clinical pathological case presentation – history ... · case presentation – history ... –...

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1 Clinical Pathological Clinical Pathological Correlation Correlation Case #1 Case #1 Omar Omar Nazif Nazif & & Alix Alix Perks Perks Grand Rounds VGH Grand Rounds VGH March 9, 2005 March 9, 2005 Case Presentation Case Presentation – History History ID ID 24 yo M 24 yo M Referred from GP to Hematologist for Referred from GP to Hematologist for polycythemia polycythemia PMHx PMHx Depression Depression – started Tx last year started Tx last year PSHx PSHx Negative Negative HPI HPI recent trip to India in February 2004 recent trip to India in February 2004 Was well throughout trip, no sick contacts Was well throughout trip, no sick contacts decr [] decr [] no HA no HA no visual changes no visual changes rare dizzy spells rare dizzy spells intermittent intermittent paresthesias paresthesias in fingers in fingers SHx SHx No No injectable injectable steroids steroids Weekend binge drinker Weekend binge drinker Smokes < 1 Smokes < 1 ppw ppw No illicit drugs No illicit drugs History Cont History Cont’d FHx FHx dyslipidemia dyslipidemia Meds Meds Bupropion Bupropion x 1 yr x 1 yr ALL ALL NKDA NKDA Physical Exam Physical Exam Healthy slim young man (24 yo) Healthy slim young man (24 yo) No No lymphadenopathy lymphadenopathy Abdomen soft, non tender, no masses Abdomen soft, non tender, no masses No HSM No HSM CVS & RESP exams N CVS & RESP exams N O2 saturation 98% RA O2 saturation 98% RA

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Page 1: Clinical Pathological Case Presentation – History ... · Case Presentation – History ... – Adult Wilms’ tumor ... Microsoft PowerPoint - one.ppt Author: Jason Created Date:

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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

Page 2: Clinical Pathological Case Presentation – History ... · Case Presentation – History ... – Adult Wilms’ tumor ... Microsoft PowerPoint - one.ppt Author: Jason Created Date:

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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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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