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Page 1: Phakomatoses - Smirniotopoulos (RSNA 2007)

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The PhakomatosesThe PhakomatosesJames G. Smirniotopoulos, M.D. James G. Smirniotopoulos, M.D.

Uniformed Services UniversityUniformed Services University4301 Jones Bridge Road4301 Jones Bridge Road

Bethesda, MD 20814 Bethesda, MD 20814 Voice: 301Voice: 301--295295--31453145

FAX: 301FAX: 301--295295--38933893

Visit us on the WEB:Visit us on the WEB:http://http://rad.usuhs.rad.usuhs.eduedu

Educational ObjectivesEducational Objectives

Describe why NFDescribe why NF--1 is truly 1 is truly ““NeurofibromatosisNeurofibromatosis””Describe three neoplasms caused by the Describe three neoplasms caused by the chromosome 22 mutation in NFchromosome 22 mutation in NF--22Explain why Tuberous Sclerosis is a Explain why Tuberous Sclerosis is a disorder of neuronal migrationdisorder of neuronal migration

DorlandDorland’’s Medical Dictionarys Medical DictionaryBirthmarksBirthmarks

“Bummer of a birthmark,

Hal”

PHAKOMATOSES:PHAKOMATOSES:Why Study Them?Why Study Them?

They are COMMON diseasesThey are COMMON diseasesDIAGNOSED by ImagingDIAGNOSED by ImagingGENETIC ImplicationsGENETIC ImplicationsSCREEN RelativesSCREEN RelativesSURVEILLANCE of AffectedSURVEILLANCE of Affected

Phakomatoses Mnemonic ToolPhakomatoses Mnemonic ToolNFNF--1 (von Reck's)1 (von Reck's)–– TRUE Neurofibromatosis #17TRUE Neurofibromatosis #17

NFNF--2 (Bilateral VS Syndrome)2 (Bilateral VS Syndrome)–– M.I.S.M.E. #22M.I.S.M.E. #22

TUBEROUS SCLEROSISTUBEROUS SCLEROSIS–– Pringle's "HAMARTOMA" DiseasePringle's "HAMARTOMA" Disease

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Tumor Suppressor Gene:Tumor Suppressor Gene:““Two HitTwo Hit”” HypothesisHypothesis

No gene

No protein

One copy of gene,

some protein

1st Mutation ‘Hit’Germ Line

Mutation: Ovary, Testis, or Embryo

Somatic

Mutation

2nd Mutation ‘Hit’

NEUROFIBROMATOSES NEUROFIBROMATOSES -- TYPESTYPES

Neurofibromatosis Type 1 (NFNeurofibromatosis Type 1 (NF--1)1)-- von Recklinghausen Diseasevon Recklinghausen Disease-- "True" Neurofibromatosis"True" Neurofibromatosis-- Prominent Cutaneous SignsProminent Cutaneous Signs-- Chromosome 17qChromosome 17q

Neurofibromatosis Type 2 (NFNeurofibromatosis Type 2 (NF--2)2)-- Bilateral Acoustic SchwannomaBilateral Acoustic Schwannoma-- "Central Neurofibromatosis""Central Neurofibromatosis"-- Minimal Skin ManifestationsMinimal Skin Manifestations-- Chromosome 22qChromosome 22q

Neurofibromatosis Type Neurofibromatosis Type 11

von Recklinghausen von Recklinghausen DiseaseDisease

Chromosome 17Chromosome 17

NIH Diagnostic Criteria: 2 from NIH Diagnostic Criteria: 2 from listlist

CafeCafe--AuAu--Lait spotsLait spots–– 6 or more6 or more–– 5 mm child, 15 mm adult5 mm child, 15 mm adult

Neurofibromas Neurofibromas -- 2 or more2 or morePlexiform Neurofibroma Plexiform Neurofibroma -- 11Axillary (Intertriginous) Freckling Axillary (Intertriginous) Freckling -- 11Optic GliomaOptic GliomaLisch Nodules (Iris) Lisch Nodules (Iris) -- 2 or more2 or more"Distinctive Bone Lesions""Distinctive Bone Lesions"11stst degree Relative with NFdegree Relative with NF--11

NFNF--1: EYE MANIFESTATIONS1: EYE MANIFESTATIONS

LISCH Nodules (Iris Hamartomas)LISCH Nodules (Iris Hamartomas)–– Penetrance > 90%Penetrance > 90%–– Specificity > 90%Specificity > 90%–– Translucent/pigmentedTranslucent/pigmented–– Small ( < 3mm.), SlitSmall ( < 3mm.), Slit--Lamp ExamLamp Exam

OPTIC GLIOMAOPTIC GLIOMA–– Up to 15% of patientsUp to 15% of patients–– Pilocytic AstrocytomasPilocytic Astrocytomas–– Benign ("HamartomaBenign ("Hamartoma--like"), Tx?like"), Tx?–– True Neoplasms, spread along SASTrue Neoplasms, spread along SAS–– up to 1/2 of Childhood ONG w/NFup to 1/2 of Childhood ONG w/NF--11

Lisch NodulesLisch Nodules

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Optic Nerve GliomaOptic Nerve Glioma Optic Nerve GliomaOptic Nerve Glioma

Bilateral Optic Nerve GliomaBilateral Optic Nerve Glioma

Courtesy of Greg Petermann, M.D.

NEUROFIBROMATOSIS NEUROFIBROMATOSIS -- 11

Cutaneous ManifestationsCutaneous Manifestations–– CafeCafe--auau--Lait spotsLait spots–– Intertriginous FrecklingIntertriginous Freckling–– Neurofibromas (Skin and SubQ)Neurofibromas (Skin and SubQ)–– Fibroma Molluscum (TNTC NFB)Fibroma Molluscum (TNTC NFB)–– Elephantiasis Neuromatosa Elephantiasis Neuromatosa

diffuse skin thickening/plexiform NFBdiffuse skin thickening/plexiform NFB--oror-- focal gigantismfocal gigantism

CafCaféé--auau--lait spotlait spot Axillary FreckleAxillary Freckle

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NEUROFIBROMATOSIS NEUROFIBROMATOSIS -- 11Bone Dysplasia and RemodelingBone Dysplasia and Remodeling

MacrocephalyMacrocephalyCraniofacial dysplasiaCraniofacial dysplasia–– especially sphenoidespecially sphenoid

VertebraeVertebrae (scalloping, scoliosis)(scalloping, scoliosis)PseudoarthrosisPseudoarthrosis–– especially congenitalespecially congenital

Genu Valgum/VarumGenu Valgum/VarumTwisted "Ribbon Ribs"Twisted "Ribbon Ribs"

Sphenoid DysplasiaSphenoid Dysplasia

Courtesy of Greg Petermann, M.D.

Sphenoid DysplasiaSphenoid Dysplasia

Courtesy of Greg Petermann, M.D.

Progressive Progressive PseudoarthrosisPseudoarthrosis

Bowing Deformity 8 mo. Later

PseudoPseudo--arthrosisarthrosis

Hyperemic

demineralization

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Focal GigantismFocal Gigantism NERVE SHEATH TUMORSNERVE SHEATH TUMORSSchwannoma (Sporadic >> NFSchwannoma (Sporadic >> NF--2 > NF2 > NF--1)1)–– focal massfocal mass–– usually sensory root, cranial and spinal nervesusually sensory root, cranial and spinal nerves

NeurofibromaNeurofibroma–– usually NFusually NF--1, esp. if spinal or paraspinal1, esp. if spinal or paraspinal–– spindle or dumbspindle or dumb--bell lesionbell lesion

Plexiform Neurofibroma (usually NFPlexiform Neurofibroma (usually NF--1)1)–– diffuse or fusiform enlargementdiffuse or fusiform enlargement

Malignant Peripheral Nerve Sheath TumorMalignant Peripheral Nerve Sheath Tumor–– NFNF--1 or Sporadic1 or Sporadic

Neurofibroma vs. SchwannomaNeurofibroma vs. Schwannoma

NeurofibromaNeurofibroma–– Schwann cellsSchwann cells–– FibroblastsFibroblasts–– Acellular materialAcellular material–– InfiltratingInfiltrating–– Resect Parent NerveResect Parent Nerve

SchwannomaSchwannoma–– Schwann Cell Schwann Cell

NeoplasmNeoplasm–– Secondary vascular Secondary vascular

changeschanges–– Mostly cellularMostly cellular–– EncapsulatedEncapsulated–– Nerve Sparing SurgeryNerve Sparing Surgery

SchwannomaSchwannoma

NeurofibromaNeurofibroma Distribution of Nerve Sheath Distribution of Nerve Sheath TumorsTumors

IntraIntra--Cranial => SchwannomaCranial => Schwannoma–– Sporadic >> NFSporadic >> NF--22Spinal => Both Types (S >> N)Spinal => Both Types (S >> N)Dumbbell => Both (N >> S)Dumbbell => Both (N >> S)PNS => BothPNS => BothCutaneous => NeurofibromaCutaneous => Neurofibroma–– Usually N in NFUsually N in NF--11

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Neurofibromatosis : SpineNeurofibromatosis : Spine

Scoliosis (NFScoliosis (NF--1, only?)1, only?)–– Simple ("idiopathic")Simple ("idiopathic")–– Acute Cervical KyphosisAcute Cervical Kyphosis

Dural Ectasia (NFDural Ectasia (NF--1, only?)1, only?)–– Vertebral ScallopingVertebral Scalloping–– Arachnoid "cysts"Arachnoid "cysts"–– Lateral Thoracic meningoceleLateral Thoracic meningocele

Acute Cervical KyphoscoliosisAcute Cervical Kyphoscoliosis

Neurofibromatosis : SpineNeurofibromatosis : Spine

Neurofibroma (NFNeurofibroma (NF--1)1)Osteoporosis (NFOsteoporosis (NF--1, only?)1, only?)–– IdiopathicIdiopathic–– Parathyroid AdenomaParathyroid Adenoma

Schwannoma (NFSchwannoma (NF--2)2)Meningioma (NFMeningioma (NF--22Ependymoma (NFEpendymoma (NF--2)2)

NF-2

Neurofibromatosis:Neurofibromatosis:Enlarged Neural ForamenEnlarged Neural Foramen

Nerve Sheath TumorNerve Sheath Tumor–– NeurofibromaNeurofibroma

NFNF--1 >> sporadic1 >> sporadic"dumbbell"dumbbell““ shapeshape

–– SchwannomaSchwannomasporadic >> NFsporadic >> NF--22

Mesodermal DefectMesodermal Defect–– NFNF--1 only?1 only?–– Dural weaknessDural weakness–– Bone weaknessBone weakness

Multiple Dumbbell LesionsMultiple Dumbbell Lesions NeurofibromaNeurofibroma vs.vs. SchwannomaSchwannoma

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Rib NotchingRib Notching•Aortic Coarctation

–Older than 5-6 years–3-9 possible–Ribs 5-8 most often–1-2 arise from subclavian artery–Usually Bilateral–Unilateral on the Right

–if Coarctation involves Left Subclavian origin

•A-V Fistulae•Nerve Sheath tumors

Plexiform NFPlexiform NF

Multiple NeurofibromasMultiple Neurofibromas Tumors of the Nerve SheathsTumors of the Nerve Sheaths

Courtesy of Greg Petermann, M.D.

NEUROFIBROMATOSIS NEUROFIBROMATOSIS -- 1:1:DBODBO’’ss MR Signal AbnormalitiesMR Signal Abnormalities

T1W Bright FociT1W Bright Foci–– globus pallidusglobus pallidus

T2W Bright FociT2W Bright Foci–– w/o mass, don't enhancew/o mass, don't enhance–– Cerebellar peduncles, Pons, midbrainCerebellar peduncles, Pons, midbrain–– globus pallidus, thalamus, optic radiationsglobus pallidus, thalamus, optic radiationsWhat in the heck are they??What in the heck are they??–– Ectopic Schwann cells, Ectopic Schwann cells, MelanocytesMelanocytes ????–– IntramyelinIntramyelin VacuolesVacuoles–– DysmyelinationDysmyelination ????–– Intracellular proteinaceous fluid ?Intracellular proteinaceous fluid ?

May become Neoplasms (uncommon)May become Neoplasms (uncommon)

High signal in Brain on T2 High signal in Brain on T2 Resolve over age 10Resolve over age 10

Courtesy of Greg Petermann, M.D.

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DBODBO’’ss of NFof NF--1: Cerebellar peduncle1: Cerebellar peduncle

Early ----- Later

Neurofibromatosis Type 1Neurofibromatosis Type 1

Neurofibromatosis Type 2Neurofibromatosis Type 2

vs.

James Brown or James S.?James Brown or James S.?

Hugh Curtin of the

Mass Eye and Ear

Little man behind the

Curtin

Neurofibromatosis Type Neurofibromatosis Type 22oror

WishartWishart DiseaseDiseaseChromosome 22Chromosome 22

NEUROFIBROMATOSES NEUROFIBROMATOSES -- TYPESTYPES

Neurofibromatosis Type 1 (NFNeurofibromatosis Type 1 (NF--1)1)-- von Recklinghausen Diseasevon Recklinghausen Disease-- "True" Neurofibromatosis"True" Neurofibromatosis-- Prominent Cutaneous SignsProminent Cutaneous Signs-- Chromosome 17qChromosome 17q

Neurofibromatosis Type 2 (NFNeurofibromatosis Type 2 (NF--2)2)-- Bilateral Acoustic SchwannomaBilateral Acoustic Schwannoma-- "Central Neurofibromatosis""Central Neurofibromatosis"-- Minimal Skin ManifestationsMinimal Skin Manifestations-- Chromosome 22qChromosome 22q

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NEUROFIBROMATOSIS NEUROFIBROMATOSIS -- Type Type 22

Incidence: 1/50,000Incidence: 1/50,000Inheritance: Autosomal DominantInheritance: Autosomal DominantAge at Presentation: Birth to 40's (peak in 20Age at Presentation: Birth to 40's (peak in 20’’s)s)Sx at Presentation: Hearing loss from VSSx at Presentation: Hearing loss from VSDiagnostic Criteria: VIII massesDiagnostic Criteria: VIII massesChromosome Abnl.: 22Chromosome Abnl.: 22Cutaneous Findings: minimal (skin tags)Cutaneous Findings: minimal (skin tags)CNS Findings: Schwannoma, Meningioma, CNS Findings: Schwannoma, Meningioma, Ependymoma (intramedullary spinal cord)Ependymoma (intramedullary spinal cord)

CNS Neoplasms CNS Neoplasms -- Chromosome Loss Chromosome Loss of Heterozygosityof Heterozygosity

Schwannoma Schwannoma -- 22q22qMeningioma Meningioma -- 22q (long arm)22q (long arm)Ependymoma Ependymoma -- 2222

NF-2

NOT Neurofibroma

NOT Astrocytoma

NOT Optic GliomaNF-1

NFNF--2 ("CENTRAL"), 1 OR MORE2 ("CENTRAL"), 1 OR MORE

Bilateral VIIIth MassesBilateral VIIIth MassesRelative with NFRelative with NF--2 and either:2 and either:–– Unilateral VIIIth MassUnilateral VIIIth Mass

–– Any Two:Any Two:"Neurofibroma", Meningioma, Glioma, "Neurofibroma", Meningioma, Glioma, Schwannoma, (Congenital) Lens OpacitySchwannoma, (Congenital) Lens Opacity

Intracanalicular SchwannomaIntracanalicular Schwannoma

Bilateral Vestibular Bilateral Vestibular SchwannomaSchwannoma

T1W axial

From Laszlo Mechtler, DNI

SCHWANNOMASCHWANNOMA

55--10% of All CNS Tumors10% of All CNS TumorsBenign, Slowly growingBenign, Slowly growingF > M (Intracranial), M > F (Spinal)F > M (Intracranial), M > F (Spinal)30's 30's -- 60's, w/NF60's, w/NF--2 10's 2 10's -- 30's30'sSensory Nerves (usually):Sensory Nerves (usually):–– CNN VIII (Sup.Vestibular), V, XCNN VIII (Sup.Vestibular), V, X–– Spine: Dorsal RootsSpine: Dorsal Roots

Majority (>90%) are SporadicMajority (>90%) are SporadicMultiple in NFMultiple in NF--2, Bilat.VIII Pathognomonic2, Bilat.VIII Pathognomonic

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Bilateral Vestibular SchwannomaBilateral Vestibular Schwannoma Bilateral Vestibular SchwannomaBilateral Vestibular Schwannoma

Bilateral vestibular and one Trigeminal Schwannoma

T1-contrast axialFrom Laszlo Mechtler, DNI

8th

8th

5th

Multiple MeningiomasMultiple Meningiomas

Multiple Schwannomas and Multiple Schwannomas and MeningiomasMeningiomas

8th 8th

Multiple Schwannomas, Multiple Schwannomas, Meningiomas, and EpendymomasMeningiomas, and Ependymomas

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M I S M E SyndromeM I S M E Syndrome

M M ultipleultipleI I nheritednheritedS S chwannomachwannomaM M eningiomaeningiomaE ependymomaE ependymoma

Tuberous SclerosisTuberous Sclerosisor or

Bourneville DiseaseBourneville DiseaseChromosomes 9 and 16Chromosomes 9 and 16

TUBEROUS SCLEROSISTUBEROUS SCLEROSIS

Original Original ““VOGT TRIADVOGT TRIAD””FACIAL NEVUS (ADENOMA SEBACEUM)FACIAL NEVUS (ADENOMA SEBACEUM)SEIZURESSEIZURESMENTAL DEFICIENCYMENTAL DEFICIENCY

TUBEROUS SCLEROSISTUBEROUS SCLEROSIS

AUTOSOMAL DOMINANTAUTOSOMAL DOMINANTNo Racial/SexualNo Racial/SexualHigh Spontaneous MutationHigh Spontaneous MutationHigh PenetranceHigh Penetrance–– "SPORADIC" over"SPORADIC" over--reportedreported

Multiple GenesMultiple Genes–– TSC1 TSC1 -- 9q9q–– TSC2 TSC2 -- 16p16p

Tuberous Sclerosis, NIH Consensus Tuberous Sclerosis, NIH Consensus ConferenceConference

Major Features: Major Features: Facial angiofibroma or forehead Facial angiofibroma or forehead plaque plaque Ungual or Periungual fibroma Ungual or Periungual fibroma >3 >3 HypomelanoticHypomelanotic macules macules ShagreenShagreen patch patch Multiple retinal nodular Multiple retinal nodular hamartomas hamartomas Cortical Tuber Cortical Tuber Subependymal Nodule Subependymal Nodule Subependymal Giant Cell Subependymal Giant Cell Astrocytoma Astrocytoma Cardiac rhabdomyoma Cardiac rhabdomyoma Lymphangiomyomatosis Lymphangiomyomatosis Renal angiomyolipoma Renal angiomyolipoma

Minor Features: Minor Features: Multiple dental Multiple dental enamalenamal pits pits Hamartomatous rectal polyps Hamartomatous rectal polyps Bone cysts Bone cysts White matter migration lines White matter migration lines Gingival fibromas Gingival fibromas NonNon--renal hamartoma renal hamartoma Retinal achromic patch Retinal achromic patch "Confetti" skin lesions "Confetti" skin lesions Multiple renal cysts Multiple renal cysts

Hyman MH, Whittemore VH:"National Institutes of Health Consensus Conference:tuberous sclerosis Complex" Arch Neurol 2000; 57: 662-665.

Definite TS - (2 Major) or (1 Major + 2 Minor)

Probable TS - 1 Major + 1 Minor

Possible TS - (1 Major) or (2 Minor)

Adenoma Sebaceum Adenoma Sebaceum

AKA PRINGLE'S DISEASEAKA PRINGLE'S DISEASENOT present at birthNOT present at birthdevelop before pubertydevelop before pubertynasolabial fold nasolabial fold -->bi>bi--malarmalarpapules of angiofibromapapules of angiofibroma

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PringlePringle’’s Diseases Disease PringlePringle’’s Diseases Disease

PringlePringle’’s Diseases Disease

PringlePringle’’s Names Name–– Entire DiseaseEntire Disease–– Facial lesion onlyFacial lesion only

Mild Mental Mild Mental RetardationRetardationSeizuresSeizuresHard PotatoesHard PotatoesTubular Can Tubular Can ––‘‘TuberousTuberous’’

Subungual/Periungual FibromaSubungual/Periungual Fibroma

Confetti HypopigmentationConfetti Hypopigmentation Depigmentation:Depigmentation:

AshAsh--Leaf SpotsLeaf Spots–– (Lance(Lance-- Ovate Shape)Ovate Shape)

ConfettiConfetti-- Like HypopigmentationLike Hypopigmentation–– (Inverse Freckle)(Inverse Freckle)

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Ash Leaf MaculeAsh Leaf Macule

Lance Ovate Shape

Astrocytic HamartomaAstrocytic Hamartoma

Astrocytic HamartomaAstrocytic Hamartoma Tuberous Sclerosis Tuberous Sclerosis -- Brain:Brain:

HETEROTOPIAS AND HAMARTOMASHETEROTOPIAS AND HAMARTOMAS–– in white and gray matterin white and gray matter

CORTICAL TUBERSCORTICAL TUBERS–– "HAMARTOMAS""HAMARTOMAS"–– but with abnormal "N" cellsbut with abnormal "N" cells–– neither Astrocyte nor Neuronneither Astrocyte nor Neuron–– Decreased MyelinationDecreased Myelination–– No laminar architectureNo laminar architecture

Tuberous Sclerosis Tuberous Sclerosis -- Brain:Brain:

SUBEPENDYMAL NODULES (almost 100%)SUBEPENDYMAL NODULES (almost 100%)–– "hamartomas" vs. neoplasia"hamartomas" vs. neoplasia–– Caudothalamic grooveCaudothalamic groove–– Polypoid "Candle Gutterings"Polypoid "Candle Gutterings"

DILATED VENTRICLESDILATED VENTRICLES–– variablevariable–– obstructive, atrophic vs. "idiopathic"obstructive, atrophic vs. "idiopathic"

TUMORS 15%TUMORS 15%SubSub--ependymal Giant Cell Astrocytomaependymal Giant Cell Astrocytoma–– True neoplasm, Benign WHO Grade ITrue neoplasm, Benign WHO Grade I

Cortical TubersCortical Tubers

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Cortical TubersCortical Tubers Periventricular Ca++Periventricular Ca++

Subependymal NodulesSubependymal Nodules Subependymal NodulesSubependymal Nodules

TS in newbornTS in newborn

Courtesy Mauricio Castillo, M.D. UNC

Subependymal NodulesSubependymal Nodules

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Germinal MatrixGerminal Matrix

Hypervascular

Neuroblast Factory

Post-mitotic neuroblasts migrate out along radial glia

Subependymal nodule Cortical

tuber

medpix20791.jpg Subependymal Giant Cell Subependymal Giant Cell AstrocytomaAstrocytoma

Tubers, Nodules, and TumorsTubers, Nodules, and Tumors AngioAngiomyomyolipomalipoma

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AngioAngiomyomyolipomalipoma ANGIOMYOLIPOMA:ANGIOMYOLIPOMA:

10% w/enough FAT for plain film10% w/enough FAT for plain film1/6 of Solitary AML Pts. Have TS1/6 of Solitary AML Pts. Have TS1/31/3--1/2 of solitary AML Pts. Have other 1/2 of solitary AML Pts. Have other stigmata of TSstigmata of TS5050--80% of Pts. W/TS will have AML80% of Pts. W/TS will have AML3/4 Multiple3/4 Multiple1/3 1/3 -- 1/2 Bilateral (probably more)1/2 Bilateral (probably more)variable amts. of variable amts. of FATFAT, Smooth mm., and , Smooth mm., and vesselsvessels

Renal CystsRenal Cysts Cardiac RhabdomyomaCardiac Rhabdomyoma

SummarySummary

Phakomatoses Mnemonic ToolPhakomatoses Mnemonic ToolNFNF--1 (von Reck's)1 (von Reck's)–– TRUE Neurofibromatosis #17TRUE Neurofibromatosis #17

NFNF--2 (2 (BilBil. VIII Syndrome) . VIII Syndrome) –– M.I.S.M.E. #22M.I.S.M.E. #22

TUBEROUS SCLEROSISTUBEROUS SCLEROSIS–– Pringle's "HAMARTOMA" Disease Pringle's "HAMARTOMA" Disease

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NeurfibromatosisNeurfibromatosis Type 1:Type 1:Skin, Nerves, Optic Skin, Nerves, Optic

Astrocytoma, Brain Astrocytoma, Brain DBODBO’’ss, , Spine and BoneSpine and Bone

Courtesy of Greg Petermann, M.D.

Neurofibromatosis Type 2Neurofibromatosis Type 2No specific skin findingsNo specific skin findings

M.I.S.M.E.M.I.S.M.E.

Courtesy of Greg Petermann, M.D.

Tuberous SclerosisTuberous SclerosisHamartomas and PringlesHamartomas and Pringles Thank You!Thank You!

EUXAPIEUXAPIΣΣTTΩΩ !!

Mahalo !Mahalo !Gracias! Gracias! ObregadoObregado

Merci BeaucoupMerci BeaucoupDanke Danke ShoenShoen