4. BenignBenign MalignantMalignant
Pelvi-calycealPelvi-calyceal T C papillomaT C papilloma T C CarcinomaT C Carcinoma
Sq C CarcinomaSq C Carcinoma
ParenchymalParenchymal Renal papillaryRenal papillary
AdenomaAdenoma
R C CR C C
WilmsWilms
Perirenal fat&Perirenal fat&
capsulecapsule
Lipoma - FibromaLipoma - Fibroma liposarcomaliposarcoma
Vessels & smoothVessels & smooth
msms
HemangiomaHemangioma
AngiomyolipomaAngiomyolipoma
OncocytomaOncocytoma
HemangiosarcomHemangiosarcom
aa
2ry tumor2ry tumor Any secon.Any secon.
7. Algorithm for radiographicAlgorithm for radiographic
evaluation ofevaluation of renal massrenal mass
Several radiographic modalities are currently available forSeveral radiographic modalities are currently available for
detection and evaluation of renal massesdetection and evaluation of renal masses
IVPIVP :: CCalcification within the mass, increased tissuealcification within the mass, increased tissue
density, irregularity of the margin, and distortion of thedensity, irregularity of the margin, and distortion of the
collecting systemcollecting system
((may miss small anterior or posterior lesions that do not distortmay miss small anterior or posterior lesions that do not distort
the collecting system or the contour of the kidney, may notthe collecting system or the contour of the kidney, may not
always distinguish solid from cystic lesionsalways distinguish solid from cystic lesions ))
Detection by IVU is onlyDetection by IVU is only 2121% when the lesion is smaller than% when the lesion is smaller than
2 cm2 cm,,
5252% when the lesion is% when the lesion is 2-32-3 cm,cm,
andand 8585% when the lesion is% when the lesion is 3 cm3 cm or more in diameteror more in diameter
8.
9.
10.
11. Algorithm for radiographic evaluation ofAlgorithm for radiographic evaluation of renal massrenal mass
U/S:U/S: The initial imaging procedure of choiceThe initial imaging procedure of choice
Reliable for differentiation ofReliable for differentiation of solidsolid tissue fromtissue from
fluidfluid (cyst) and can establish the diagnosis of a(cyst) and can establish the diagnosis of a
simple renal cystsimple renal cyst..
It can also allow the diagnosis of anIt can also allow the diagnosis of an
angiomyolipomaangiomyolipoma by the characteristic increasedby the characteristic increased
echogenicity produced by high fat content.echogenicity produced by high fat content.
Sonographic criteria forSonographic criteria for simple cystssimple cysts include:include: 1-1-
smooth cyst wall,smooth cyst wall, 2-2- round or oval shaperound or oval shape 3-3-
without internal echoes (clear fluid)without internal echoes (clear fluid) 4-4- NoNo
calcification nor septationcalcification nor septation
14. Algorithm for radiographicAlgorithm for radiographic
evaluation ofevaluation of renal massrenal mass
If US equivocal, or suggestive of malignancyIf US equivocal, or suggestive of malignancy
solid or complexsolid or complex
with internal echoeswith internal echoes
and irregular wallsand irregular walls
if calcifications or septae are seenif calcifications or septae are seen
========Then proceed to CT========Then proceed to CT
15. Algorithm for radiographic evaluation ofAlgorithm for radiographic evaluation of renal massrenal mass
AA renal CTrenal CT scan remains the single mostscan remains the single most
important radiographic test for delineating theimportant radiographic test for delineating the
nature of a renal massnature of a renal mass..
In general, any renal mass that enhances withIn general, any renal mass that enhances with
intravenous administration of contrast materialintravenous administration of contrast material
on CT scanning by more than 15 Hounsfield unitson CT scanning by more than 15 Hounsfield units
((HUHU)) should be considered a renal cell carcinomashould be considered a renal cell carcinoma
((RCCRCC)) until proved otherwiseuntil proved otherwise
In approximately 10% of solid renal masses, CTIn approximately 10% of solid renal masses, CT
findings are indeterminate, and additional testing orfindings are indeterminate, and additional testing or
surgical exploration is needed to establish a definitivesurgical exploration is needed to establish a definitive
16. CT scan withoutCT scan without
administration ofadministration of
contrast materialcontrast material
After administration
of the contrast agent
18. Renal Cell CarcinomaRenal Cell Carcinoma
3D CT scan of a small RCC that is peripheral3D CT scan of a small RCC that is peripheral
and exophytic which is ideal for nephron sparingand exophytic which is ideal for nephron sparing
surgerysurgery
20. Algorithm for radiographicAlgorithm for radiographic
evaluation ofevaluation of renal massrenal mass
MRIMRI : is reserved for >>: is reserved for >>
contrast hypersensitive patientscontrast hypersensitive patients
high serum creatininehigh serum creatinine
Vascular invasion, IVC thrombiVascular invasion, IVC thrombi
21. Algorithm for radiographicAlgorithm for radiographic
evaluation ofevaluation of renal massrenal mass
FineFine--needle aspirationneedle aspiration or biopsy hasor biopsy has
traditionally been of limited value intraditionally been of limited value in
the evaluation of renal massesthe evaluation of renal masses
The primary indications for needleThe primary indications for needle
aspiration or biopsy of a renal mass areaspiration or biopsy of a renal mass are
when a renalwhen a renal abscess or infected cystabscess or infected cyst isis
suspected and when RCC must besuspected and when RCC must be
differentiated fromdifferentiated from metastaticmetastatic
malignant disease ormalignant disease or renal lymphomarenal lymphoma
22. Algorithm for radiographicAlgorithm for radiographic
evaluation ofevaluation of renal massrenal mass
Metastatic workupMetastatic workup
routine chest radiographroutine chest radiograph
liver function testsliver function tests
bone scanbone scan
C T chestC T chest
23. RevisionRevision
What are the indications for MRI in RCC?What are the indications for MRI in RCC?
•• possible venous involvementpossible venous involvement
•• renal insufficiencyrenal insufficiency
•• allergy to IV contrastallergy to IV contrast
What are the features suggestive ofWhat are the features suggestive of
malignancy on IVP?malignancy on IVP?
•• calcification w/i the masscalcification w/i the mass
•• increased tissue densityincreased tissue density
•• irregular marginirregular margin
•• invasion of the collecting systeminvasion of the collecting system
24. BENIGN RENAL TUMORSBENIGN RENAL TUMORS
Arise from cortical tissue (e.g.,Arise from cortical tissue (e.g., adenoma,adenoma,
oncocytomaoncocytoma) or from the various) or from the various
mesenchymal derivatives within themesenchymal derivatives within the
parenchyma or capsule of the kidneyparenchyma or capsule of the kidney
(Angiomyolipoma).(Angiomyolipoma).
Differentiation from malignantDifferentiation from malignant
renal masses by radiographic orrenal masses by radiographic or
clinical means can be challenging.clinical means can be challenging.
06/25/13
25. BENIGN RENAL TUMORSBENIGN RENAL TUMORS
Renal papillary AdenomaRenal papillary Adenoma : epithelial lesions: epithelial lesions
with a tubulowith a tubulo--papillary architecturepapillary architecture ..UsuallyUsually
measuring less than 0.5cmmeasuring less than 0.5cm
The “3-cm rule”The “3-cm rule”
Renal exploration and wedge resectionRenal exploration and wedge resection oror
other ablative therapies should be stronglyother ablative therapies should be strongly
considered for all such clinically evidentconsidered for all such clinically evident
lesions, with appropriate consideration oflesions, with appropriate consideration of
the patient's age, comorbidities, and otherthe patient's age, comorbidities, and other
relevant factors.relevant factors.
26. BENIGN RENAL TUMORSBENIGN RENAL TUMORS
OncocytomaOncocytoma:: epithelialepithelial tumor composedtumor composed
of oncocytes, largeof oncocytes, large eosinophiliceosinophilic cells havingcells having
small, round, benign-appearingsmall, round, benign-appearing nucleinuclei withwith
largelarge nucleolinucleoli..
Arise from the intercalated cells ofArise from the intercalated cells of
collecting ductscollecting ducts of the kidneyof the kidney
UnfortunatelyUnfortunately, most renal oncocytomas, most renal oncocytomas
cannot be differentiated from malignantcannot be differentiated from malignant
RCC by clinical or radiographic meansRCC by clinical or radiographic means
27. BENIGN RENAL TUMORSBENIGN RENAL TUMORS
AngiomyolipomaAngiomyolipoma :: benign tumorbenign tumor consisting ofconsisting of
varying amounts of maturevarying amounts of mature adipose tissueadipose tissue,,
smooth muscle, andsmooth muscle, and thickthick--walled vesselswalled vessels
Approximately 20% to 30% of AMLs are found inApproximately 20% to 30% of AMLs are found in
patients withpatients with tuberous sclerosistuberous sclerosis syndromesyndrome ((TSTS)),,
an autosomal dominant disorder characterized byan autosomal dominant disorder characterized by
mental retardation, epilepsy, and adenomamental retardation, epilepsy, and adenoma
sebaceumsebaceum
Middle aged femaleMiddle aged female
C/PC/P : Asymptomatic or: Asymptomatic or ( loin pain , hematuria,( loin pain , hematuria,
palpable mass, and hypovolemic shock)palpable mass, and hypovolemic shock)
28. BENIGN RENAL TUMORSBENIGN RENAL TUMORS
Angiomyolipoma:Angiomyolipoma: Diagnosed byDiagnosed by TheThe
presence of even a small amount of fatpresence of even a small amount of fat
within a renal lesion on CT scan (confirmedwithin a renal lesion on CT scan (confirmed
by a value of -20 HU or lower) virtuallyby a value of -20 HU or lower) virtually
excludes the diagnosis of RCC and isexcludes the diagnosis of RCC and is
considered diagnostic of AMLconsidered diagnostic of AML
ManagementManagement ::
1- Asymptomatic, smaller AMLs, less than 41- Asymptomatic, smaller AMLs, less than 4
cm, can be observed expectantlycm, can be observed expectantly
2- Intervention should be considered for2- Intervention should be considered for
larger tumors, particularly if the patient islarger tumors, particularly if the patient is
symptomaticsymptomatic
29.
30. Renal Cell CarcinomaRenal Cell Carcinoma
DefinitionDefinition :: Malignant tumor of renalMalignant tumor of renal
parenchymaparenchyma
TerminologyTerminology : renal cell carcinoma ,: renal cell carcinoma ,
hypernephroma , grawitz tumor , renalhypernephroma , grawitz tumor , renal
adenocarcinomaadenocarcinoma
OriginOrigin : The epithelial lining of the: The epithelial lining of the
proximal convoluted tubuleproximal convoluted tubule
Age:Age: 55thth
– 6– 6thth
decadedecade
SexSex :: M to FM to F 2:12:1
31. Renal Cell CarcinomaRenal Cell Carcinoma
Risk factorsRisk factors:: The only generally acceptedThe only generally accepted
environmental risk factor for RCC is tobaccoenvironmental risk factor for RCC is tobacco
exposureexposure , Others : obesity , hypertension ,, Others : obesity , hypertension ,
heavy metalsheavy metals
GeneticsGenetics :: Von Hippel LindauVon Hippel Lindau syndrome,syndrome,
affecting 50% of individual with this ADaffecting 50% of individual with this AD
syndrome, ch ch by , renal & pancreatic cyst,syndrome, ch ch by , renal & pancreatic cyst,
phaeochromocytoma, cerebellarphaeochromocytoma, cerebellar
hemangioblastoma , often bilaterallyhemangioblastoma , often bilaterally
34. Renal Cell CarcinomaRenal Cell Carcinoma
C/PC/P ::
1.1. AsymptomaticAsymptomatic
2.2. HematuriaHematuria
3.3. Loin painLoin pain
4.4. Palpable swellingPalpable swelling
5.5. Symptoms of metastasisSymptoms of metastasis
6.6. Paraneoplastic syndromeParaneoplastic syndrome
Classic
triad
Less than
10-15%
35. RENAL CELL CARCINOMARENAL CELL CARCINOMA
INVESTIGATIONS ;INVESTIGATIONS ;
*Laboratory studies in the evaluation of renal cell carcinoma*Laboratory studies in the evaluation of renal cell carcinoma
should include a workup for paraneoplastic syndromes. Initialshould include a workup for paraneoplastic syndromes. Initial
studies are as follows:studies are as follows:
-Urine analysis-Urine analysis
-CBC count with differential-CBC count with differential
-Electrolytes-Electrolytes
-Renal profile-Renal profile
*Liver function tests (AST and ALT)*Liver function tests (AST and ALT)
*Calcium*Calcium
*Erythrocyte sedimentation rate*Erythrocyte sedimentation rate
*Prothrombin time*Prothrombin time
*Activated partial thromboplastin time*Activated partial thromboplastin time
*Other tests indicated by presenting symptoms*Other tests indicated by presenting symptoms
37. Renal Cell CarcinomaRenal Cell Carcinoma
Paraneoplastic syndromeParaneoplastic syndrome
found infound in 20%20% of patients with RCCof patients with RCC
Due to ectopic hormone secretion by the tumorDue to ectopic hormone secretion by the tumor
Not related to the stageNot related to the stage
In general, treatment of paraneoplasticIn general, treatment of paraneoplastic
syndromes associated with RCC has requiredsyndromes associated with RCC has required
nephrectomy or systemic immunotherapy, andnephrectomy or systemic immunotherapy, and
except for hypercalcemia, medical therapiesexcept for hypercalcemia, medical therapies
have not proved helpfulhave not proved helpful
39. Stauffer’s syndromeStauffer’s syndrome is a reversible syndromeis a reversible syndrome
of hepatic dysfunction in the absence of hepaticof hepatic dysfunction in the absence of hepatic
metastases associated with RCC and can occurmetastases associated with RCC and can occur
in up to 20% of patients.in up to 20% of patients.
Hepatic function abnormalities include elevationHepatic function abnormalities include elevation
of alkaline phosphatase and bilirubin,of alkaline phosphatase and bilirubin,
hypoalbuminemia, prolonged prothrombin time,hypoalbuminemia, prolonged prothrombin time,
and hypergammaglobulinemia.and hypergammaglobulinemia.
48. Obstacles Towards TreatmentObstacles Towards Treatment
RCC is historically resistant to many types of treatmentRCC is historically resistant to many types of treatment
Chemotherapy (MDR-1)Chemotherapy (MDR-1)
RadiationRadiation
Very aggressive in nature (TGF alpha and EGFR)Very aggressive in nature (TGF alpha and EGFR)
Highly vascular (VEGF secondary to loss of vHL)Highly vascular (VEGF secondary to loss of vHL)
Expresses tumor-associated antigens (PRAME, RAGE-Expresses tumor-associated antigens (PRAME, RAGE-
1, gp75, and MN-9) which contributes to its1, gp75, and MN-9) which contributes to its
immunogenicityimmunogenicity
49. Tx of Localized RCCTx of Localized RCC
Radical nephrectomyRadical nephrectomy
Nephron-sparing surgery (NSS)Nephron-sparing surgery (NSS)
NSS with normal opposite kidneyNSS with normal opposite kidney
NSS with vHL diseaseNSS with vHL disease
Thermal ablative therapiesThermal ablative therapies
ObservationObservation
50. Radical nephrectomyRadical nephrectomy
Robson and colleagues “gold standard”Robson and colleagues “gold standard”
19691969
Prototype – A then B, Gerota’s intact, ipsiPrototype – A then B, Gerota’s intact, ipsi
adrenal, LND (crus to aortic bifurcation)adrenal, LND (crus to aortic bifurcation)
Now – no adrenal if no radiolgicalNow – no adrenal if no radiolgical
evidence unless : extensive renalevidence unless : extensive renal
involvement, locally advanced, locatedinvolvement, locally advanced, located
upper pole, immediately adjacent to adrenalupper pole, immediately adjacent to adrenal
51. Today – LND = controversialToday – LND = controversial
Heme & Lymph spreadHeme & Lymph spread
Lymphatic drainage variableLymphatic drainage variable
<2-3% benefit<2-3% benefit
However, more accurate stagingHowever, more accurate staging
Risk factors indicating LNDRisk factors indicating LND
High tumor gradeHigh tumor grade
Sarcomatoid componentSarcomatoid component
Histologic tumor necrosisHistologic tumor necrosis
Large size (> 10 cm)Large size (> 10 cm)
pT3 or pT4pT3 or pT4
*incidence 10% with 2 or >, 0.6% if <*incidence 10% with 2 or >, 0.6% if <
52. Surgical approach determined by size,Surgical approach determined by size,
location of tumor and body habituslocation of tumor and body habitus
TransperitonealTransperitoneal
SubcostalSubcostal
thoracoabdominalthoracoabdominal
ExtraperitonealExtraperitoneal
FlankFlank
Laparoscopic (trans, retro, hand-assist)Laparoscopic (trans, retro, hand-assist)
53. LaparoscopicLaparoscopic
Cancer specific survival comparable to openCancer specific survival comparable to open
Usually < 8-10cm; localized with no local invasion,Usually < 8-10cm; localized with no local invasion,
renal vein involvement, or lymphadenopathyrenal vein involvement, or lymphadenopathy
54. Postoperative Surveillance after RadicalPostoperative Surveillance after Radical
Nephrectomy for Localized Renal Cell CarcinomaNephrectomy for Localized Renal Cell Carcinoma
PathologicPathologic
TumorTumor
StageStage
History,History,
Examination,Examination,
and Bloodand Blood
TestsTests
ChestChest
RadiographRadiograph
AbdominalAbdominal
CT ScanCT Scan
T1 N0 M0T1 N0 M0 YearlyYearly —— ——
T2 N0 M0T2 N0 M0 YearlyYearly YearlyYearly Every 2 yearsEvery 2 years
T3a-c N0 M0T3a-c N0 M0 Every 6Every 6
months for 3months for 3
years, thenyears, then
yearlyyearly
Every 6Every 6
months for 3months for 3
years, thenyears, then
yearlyyearly
At 1 year, thenAt 1 year, then
every 2 yearsevery 2 years
55. Nephron-Sparing SurgeryNephron-Sparing Surgery
Czerny 1890Czerny 1890
Vermooten 1950 – NSSVermooten 1950 – NSS
Indications include situations where ptIndications include situations where pt
would be anephric or high risk of needingwould be anephric or high risk of needing
HDHD
Solitary kidney RCCSolitary kidney RCC
Bilateral RCCBilateral RCC
Contralateral disease (Hydro, chronic pyelo,Contralateral disease (Hydro, chronic pyelo,
reflux, stones, DM, nephrosclerosis)reflux, stones, DM, nephrosclerosis)
56. Indications for NSSIndications for NSS
Absolute:Absolute: Anatomical/ functional solitaryAnatomical/ functional solitary
kidneykidney
Relative:Relative: Functioning opposite kidney isFunctioning opposite kidney is
affected by a condition that mayaffected by a condition that may
impair renal function in futureimpair renal function in future
Elective:Elective: Localized unilateral RCC with aLocalized unilateral RCC with a
healthy contralateral kidneyhealthy contralateral kidney
57. A functional remnant of at least 20% ofA functional remnant of at least 20% of
one normal kidney is necessary to avoidone normal kidney is necessary to avoid
end-stage renal failureend-stage renal failure
IF solitary kidney, > 50% reduction inIF solitary kidney, > 50% reduction in
renal mass = incr risk of hyperfiltrationrenal mass = incr risk of hyperfiltration
renal injury (proteinuria, focal segmentalrenal injury (proteinuria, focal segmental
glomerulosclerosis, progressive renalglomerulosclerosis, progressive renal
failure)failure)
Prevention: Protein restriction & ACEIPrevention: Protein restriction & ACEI
58. Preoperative testingPreoperative testing
r/o local extension, mets, vascular/collecting systemr/o local extension, mets, vascular/collecting system
relationshiprelationship
Renal angio, veno, 3DCT or MRIRenal angio, veno, 3DCT or MRI
Cancer-specific survival rates 78-100%Cancer-specific survival rates 78-100%
Recurrence – undetected dz in remnantRecurrence – undetected dz in remnant
Complications – majority hemorrhagicComplications – majority hemorrhagic
59. NSS SurveillanceNSS Surveillance
StageStage H/E/labsH/E/labs CXRCXR CTa/pCTa/p
T1NOMOT1NOMO yearlyyearly -------- --------
T2NOMOT2NOMO yearlyyearly yearlyyearly q 2 yrsq 2 yrs
T3NOMOT3NOMO q 6m x 3 yr - yrq 6m x 3 yr - yr samesame q6m x3y –q2yrq6m x3y –q2yr
60. Thermal ablativeThermal ablative
Both perc or lap approachBoth perc or lap approach
Lack of histo/path stagingLack of histo/path staging
? High recurrence rate? High recurrence rate
Ideal – advanced age, comorbidities, localIdeal – advanced age, comorbidities, local
recurrance, hereditary renal cancerrecurrance, hereditary renal cancer
CryosurgeryCryosurgery
Repetition of freeze-thaw cycle (-20C)Repetition of freeze-thaw cycle (-20C)
Immediate cellular cryodestruction andImmediate cellular cryodestruction and
delayed microcirculatory failure.delayed microcirculatory failure.
Radiofrequency ablationRadiofrequency ablation
45C irreversible cell damage45C irreversible cell damage
55-60C immediate cell death55-60C immediate cell death
61. Thermal Ablative PearlsThermal Ablative Pearls
In general, enhancement within the tumor bed onIn general, enhancement within the tumor bed on
extended follow-up has been considered diagnosticextended follow-up has been considered diagnostic
of local recurrence, and the clinical experience thusof local recurrence, and the clinical experience thus
far has supported thisfar has supported this
62. ObservationObservation
Median growth rate 0.36 cm/yrMedian growth rate 0.36 cm/yr
Alternative for asymptomatic elderly andAlternative for asymptomatic elderly and
poor surgical risk, consider withpoor surgical risk, consider with
solid/small/enhancing/well-solid/small/enhancing/well-
marginated/homogeneousmarginated/homogeneous
Serial imaging 6mo or 1yr intervalsSerial imaging 6mo or 1yr intervals
Not appropriate: >3cm, poor margins,Not appropriate: >3cm, poor margins,
nonhomogeneous, young healthy with abnnonhomogeneous, young healthy with abn
imagingimaging
63. Renal Cell CarcinomaRenal Cell Carcinoma
StageStage SurgerySurgery
T 1T 1 Nephron – sparing surgeryNephron – sparing surgery openopen
laparoscopiclaparoscopic
Radical nephrectomyRadical nephrectomy laparoscopiclaparoscopic
openopen
T 2T 2 Radical nephrectomyRadical nephrectomy laparoscopiclaparoscopic
openopen
Nephron – sparing surgeryNephron – sparing surgery
T3 &T3 &
T4T4
Radical nephrectomyRadical nephrectomy openopen
64. Tx of Locally AdvancedTx of Locally Advanced RCCRCC
IVC involvementIVC involvement
Locally invasive RCCLocally invasive RCC
Local recurrence after RN or NSSLocal recurrence after RN or NSS
Adjuvant therapy for RCCAdjuvant therapy for RCC
65. IVC InvolvementIVC Involvement
Unique feature of RCCUnique feature of RCC
45-70% of RCC with IVC thrombus cured45-70% of RCC with IVC thrombus cured
Local extension/invasion much higher risk ofLocal extension/invasion much higher risk of
recurrencerecurrence
Occurs 4-10% of patientsOccurs 4-10% of patients
Suspect with : LE edema, R varicocele,Suspect with : LE edema, R varicocele,
distended abd veins, proteinuria, PE, Rdistended abd veins, proteinuria, PE, R
atrial mass, nonfxn kidneyatrial mass, nonfxn kidney
66. IVC Thrombus stagingIVC Thrombus staging
I – adjacent to ostium of renal veinI – adjacent to ostium of renal vein
II – extends up to liverII – extends up to liver
III – intrahepatic portion of IVC belowIII – intrahepatic portion of IVC below
diaphragmdiaphragm
IV – above the diaphragmIV – above the diaphragm
ImagingImaging
CT & AUSCT & AUS
Contrast inferior venacavography – if probContrast inferior venacavography – if prob
with MRIwith MRI
MRI – study of choiceMRI – study of choice
? Renal arteriography? Renal arteriography
67. ImagingImaging (contd)(contd)
Investigate locally advanced malignancy,Investigate locally advanced malignancy,
and specially forand specially for classification of Venaclassification of Vena
caval thrombus:caval thrombus:
Peri-renalPeri-renal
Infra-hepaticInfra-hepatic
Intra-hepaticIntra-hepatic
Supra-hepaticSupra-hepatic
68. Locally Invasive RCCLocally Invasive RCC
Present with pain from invasion of posteriorPresent with pain from invasion of posterior
abd wall, nerve roots or paraspinous musclesabd wall, nerve roots or paraspinous muscles
Duodenal & pancreas uncommonDuodenal & pancreas uncommon
En bloc may be beneficialEn bloc may be beneficial
Partial / debulking – only 12% alive in 1 yrPartial / debulking – only 12% alive in 1 yr
Preoperative rad – not beneficial (van derPreoperative rad – not beneficial (van der
Werf-Messing 1973)Werf-Messing 1973)
Residual tumor, rad may retard growth (Kao etResidual tumor, rad may retard growth (Kao et
al 1994)al 1994)
69. Adjuvant Therapy for RCCAdjuvant Therapy for RCC
Include hormonal manipulation,Include hormonal manipulation,
radiotherapy, vaccines, cytokines, etc…radiotherapy, vaccines, cytokines, etc…
Most studies to date – not significantMost studies to date – not significant
Vaccine – irradiated tumor cells/BCG, heatVaccine – irradiated tumor cells/BCG, heat
shock proteins (HSPPC) = no provenshock proteins (HSPPC) = no proven
benefitbenefit
Interferon alfa – modest survival benefitInterferon alfa – modest survival benefit
IL-2 – no benefitIL-2 – no benefit
70. Tx of Metastatic RCCTx of Metastatic RCC
NephrectomyNephrectomy
Hormonal therapyHormonal therapy
ChemotherapyChemotherapy
Radiation therapyRadiation therapy
Cytokines and Immunologic therapyCytokines and Immunologic therapy
Multimodal therapyMultimodal therapy
71. NephrectomyNephrectomy
1/31/3rdrd
of RCC have metsof RCC have mets
40-50% will develop mets after initial dx40-50% will develop mets after initial dx
Regression of mets after RN – 1-2% (lung)Regression of mets after RN – 1-2% (lung)
Benefit for synchronous mets withBenefit for synchronous mets with
interferon alfa after RNinterferon alfa after RN
Individuals with: adv dz (PS > 2), mets (CNS,Individuals with: adv dz (PS > 2), mets (CNS,
SC compression), MOD, significantSC compression), MOD, significant
comorbidities – not candidatecomorbidities – not candidate
72. Hormone TherapyHormone Therapy
Minimal valueMinimal value
Progesterone – inhibit growth of DES-induced renalProgesterone – inhibit growth of DES-induced renal
tumors in Syrian hamsterstumors in Syrian hamsters
No correlation with human RCCNo correlation with human RCC
Progestational agents = useful for symptomProgestational agents = useful for symptom
palliationpalliation
73. RevisionRevision
Why were RCCs originally called hypernephromas?Why were RCCs originally called hypernephromas?
idea of suprarenal origin of renal tumoursidea of suprarenal origin of renal tumours
What is the role of FNA in evaluation of renalWhat is the role of FNA in evaluation of renal
masses?masses?
•• limited valuelimited value
→→ high incidence of false negative biopsieshigh incidence of false negative biopsies
•• IndicationsIndications
→→ renal abscess or infected cyst suspectedrenal abscess or infected cyst suspected
→→ RCC must be differentiated from met orRCC must be differentiated from met or
lymphoma
74. RevisionRevision
What is the treatment for renal adenomas?What is the treatment for renal adenomas?
•• consider all to be malignantconsider all to be malignant
→→ consider wedge resection, other ablativeconsider wedge resection, other ablative
therapiestherapies
Which patient groups are at increased riskWhich patient groups are at increased risk
of developing AML?of developing AML?
•• tuberous sclerosis 20% of all AMLstuberous sclerosis 20% of all AMLs
•• female predominancefemale predominance
75. RevisionRevision
Describe the clinical presentation of RCCDescribe the clinical presentation of RCC..
•• asymptomatic and nonpalpable until advancedasymptomatic and nonpalpable until advanced
→→ >50% of RCCs now detected incidentally>50% of RCCs now detected incidentally
•• hemorrhage, paraneoplastic syndromes, or metshemorrhage, paraneoplastic syndromes, or mets
•• classic triad: flank pain, gross hematuria, andclassic triad: flank pain, gross hematuria, and
palpable abdominal mass rarely foundpalpable abdominal mass rarely found
•• indicators of advanced diseaseindicators of advanced disease
→→ constitutional sy: wt loss, fever, NS, bone pain,constitutional sy: wt loss, fever, NS, bone pain,
persistent coughpersistent cough
→→ paraneoplastic syndromesparaneoplastic syndromes
77. NEPHROBLASTOMANEPHROBLASTOMA
((Wilms’ tumour )Wilms’ tumour )
Embryonal tumour arising fromEmbryonal tumour arising from
nephrogenic blastemal cellsnephrogenic blastemal cells
can differentiate in to several cell lines -can differentiate in to several cell lines -
blastemal, epithelial and stromalblastemal, epithelial and stromal
many replicate developing kidneysmany replicate developing kidneys
Common in young children /Common in young children /
uncommon in neonates and infantsuncommon in neonates and infants
90% in < 6yrs. old ( mean: 3yrs. in boys90% in < 6yrs. old ( mean: 3yrs. in boys
and 3.5yrs. in girlsand 3.5yrs. in girls ))
78. NEPHROBLASTOMANEPHROBLASTOMA
Etiology and cytogeneticsEtiology and cytogenetics
Generally unknownGenerally unknown
World wide i.e. … No environmental factorsWorld wide i.e. … No environmental factors
Present in many syndromes :Present in many syndromes :
1-WAGR syndrome1-WAGR syndrome (Wilms, aniridia,(Wilms, aniridia,
genitourinary abnormalities, mental retardationgenitourinary abnormalities, mental retardation))
2-2-Beckwith-WiedemannBeckwith-Wiedemann syndrome (exomphalos-syndrome (exomphalos-
macroglossia-gigantismmacroglossia-gigantism ))
3-Denys-Drash syndrome3-Denys-Drash syndrome ((Gonadal dysgenesis,Gonadal dysgenesis,
nephropathy)nephropathy)
79. The most common presentation ofThe most common presentation of
Wilms tumor is the presence of anWilms tumor is the presence of an
asymptomatic abdominal mass.asymptomatic abdominal mass.
HypertensionHypertension,, gross hematuriagross hematuria, and, and
fever are observed in 5-30% of patients.fever are observed in 5-30% of patients.
A small number of patients who haveA small number of patients who have
hemorrhaged into their tumor mayhemorrhaged into their tumor may
present with signs ofpresent with signs of hypotension,hypotension,
anemiaanemia, and fever., and fever.
Rarely, patients with advanced-stageRarely, patients with advanced-stage
disease may present withdisease may present with respiratoryrespiratory
symptomssymptoms related to the presence ofrelated to the presence of
lung metastases.lung metastases.
NEPHROBLASTO
MA
80. NEPHROBLASTOMANEPHROBLASTOMA
Pathologic findingsPathologic findings (gross)(gross)
Usually solitary, sharply (well)Usually solitary, sharply (well)
defined masses with pseudocapsuledefined masses with pseudocapsule
Variable size & weight (60-6350 gms.Variable size & weight (60-6350 gms.
with a mean of 550 gms.)with a mean of 550 gms.)
Uniform, pale gray to tan, divided byUniform, pale gray to tan, divided by
prominent fibrous septa in to lobulesprominent fibrous septa in to lobules
May be cystic, hemorrhagic orMay be cystic, hemorrhagic or
necroticnecrotic
No specific location
81.
82. NEPHROBLASTOMANEPHROBLASTOMA
Microscopic findingsMicroscopic findings
Generally triphasic pattern :Generally triphasic pattern :
blastemal, epithelial and stromal cell typeblastemal, epithelial and stromal cell type
may contains heterologous elementsmay contains heterologous elements
““favorable” or “unfavorable” histologyfavorable” or “unfavorable” histology
on the bases of nuclear anaplasia i.e. . . .on the bases of nuclear anaplasia i.e. . . .
marked nuclear enlargement (3x)marked nuclear enlargement (3x)
abnormal mitoses i.e. . . . increased DNAabnormal mitoses i.e. . . . increased DNA
83. STAGINGSTAGING
Histopathology and staging are the mostHistopathology and staging are the most
important determinants of outcomeimportant determinants of outcome
StageStage DescriptionDescription
II Tumor limited to kidney & completely excised. Intact renal capsule,Tumor limited to kidney & completely excised. Intact renal capsule,
tumor unruptured. No residual tumortumor unruptured. No residual tumor
IIII Tumor extends thruogh capsule but completely excised. LocalTumor extends thruogh capsule but completely excised. Local
spillage confined to flank or biopsied tumor. Extra renal vesselsspillage confined to flank or biopsied tumor. Extra renal vessels
contain tumor thrombus or infiltratedcontain tumor thrombus or infiltrated
IIIIII Residual nonhaematogenous tumor confined to abdomen: lymphResidual nonhaematogenous tumor confined to abdomen: lymph
node involvement, diffuse peritoneal spillage, peritoneal implants,node involvement, diffuse peritoneal spillage, peritoneal implants,
tumor beyond surgical margin grossly or microscopically or tumortumor beyond surgical margin grossly or microscopically or tumor
not completely removednot completely removed
IVIV Haematogenous mets to lung, liver, bone, brain or other organHaematogenous mets to lung, liver, bone, brain or other organ
VV Bilateral renal involvement at diagnosisBilateral renal involvement at diagnosis
85. Wilms Tumor
(Nephroblastoma(
Plain X-Plain X-
Ray:Ray:
Large soft tissue massLarge soft tissue mass
displacing bowel gasdisplacing bowel gas
Calcification is uncommon.Calcification is uncommon.
93. Wilms tumor in a 2-year-old boy. A, B: Contrast-enhanced axial computed
tomography and coronal multiplanar reconstruction demonstrate a large,
round, low-density mass that distorts and displaces the enhancing
parenchyma (arrows) in the lower pole of the right kidney
Wilms Tumor
(Nephroblastoma(
95. Timing of surgeryTiming of surgery
NWTSG VS SIOP:NWTSG VS SIOP:
In NWTSGIn NWTSG: surgery with complete resection of a: surgery with complete resection of a
resectable tumour is to be performed first.resectable tumour is to be performed first.
Chemotherapy first is to be given only for those withChemotherapy first is to be given only for those with
irresectable tumour, bilateral wilms tumour, tumourirresectable tumour, bilateral wilms tumour, tumour
thrombusthrombus
In SIOPIn SIOP: surgery always is after neoadjuvant: surgery always is after neoadjuvant
chemotherapy even for resectable tumourschemotherapy even for resectable tumours
J urol 1998 159 1316-1325J urol 1998 159 1316-1325
96. NEPHROBLASTOMANEPHROBLASTOMA
prognosis and treatmentprognosis and treatment
Depends upon :Depends upon :
stage, age and histologystage, age and histology
Surgery with chemotherapy for :Surgery with chemotherapy for :
stage I & II with favorable histologystage I & II with favorable histology
surgery with chemotherapy andsurgery with chemotherapy and
radiotherapy for higher stages andradiotherapy for higher stages and
unfavorable histologyunfavorable histology
97. Stage, HistologyStage, Histology SurgerySurgery ChemotherapyChemotherapy Radiotherapy*Radiotherapy*
Stage I or II withStage I or II with
FHFH
Stage I withStage I with
anaplasiaanaplasia
NephrectomyNephrectomy
VincristineVincristine
DactinomycinDactinomycin
NoneNone
Stage III or IV withStage III or IV with
FHFH
Stage II, III, or IVStage II, III, or IV
with focalwith focal
anaplasiaanaplasia
NephrectomyNephrectomy
VincristineVincristine
DactinomycinDactinomycin
DoxorubicinDoxorubicin
YesYes
Stage II, III, or IVStage II, III, or IV
with diffusewith diffuse
anaplasiaanaplasia
Stage I, II, III, or IVStage I, II, III, or IV
CCSKCCSK
NephrectomyNephrectomy
VincristineVincristine
DoxorubicinDoxorubicin
CyclophosphamidCyclophosphamid
ee
EtoposideEtoposide
YesYes
Stage I, II, III, or IVStage I, II, III, or IV
RTKRTK
NephrectomyNephrectomy
CyclophosphamidCyclophosphamid
ee
EtoposideEtoposide
CarboplatinCarboplatin
YesYes
98. TREATMENTTREATMENT
Pre-operative chemo should be given to patientsPre-operative chemo should be given to patients
withwith
Bilateral involvementBilateral involvement
Inoperable disease at explorationInoperable disease at exploration
IVC extension above portal veinsIVC extension above portal veins
Primary nephrectomy for other patientsPrimary nephrectomy for other patients
Initial biopsy then chemotherapy then 2Initial biopsy then chemotherapy then 2ndnd
look atlook at
8 – 10 weeks for bilateral disease8 – 10 weeks for bilateral disease
99. COMPLICATIONSCOMPLICATIONS
Following irradiationFollowing irradiation
ScoliosisScoliosis
Hypogonadism & temporary azoospermiaHypogonadism & temporary azoospermia
Ovarian failure (12%)Ovarian failure (12%)
Second malignancySecond malignancy
Following chemotherapyFollowing chemotherapy
CCF following treatment with DoxorubicinCCF following treatment with Doxorubicin
100. RevisionRevision
What is the most common primary malignantWhat is the most common primary malignant
renal tumour of childhood?renal tumour of childhood?
Wilms' tumour (a.k.a. nephroblastoma)Wilms' tumour (a.k.a. nephroblastoma)
What is the embryologic origin of Wilms'What is the embryologic origin of Wilms'
tumour?tumour?
•• develops from remnants of immature kidneydevelops from remnants of immature kidney
What pathologic markers are associated w/What pathologic markers are associated w/
favourable outcome in Wilms'?favourable outcome in Wilms'?
•• classic (triphasic) composition: blastemal,classic (triphasic) composition: blastemal,
epithelial, stromal elementsepithelial, stromal elements
→→ worse w/ more blastemal elementsworse w/ more blastemal elements