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Clinical Pathological Clinical Pathological CorrelationCorrelation
Case #1Case #1
Omar Omar NazifNazif & & AlixAlix PerksPerksGrand Rounds VGHGrand Rounds VGH
March 9, 2005March 9, 2005
Case Presentation Case Presentation –– HistoryHistory
•• IDID–– 24 yo M24 yo M–– Referred from GP to Hematologist for Referred from GP to Hematologist for
polycythemiapolycythemia•• PMHxPMHx
–– Depression Depression –– started Tx last yearstarted Tx last year•• PSHxPSHx
–– NegativeNegative
HPIHPI
•• recent trip to India in February 2004recent trip to India in February 2004–– Was well throughout trip, no sick contactsWas well throughout trip, no sick contacts
•• decr [] decr [] •• no HAno HA•• no visual changesno visual changes•• rare dizzy spellsrare dizzy spells•• intermittent intermittent paresthesiasparesthesias in fingersin fingers
SHxSHx
•• No No injectableinjectable steroidssteroids•• Weekend binge drinkerWeekend binge drinker•• Smokes < 1 Smokes < 1 ppwppw•• No illicit drugsNo illicit drugs
History ContHistory Cont’’dd
•• FHxFHx–– dyslipidemiadyslipidemia
•• MedsMeds–– BupropionBupropion x 1 yrx 1 yr
•• ALLALL–– NKDANKDA
Physical ExamPhysical Exam
•• Healthy slim young man (24 yo)Healthy slim young man (24 yo)•• No No lymphadenopathylymphadenopathy•• Abdomen soft, non tender, no massesAbdomen soft, non tender, no masses•• No HSMNo HSM•• CVS & RESP exams NCVS & RESP exams N•• O2 saturation 98% RAO2 saturation 98% RA
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InvestigationsInvestigations
•• HbHb 207 207 [135[135--175]175]•• WBC 11.5WBC 11.5 [4.0[4.0--11.0]11.0]
–– Differential NDifferential N–– No No eosinophiliaeosinophilia
•• PltPlt 203203 [125[125--350]350]•• INR 1.24INR 1.24 [0.9[0.9--1.20]1.20]•• LytesLytes NN•• Cr NCr N
ImpressionImpression
•• PolycythemiaPolycythemia NYDNYD
PlanPlan
•• EPO levelEPO level–– Rule out occult EPO secreting tumorsRule out occult EPO secreting tumors
•• Abd USAbd US•• CT HeadCT Head•• Phlebotomy for 400 cc of blood for Phlebotomy for 400 cc of blood for
symptomatic improvsymptomatic improv’’t w target t w target HbHb of 160of 160
PolycythemiaPolycythemia
•• HCT > 54% MHCT > 54% M•• HCT > 51% FHCT > 51% F•• Absolute Absolute polycythemiapolycythemia
–– Incr in RBC massIncr in RBC mass
•• Relative Relative polycythemiapolycythemia–– Decr in plasma Decr in plasma volvol
Symptoms Symptoms –– PolycythemiaPolycythemia
•• HAHA•• Blurry visionBlurry vision•• DizzinessDizziness•• StrokesStrokes•• Cardiac ischemiaCardiac ischemia•• Peripheral thrombosesPeripheral thromboses
DDxDDx of of PolycythemiaPolycythemia
•• PrimaryPrimary–– PolycythemiaPolycythemia rubrarubra veravera–– Primary congenital and familial Primary congenital and familial polycythemiapolycythemia
•• SecondarySecondary–– EPO secreting tumor EPO secreting tumor –– Chronic hypoxiaChronic hypoxia–– CarboxyhemoglobinemiaCarboxyhemoglobinemia–– CushingCushing’’s s syndrsyndr–– Corticosteroids, exogenous androgensCorticosteroids, exogenous androgens–– AltitudeAltitude–– R to L shuntR to L shunt
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CT HeadCT Head
•• NormalNormal
RadiologyRadiology
Abdominal USAbdominal US
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RK
RadiologyRadiology
CT Abdomen / PelvisCT Abdomen / Pelvis
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6
7
8
9
10
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Imaging SummaryImaging Summary
•• LiverLiver–– Segment 6Segment 6
•• 3 x 4 x 5 cm 3 x 4 x 5 cm multiloculatedmultiloculated cyst with thickened septacyst with thickened septa•• ? old (post infectious) ? old (post infectious) vsvs hydatidhydatid cystcyst
•• RKRK–– 2.5 x 3 x 3 cm echo poor mass 2.5 x 3 x 3 cm echo poor mass interpolarinterpolar cortexcortex–– Mild enhancement on CTMild enhancement on CT–– Solid Solid hypovascularhypovascular mass w Dopplermass w Doppler
CT Abdomen CT Abdomen –– SummarySummary
•• LiverLiver–– 4 x 2.5 cm 4 x 2.5 cm multiloculatedmultiloculated cystic lesion cystic lesion
segment 6segment 6–– Thick Thick septationsseptations ––no solid componentno solid component
–– Unlikely to be abscess re. healthy Unlikely to be abscess re. healthy presentatpresentat’’nn–– Findings suspicious for Findings suspicious for EchinococcusEchinococcus (travel (travel
HxHx))
CT Abdomen CT Abdomen –– SummarySummary
•• RKRK–– Solid lesion RK measuring 3.5 x 2 cmSolid lesion RK measuring 3.5 x 2 cm–– Incr enhancementIncr enhancement–– R renal vein NR renal vein N–– No LNNo LN’’s seens seen–– Lesion extends into renal sinusLesion extends into renal sinus
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DDxDDx
•• Solid renal massSolid renal mass–– MalignantMalignant–– BenignBenign–– InfectiousInfectious–– InflammatoryInflammatory
•• Liver CystLiver Cyst–– HydatidiformHydatidiform
EchinococcusEchinococcus
•• Tissue Tissue infectinfect’’nn d/t d/t EchinococcusEchinococcus granulosagranulosa•• Uncommon in CanadaUncommon in Canada•• Present inPresent in
–– Central AsiaCentral Asia–– Middle EastMiddle East–– MediterraneanMediterranean–– S. AmericaS. America–– US US –– AZ, CA, NM, UTAZ, CA, NM, UT
EchinococcusEchinococcus –– Risk FactorsRisk Factors
•• Exposure toExposure to–– Intermediate hostsIntermediate hosts
•• Cattle, sheep, pigs, deerCattle, sheep, pigs, deer
–– Found in feces of the definitive hostFound in feces of the definitive host•• Dogs, wolves, coyotesDogs, wolves, coyotes
•• Rare infection in US, rarer in CanadaRare infection in US, rarer in Canada
Echinococcus granulosus
Special TestsSpecial Tests
•• Right kidney FNARight kidney FNA–– Negative for Negative for
•• PMNPMN’’ss, organisms, Gram stain, AFB, Fungus, organisms, Gram stain, AFB, Fungus
•• Liver segment 6 FNA Liver segment 6 FNA –– Negative forNegative for
•• PMNPMN’’ss, organisms, Gram stain, O&P, AFB, Fungus, organisms, Gram stain, O&P, AFB, Fungus•• Few lymphocytes & macrophages presentFew lymphocytes & macrophages present
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Special TestsSpecial Tests
•• Renal Core Renal Core BxBx–– Negative for RCCNegative for RCC–– Diagnostic for Diagnostic for metanephricmetanephric adenomaadenoma
•• But, possibility of other tumors cannot be excluded based on But, possibility of other tumors cannot be excluded based on small sample sizesmall sample size
•• DDxDDx–– Adult Adult WilmsWilms’’ tumortumor–– MetanephricMetanephric adenofibromaadenofibroma–– Low grade papillary RCCLow grade papillary RCC
Renshaw AA, et al: Diagnostic Cytopathology 1997;16(2):107-111
Urologic ManagementUrologic Management
•• Open Right Radical Open Right Radical NephrectomyNephrectomy–– SubcostalSubcostal approach off tip of 12approach off tip of 12thth ribrib–– Extension of mass into renal sinus prevented Extension of mass into renal sinus prevented
partial partial NxNx–– Adrenal sparingAdrenal sparing
Gross PathologyGross Pathology
Final PathologyFinal Pathology
•• MetanephricMetanephric adenomaadenoma•• 4 x 3 x 2 cm4 x 3 x 2 cm•• Tumor confined to kidneyTumor confined to kidney•• PerinephricPerinephric fat, fat, pelvipelvi calycealcalyceal urothelialurothelial
mucosa not involved w tumormucosa not involved w tumor•• No vascular or lymphatic invasionNo vascular or lymphatic invasion•• Reactive fibrosis w/in tumor Reactive fibrosis w/in tumor c/wc/w BxBx sitesite
MetanephricMetanephric AdenomaAdenoma
1995
2005
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MetanephricMetanephric AdenomaAdenoma
•• Newly described tumorNewly described tumor•• Benign biological Benign biological behaviourbehaviour•• Described in 1995Described in 1995
–– Jones EJ et al Jones EJ et al –– 7 pt7 pt’’s s –– Davis CJ et al Davis CJ et al –– 50 pt50 pt’’ss
•• RareRare–– 80 well documented cases in literature (1999)80 well documented cases in literature (1999)
•• Occurs in children and adultsOccurs in children and adults•• M:F 1:4M:F 1:4
Jones EJ et al Am J Jones EJ et al Am J SurgSurg PatholPathol 1995:19:6151995:19:615--626626Davis CJ et al Am J Davis CJ et al Am J SurgSurg PatholPathol 1995;19:11011995;19:1101--1414
Pins MR et al Arch Pins MR et al Arch PatholPathol Lab Med 1999;123:415Lab Med 1999;123:415--420420
MetanephricMetanephric AdenomaAdenoma
•• SSxSSx–– Flank painFlank pain–– Gross Gross hematuriahematuria–– Palpable massPalpable mass
•• LabsLabs–– PolycythemiaPolycythemia–– hyperCahyperCa
Mahoney CP, et al: Pediatr Nephrol 1997 Jun; 11(3):339-42
Gross PathologyGross Pathology
•• Size 0.3Size 0.3--15.5 cm 15.5 cm •• Tumor may regress in form of scarringTumor may regress in form of scarring•• Calcification in 20%Calcification in 20%•• Gray, yellow, or tanGray, yellow, or tan•• Sharply circumscribed; satellite lesionsSharply circumscribed; satellite lesions•• Hemorrhage or necrosis may be evidentHemorrhage or necrosis may be evident•• Non encapsulatedNon encapsulated•• No multiNo multi--centricitycentricity
Microscopic FindingsMicroscopic Findings
•• Bland architecture Bland architecture –– rare mitosesrare mitoses•• Resembles Resembles epithepith component of component of WilmsWilms’’•• Extremely cellularExtremely cellular•• Basophilic epithelial cellsBasophilic epithelial cells•• Lacks fibrous interface w adjacent renal Lacks fibrous interface w adjacent renal
parenchymaparenchyma•• High High nucleus:cytoplasmnucleus:cytoplasm ratioratio
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Microscopic FindingsMicroscopic Findings
•• Nucleoli inconspicuousNucleoli inconspicuous•• No infiltrative growthNo infiltrative growth•• No vascular invasionNo vascular invasion•• Minimal cytoplasmMinimal cytoplasm•• Mitotic figures Mitotic figures –– rare or absentrare or absent
MetanephricMetanephric AdenomaAdenoma
Needle Bx
• Tightly packed small tubules with little intervening stroma
MetanephricMetanephric AdenomaAdenoma
• N parenchyma L
• Tumor is sharply demarcated from surrouding N renal parenchyma L
MetanephricMetanephric AdenomaAdenoma• Small uniform round acini and tuular structures separated by scant stroma
• Lining epithelaialcells have scant cytoplams w hyperchromaticnuclei
GeneticsGenetics
•• No chromosome No chromosome trisomytrisomy 7 & 177 & 17•• Unrelated to papillary RCC & Unrelated to papillary RCC & WilmsWilms’’
Renshaw AA, et al: Diagnostic Cytopathology 1997;16(2):107-111Pesti T, Jones EC: Human Pathology 2001;32(1):101-4Brunelli M, et al: Mod Pathol 2003;16(10)1060-3
MetastaticMetastatic PotentialPotential
•• 1 case report of a 1 case report of a metastaticmetastatic metanephricmetanephricadenoma in a 7 yo Fadenoma in a 7 yo F–– Mets to Mets to paraAOparaAO, , hilarhilar, AO bifurcation LN, AO bifurcation LN’’ss
Renshaw AA, et al. Am J Surg Pathol24(4):570-4, 2000
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Synchronous MalignancySynchronous Malignancy
•• Case report of 8 yo F with Case report of 8 yo F with metanephricmetanephricadenoma and foci of adenoma and foci of pRCCpRCC and metastasisand metastasis
Drut R, et al: Int J Surg Pathol 2001;9(3):241-7
MetanephricMetanephric AdenomaAdenoma
• Tight packaging of small acini
• Near solid appearance
• No cytologicatypia
• Mitotic figures rare
MetanephricMetanephric AdenomaAdenoma• 50% of cases have papillary structures
• Minute cysts into wc blunt papillary structures protrude
• Resemble immature glomeruli
• Typ no bld vessels in these papillary strucutures
MetanephricMetanephric AdenomaAdenoma
• Psammomabodies are commonly seen in MA
• 1st defined by EC Jones
SummarySummary
•• Almost always benign clinical course with Almost always benign clinical course with paraneoplasticparaneoplastic syndromesyndrome
•• Primarily a pathological DxPrimarily a pathological Dx–– lack of clinical, radiographic, or lack of clinical, radiographic, or cytologiccytologic
means of making definitive Dxmeans of making definitive Dx