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Renal Cysts and ADPKD

J. T. Bestoso

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Overview

Cysts - simple and complex ADPKD Types 1&2

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5 things to remember about renal cysts

1. Develop from pre-existing renal tubulesegments

2. The cyst epithelium shows abnormaldifferentiation and sustained proliferation

3. The epithelium transforms from absorptive tosecreting

4. The extracellular matrix is remodeled toaccommodate the growth

5. Endocrine, autocrine, paracrine factorsapparently modulate the process

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Simple cysts

Prevalence

Mostly in patients > 50 Twice as common in males. Bilateral rare under 50. In half of population by age 50.

(Clinical Radiology, 1983)

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Simple Cysts

Prevalence

U/S survey in 729 patients (AJKD1993)

Age Male Female

15-29 0 0

30-49 1.9 1.4

50-69 15 6.7

>70 32.3 14.6

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Simple Cysts

Characteristics

Micro: lined with simple cuboidalepithelium

Fluid clear, homogeneous, similar tourine

Most 0.5-1.0 cm, commonly 3-4 cm,occasionally much larger

Not calcified Few or no thin septations 1-a few on each kidney - rarely

diffuse

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Simple Cyst

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Large Simple Cysts

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VeryLargeSimpleCyst

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Simple Cysts

Complications

Generally clinically silent Rare high-renin hypertension (better

with drainage)

Rare pain and hemorrhage Rare infection - generally must drain

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Simple Cysts

Imaging - U/S

Round, smooth wall, sharply demarcated. No internal echoes Strong posterior wall echo

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Normal Renal Ultrasound

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Simple Cyst

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Simple Cyst

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Simple Cysts

Imaging - CT

Sharply demarcated Smooth, thin wall Generally < 20 Hounsfield units

(occasionally higher) No enhancement with contrast

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Simple Cyst Non-Contrast CT

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Simple Cyst Contrast CTno enhancement

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Simple Cyst Contrast CTno enhancement

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MRI Simple Cyst - noadvantage over CT

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MR vs CT

Radiology 2004;231:365-371

Bosniak study69 cysts imaged by CT, then MR within 1 year 7 (10%) upgraded (worse) by MR for increased number or

thickness of septae or change in density

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IVP

SimpleCyst

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Complex cysts

Causes

Hemorrhage Infection Tumor

Idiopathic

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Malignant Potential

Very rare to find tumor arising from asimple cyst

Suggestive of neoplasia:

Thickened, irregular walls Thickened or enhanced septaeEnhancement with contrastMultiloculationInternal echoesCalcification in wall

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Bosniak Cyst Grading

CT Classification

I. Thin wall (1 mm), no septations, no

calcifications, density 0-20, noenhancementII. Same, but with few septations and/or

few calcifications. IIf is slightly worseIII. Thick wall, septations, calcifications,

density 0-20, no enhancementIV. Thick wall, thick septations, course

calcifications, density more than 20,enhancement

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What it means

I. We know its a cystII/IIf. Were still pretty certain its just a

cystIII. We dont know what it isIV. Its probably cancer

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What to do about it

I. Do nothingII. Do nothing for 1 cyst, monitor with

serial scans if multipleIII. Surgical exploration/resectionIV. Surgical exploration/resection

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Usually dont aspirate III or IV

If positive, it is true positive, but might seed track

If negative, might still be positive

Consider aspiration

To rule out infection If lymphoma of metastases strongly suspected

Bosniak Cyst Aspiration

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QuickTime and aTIFF (Uncompressed) dec

Bosniak I

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Bosniak II

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QuickTime and aTIFF (Uncompresse

Another Bosniak II

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Bosniak

Class IIIHyperdensewith irregular,thickened wall

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Bosniak III

Non-cancerous

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BosniakClass III

Hyperdensewiththickenedwall

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Bosniak III

Non-cancerous

Bosniakhimself calledthis particularimage IIF

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Bosniak IV Cyst

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QuickTime and aTIFF (Uncompressed) decompressorare needed to see this picture.

Bosniak IV in A,Could mis-read as I or II in right

(lower) cut

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Bosniak IV cystsAssociated with renal carcinoma

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Questions about cysts?

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Autosomal Dominant Polycystic Kidney Disease

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ADPKDIncidence/Prevalence

1:400 - 1:1000 worldwide prevalence All races equally M=F US: 250,000-600,000 have the disease 4% of ESRD in US, more elsewhere

Fourth most common cause of ESRD

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ADPKD

250,000-300,000 have disease in US As of 2007, 12,500-15,000 made more than

$200,000 Their babies are as cute as buttons They dont want those kids on dialysis They give lots of money to PKD research

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ADPKD

Genetics

Autosomal dominant Nearly complete penetrance but

variable expression 85% ADPKD1 - chromosome 16 -

faster progression, worse prognosis 15% ADPKD2 - chromosome 4

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Further Heterogeneity ADPKD Mutation Database: pkdb.mayo.edu Substitutions, splices, deletions, frameshifts, nonsense, as of

2/22/10

PKD1: 436 unique pathogenic mutations of chromosome 16(16p13.3)

PKD2: 115 unique pathogenic mutations of chromosome 4 (4q21-q23)

Plus apparent modifier genes

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ADPKD

Pathogenesis

Gene products are transmembraneproteins called polycystins

Found in tissues throughout the body

Polycystin-1

Polycystin-2

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QuickTime and aTIFF (Uncompressed) decompressorare neede

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Polycystin Functions

Strongest evidence: associated with a cilium-triggered calcium channel

Good evidence for intercellular adhesion functions Possibly both or more 2 hit models to create cysts Not clear if/how they are associated with cAMP

(appears to stimulate cyst proliferation)

PC1 interacts with mTOR

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6 Possible

Polycystincomplexactionsites

Nephron Exp Nephrol 2006;103:e149e155

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ADPKD

Pathology

Less than 1% of tubules affected Cysts throughout kidney Cyst characteristics vary with nephron part Cyst fluid accumulates at rates of 0.1-1.0

ml/day Both kidneys abnormal

Renal architecture can be completelydistorted. Can weigh 4kg or more each, but generally

much less

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ADPKD

Natural History

Begins in utero in most patients (Adult PKD isa misnomer)

Generally discovered in 30s or 40s. Rarely, symptomatic in childhood or not

discovered until autopsy . 40% cant give family history - spontaneous

mutations vs variable expression 100% have evidence of disease by age 80, butonly half at ESRD

Cannot predict when/whether renal failure willdevelop in a patient.

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ADPKD

Diagnosis in established disease

Most palpable

50% with increased abdominal girth Hypertension common Hematuria - macrohematuria

associated with more rapid decline Mild polyuria U/S - multiple echo-free areas in both

kidneys CT (preferred) - cysts readily

distinguished

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ADPKD

Early disease (children)

1 cyst suggestive with familyhistory

3 or more bilateral cysts

diagnostic DNA testing Wisdom of testing for an

untreatable disease is ethicaldilemma

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ADPKD

Risk factors for more rapid progression

Younger age at diagnosis

Race - Blacks > whites, more severe with sickle trait Gender - Male > female Genetic abnormality - PKD1 > PKD2 Gross hematuria (cause vs effect) Hypertension Increased renal size Combined risk factors

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CRISP Study Consortium for Radiologic Imaging Studies of

Polycystic Kidney Disease Followed 241 patients by sequential MRI Renal and cyst volumes correlated

Inversely with the GFR, Directly with hypertension and urinary albumin

excretion Avg annual increase in kidney volume was 5.3%

Cyst growth more aggressive with large kidneys young age at diagnosis

Kidney Int 2003;64:1035-45.N Engl J Med 2006;354:2122-30.

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Mechanisms of Renal Failure

Unclear Does not appear to be compression of normal

structures Path: vascular sclerosis and interstitial fibrosis No primary glomerular injury Possibly apoptosis of non-tubular cells Correlates with kidney volume Rise in creatinine is late finding

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Clinical Features - Renal

Hypertension Concentrating defect Reduced renal blood flow Modest proteinuria Pain Kidney stones

Infection Elevation of creatinine

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Clinical Feature - Extra-renal

Liver cysts Abdominal wall weakness and herniations

GERD Migraine Valve disease (MVP)

Diverticulosis Intracranial aneurysms Aortic aneurysms?

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Hypertension

Often precedes cyst formation, with LVH

High-renin from cyst compression

of surrounding tissue ACE-I and ARB effective

No glomerular benefit, but usual CV

benefit Salt restriction, weight control, exerciseall advised

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ADPKD Gross Specimen

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ADPKD Cut Specimen

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ADPKD Cyst - Micro

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ADPKD Cyst - Micro

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ADPKD

Liver Cysts

Ultimately 75-90% affected Correlation with female sex and parity Liver dysfunction very rare Rare association with cholangiocarcinoma

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ADPKD Liver Disease(nl transaminases)

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ADPKD - Liver Cysts

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ADPKD on Ultrasound

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Liver and Kidney Cysts

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ADPKD on CT

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ADPKDCerebral aneurysms - conflicting data

4-10% prevalence, increases with age 2-4% in general population Aneurysm rupture kills 7-13% of patients Familial clustering - ask FH of CNS bleed Chronic headaches do not correlate with

CNS aneurysms Do not screen routinely

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ADPKDCerebral aneurysms - why not screen?

Expensive Absence of aneurysm does not precludelater development

Presence does not guarantee rupturemuch more than in general population

Therapy with significant M&M

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ADPKDCerebral aneurysms - prophylaxis

Superior BP control Avoid tobacco Control lipids Take new headaches very seriously

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Ruptured Berry Aneurysm

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ADPKD

Other CV anomalies

Mitral valve anomalies in 30% Increased frequency of other

incompetence of other valves.

Thoracic aortic aneurysms increasedup to 7-fold? Not holding up toscrutiny.

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ADPKD

Infections

Hard to diagnose

Urine cultures rarely positive Infected cysts hard to find, harderto drain

Antibiotics must be able to enter

cysts - use sulfa, floroquinolones Avoid aminoglycosides and

penicillins

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ADPKD

Kidney stones

About 20% CT best for detection Mostly CaOx or urate Treat as other stones

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Pain

Acute With cyst rupture May require narcotics and sedation

Chronic Can lead to narcotic dependence

Avoid demerol Operative interventions - generally declined

Drain dominant cysts Marsupialize Denervate

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Laparoscopic denervation

J. Urol 175, 2274-2276, 2006

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ADPKD

Miscellaneous effects

Increased diverticulosis Increased inguinal/abdominal hernia Increased migraine headache Incidental splenic and pancreatic cysts

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ADPKD

Therapy

Avoid hypokalemia - seems toincrease cyst growth

Avoid contact sports

Acute pain/hemorrhage -bedrest and analgesics, cystdrainage

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Older, Unproven Options

Assuming cyst enlargement causes renalfailure:

Amiloride reduces cyst growth in animalsby blocking Na transport KI 1989; 35:1379-89 Caffeine in vitro increases cAMP

production and tubule cell secretion JASN 2002;13:2723-9

Neither studied in humans

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Polycystin Functions

Strongest evidence: associated with a cilium-triggered calcium channel

Good evidence for intercellular adhesion functions

Possibly both or more 2 hit models to create cysts Not clear if/how they are associated with cAMP

(appears to stimulate cyst proliferation)

PC1 interacts with mTOR

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6 PossiblePolycystincomplexactionsites

Nephron Exp Nephrol 2006;103:e149e155

Intracellular Pathways in ADPKD

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Kidney International 76 , 14916, 2009

Intracellular Pathways in ADPKD

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Future DirectionsSirolimus

Sirolimus = Rapamycin Anti-proliferative immunosuppressant ADPKD kidneys often continue to grow atfer

transplant NK regression noted in many pts with sirolimus;

also seen in rat models Abnormal Polycystin-1 may be a dysregulator of

mTOR (mammalian Target of Rapamycin) Blocking mTOR may block cyst development

Proc Natl AcadSci USA 103: 5466-712006

NDT2006Mar;21(3):598-604.

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mTOR

Serine/threonine kinase Specifically and uniquely inhibited by

rapamycin (sirolimus) Component of 2 distinct signaling pathways mTORC1 stimulates cell growth and proliferation mTORC2 involved in cell polarity and cytoskeleton

Rapamycin has no effect on mTORC2

J Am Soc Nep hrol 20 :2 4 9 32 5 0 2, 2 0 0 9

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mTORC1 effects

Involved in coordinating growth of cellsas they move through cell cycle

Mitosis results in 2 equal daughter cellsof half original size

mTORC1 seems to help stimulate and

regulate the growth process

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The cytoplasmic tail of polycystin 1 interactswith and inhibits mTOR

Less PC1 in ADPKD1 so Marked increase in mTOR activity which Leads to increased cellular proliferation in

renal tubular cyst cells

Rapamycin seems to inhibit the process Unknown how or if polycystin 2 interacts withmTOR

mTOR in ADPKD

Shillingford et al, Proc Natl Acad Sci 103:54665471, 2006

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Clinical Observations

Rapamycin slows cyst growth in nativekidneys of humans with kidney

transplants Works in rat and mouse models of PKD Pilot sudy showed promise

Three major clinical trials ongoing

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Pilot Study of Sirolimus

16 patients 8 with ARB 8 with ARB

and sirolimus1 mg/day 6 months Creat < 2

Sirolimus Control

Age 40.4 41.1

M/F 7/1 6/2

MRI kidneyvolume pre

2845 2667

MRI kidneyvolume post

3221 3590

Change in size +13% +35%

P NS P< 0.05

Sirolimus level 4.6

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V2 receptor antagonists

The polycystin complex has a role in intracellularcalcium homeostasis

PC1 signals PC2 to form calcium channel

Increased calcium leads to increased cAMPproduction

Vasopressin stimulation separately associated withcAMP production

cAMP part of cascade to insert AQP2 into tubule cellmembranes

Increased AQP2 may lead to cyst growth

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Tolvaptan already approved for SIADH and CHF Major side effect so far is polyuria (4-5 liters/day)

Inhibits PKD in rat and mouse models PKD rats with vasopressin knockouts are protected,

but develop cysts with exogenous vasopressin TEMPO Trials (Tolvaptan Efficacy and Safety in

Management of PKD and Outcomes) Phase 3 ongoing with 1445 patients randomized in 133

centers

V2 receptor antagonists

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Somatostatin analogue Inhibits cAMP production

Two small studies showed small decreaseskidney and liver volumes at 6 months

GFR not improved Two more studies ongoing.

Octreotide

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ADPKD

ESRD

50% by age 60 ADPKD patients tolerate dialysis better

than most Often need no (or less) EPO PD assumed complicated hernias, by lack

of space for fluid Transplantation may lead to need for

nephrectomy To make room To extirpate infection To avoid hemorrhage

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ADPKD

Counseling

Tell parents about genetics/50%inheritance

Genetic testing not clinically relevant Diagnosis in child will lead to loss of insurance

Follow BP and physical exam in

children annually Everyone with ADPKD has obviouscysts by age 30 - if none present,disease not there

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Questions about ADPKD?

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Autosomal RecessivePolycystic Kidney Disease

(ARPKD)

Overview

1:6000-40,000 births

1:4 chance of inheriting Genetic defect on chromosome

6 Affects liver and kidneys

inversel

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ARPKD

Natural history

Considerable variation of expression 75% die within days of birth

(pulmonary hypoplasia/insufficiency)

Remainder have 75% survival at age15

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ARPKD

Pathology - severe form

Kidneys symmetrically enlarged to

10X normal (300 gm vs 25 gm) 1-2 mm cortical cysts connect with 1-8 mm radial medullary cysts - 60-

90% of collecting ducts

Glomeruli and proximal nephronsspared but crowded

Collecting system mostly normal

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ARPKD -Autopsy

Specimen

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ARPKD - Gross Specimen

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ARPKD - Cut Specimen

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ARPKD - Micro

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ARPKD

Pathology - milder forms (survivors)

10-25% of collecting ducts Cysts tend to be larger and

round Can be confused with medullary

sponge kidney or ADPKD Distinguish ADPKD from ARPKD

by U/S of parents

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ARPKD

Hepatic lesion

Central portal bile ducts absentor reduced in number

Peripheral portal ducts increasedin number, but with bizarre

shapes/orientations Ultimately hepatic fibrosis andportal hypertension at age 5-10

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Hepatic Fibrosis in ARPKD

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Acquired Cystic Kidney Disease

Caused by azotemia - not necessarily dialysis Diagnosis by CT

At least 3 simple cysts in each kidney0.5-4 cmIn patient with renal failureAfter exclusion of prior cystic process

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ACKD Complications

RCC - but rarely aggressive Infection

Hemorrhage/kidney rupture Erythrocytosis Stones

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ACKD

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ACKD Gross

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ACKD Gross

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ACKD asRCCKI 2002

QuickTime and a TIFF (LZW) decompressor

are needed to see this picture.

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ACKD US

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ACKD US

Clin Nephrol. 2003 Mar;59(3):153-9

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Twenty-year follow-up of acquired renal cystic disease.Ishikawa I, Saito Y, Asaka M, Tomosugi N, Yuri T, Watanabe M, Honda R.

36 HD patients followed 20 years (79-99) Renal size increased on average because of cysts - x3

males, x2 females. Stable after 15 years

1 death from spontaneous hemorrhage Rate of RCC was high No deaths from RCC - all detected early A transplanted cohort did not have higher volumes

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Medullary Sponge Kidney

Pathology

Marked enlargement of the medullarycollecting ducts

70% are bilateral Ducts dilated to 1-3 mm commonly,

rarely 7 mm Ducts contain inspissated material or

stones Intra-renal obstruction and infection are

common Cortex generally OK

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Prevalence 1:5000-1:20000 M:F = 1:2 (or 1:1) Congenital disorder - not inherited (?)

Fully developed at birth Renal failure uncommon 25% with MSK have hemi-hypertrophy

of body (1:100,000 in generalpopulation)

5-10% with hemi-hypertrophy have MSK

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Natural history

Clinically apparent in 30s-40s GFR often decreased ESRD rare

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Medullary SpongeKidney

Kidney stones

Acidification defect Hypercalciuria Hyperuricosuria

Hypocitraturia Sluggish urine flow

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Diagnosis

IVP shows characteristic pattern of radial,linear striations

Blush pattern, bunch of grapes, bouquet of flowers

CT and US usually not needed

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QuickTime and aTIFF (UncompMedullary SpongeKidney - Schematicof IVP Patterns

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MedullarySponge Kidney- Schematic of

Plain FilmPatterns

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Medullary Sponge Kidney - IVP

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Medullar

ySpongeKidney -

IVP

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MSK - IVP

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MedullarySponge

Kidney IVP -Grapes

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Medullary Sponge Kidney - US

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Medullary Sponge KidneyDifferential Diagnosis

Renal TB Papillary necrosis - analgesic, DM, SSD Other nephrocalcinosis - Primary PTH,

hypercalcemia, distal RTA

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Medullary Sponge Kidney Treatment

Acidosis with K-citrate (citrate becomes bicarb) Stones with fluids (> 2 liters/day) and K-citrate Avoid sodium Consider thiazide diuretics Treat infection vigorously and completely

Documents

ADPKD Feb 10