • 59. Cystic Renal Disease  Review         Simple Cyst  complicated  hyperdense  Bosniak classification Cysts and cancer  ACKD, TS & VHL Multicystic dysplastic kidneys Autosomal Dominant and Recessive Polycystic Medullary Sponge Kidney Medullary Cystic Disease Localized Cystic Disease Renal Sinus Cysts

Editor's Notes

  1. Go to school - a common vexer in radiology - probably cyst on US. Take advantage of opportunities to look at US of known cysts to get better ie Seek veritas
  2. Figure 9.  Multicystic dysplastic kidney. (b) US scan shows a different pattern with larger cysts and more echogenic, dysplastic parenchyma.
  3. Multicystic dysplastic kidney in a 23-week-old fetus. (a) Sagittal MR image shows a large, multicystic structure in the left renal fossa (arrowhead). The cysts vary in size and are not connected, findings that are consistent with multicystic dysplastic kidney. The stomach is seen just inferior to the diaphragm (arrow). (b) Sagittal MR image shows a normal right kidney (arrowhead).
  4. Hi-res us now shows cystic areas
  5. Prenatal us on left with echogenic enlarged kidneys, ct on right in newborn