PATOLOGIE TUMORALI

ENDOCANALARI

U.O. NeuroradiologiaIstituto Neurologico IRCCS Fondazione C. Mondino, Pavia

Dott.ssa Anna Pichiecchio

SPINAL TUMORSSPINAL TUMORS

Uncommon lesions Classification based on their location

EXTRADURAL (60% of all spinal

tumors)

INTRAMEDULLARY (5%-10% of all spinal

tumors) INTRADURAL-

EXTRAMEDULLARY (30% of all spinal

tumors)

gliomas

Meningiomas and neurinomaLeptomeningeal dissemination

MetastasisExtension of primary bone

tumors

MRI gold standard!TR TE FOV Matrix

Sag T2-w 3500 120 275 304X225

Sag T1-w 400 10 275 304X225

Sag T2 STIR

2500 (TI=170)

70 275 256X190

Ax T2-w 4500 120 230 288X200

Ax T1-w 541 15 225 304X212

Sag T1-w fat

suppression post

contrast

400 10 275 304X212

Ax T1-w post

contrast541 15 225 304X212

EPIDURAL FAT

SUBDURAL SPACE

EPIDURAL FAT

SEPTUM POSTICUM

DURA MATER

ARACHNOIDDURAL NERVE ROOT SLEEVE

DENTICULATE LIGAMENT

SPINAL CORD AND

PIA

JUNCTION OF DURAL SLEEVE AND SPINAL NERVE PERINEURIUM

1. INTRAMEDULLARY TUMORS1. INTRAMEDULLARY TUMORS

Summary

Three general characteristics:

1)they tend to enlarge the cord either focally or diffusely

2) on T2-w they produce high signal intensity

3) They enhance

1.a. SPINAL CORD 1.a. SPINAL CORD ASTROCYTOMAASTROCYTOMA

From astrocytesMost WHO I-II

Most common in CHILDREN

80-90% low gradePilociticFibrillary

Association with abnormalities of chromosome 17p (TP53

gene)

•20% intraspinal neoplasms•60% primary spinal cord tumors

subarachnoid dissemination may occur!

BEST DIAGNOSTIC

CLUE

Enhancing infiltrating cord mass in childNo capsule or cleavage

LOCATION Thoracic >Cervical > Lumbar

SIZE Usually 1-3 cm, less than 4 segments

MORPHOLOGY

fusiform expansion of cord, eccentric , posterior

General featuresGeneral features

Risk in patients with Neurofibromatosis (NF1 and NF2)

Demographic features:

A) Age: two peaks: 10 - 30 ys

B) Gender: M > F (1,3:1); no gender difference in

children!

b) 40% cysts and syringohydromyelia c) Solid portion (hypo-isointense)

CORD EXPANSIO

N

MR FindingsMR Findings

Usually < 4 segments

T1-w:a) Cord expansion

Occasionally multisegmental (holocord more common with

pilocytic astrocytomas)

From Rossi et al 2007

MR FindingsMR FindingsT2-w:a) Hyperintense

b) Not as hyperintense if cysts/syrinx

a) Rarely hemorrhagic

T2* GRE:

HYPERINTENSE LESION

CYSTS

From Rossi et al 2007

RARELY HEMORRAGE

CYSTS

MODERATE C.E. ALWAYS!!

From Rossi et al 2007

T1-w post-contrast:

a) enhancement

From Baker et al 1999

DIFFERENTIAL DIFFERENTIAL DIAGNOSISDIAGNOSISSARCOIDOSIS

INTRAMEDULLARY EPENDYMOMA INTRAMEDULLARY

HEMANGIOBLASTOMA

SYRINGOMYELIA

ARNOLD-CHIARI I MALFORMATION

1.b. SPINAL CORD 1.b. SPINAL CORD EPENDYMOMAEPENDYMOMA

Myxopapillary ependymomas

(from ependymal cells of filum terminale)

Cellular ependymomas (lining spinal cord central canal)

Neoplasm of ependyma lining spinal cord central canal

•Most common in ADULT (60%) in lower spine[ependymoma: 4% of all primary CNS tumors]

•2nd most common in CHILDREN

Associated with:•ESA•Superficial siderosis•NF2 deletion or traslocation chr 22

CELLULAR EPENDYMOMACELLULAR EPENDYMOMA

BEST DIAGNOSTIC

CLUE

Circumscribed, enhancing cord mass with hemorrhage

LOCATION Conus> Cervico-Thoracic

SIZEMultisegmental (typically 3-4 segments)

MORPHOLOGY

Well-circumscribed, symmetric cord expansion, occasional exophytic component

General featuresGeneral features

Demographic features:

A) Age: 40 yrs

B) Gender: M:F=3:1

C) Ethnicity: non racial predilection

Radiological featuresRadiological features

MR FindingsMR Findings

T1-w:

a) Iso- or slightly hypointense to spinal cord

b) Hemorrhage

ISO- HYPOINTENSE LESION

Radiological featuresRadiological features

MR FindingsMR FindingsT2-w:

c) Syrinxd) Focal hypointensitye) Surrounding cord

oedema

Hemosiderin“Cap sign”

a)Hyperintenseb) Polar (rostral or caudal) or intratumoral cysts

(50-90%)

HEMOSIDERIN“CAP SIGN”

MR FindingsMR FindingsSTIR:

a) HyperintenseT1-w post-contrast:a) Intense,well-

delineated homogeneous c.e. (50%)

b) Nodular, peripheral, heterogeneous c.e.

c) Minimal or no c.e. rare

Radiological featuresRadiological features

C.E.

HEMOSIDERIN“CAP SIGN”

ASTROCYTOMA

Astrocytoma Ependymoma

cervical conus

Eccentric posterior

central

- hemorragic

hyercellularity

Infiltrative (poor borders)

Pseudocapsule (plane)

EPENDYMOMA

from ependymal cells of filum terminale

MYXOPAPILLARY MYXOPAPILLARY EPENDYMOMAEPENDYMOMA

27-30% of all ependymomas

90% of all filum terminale tumors

Radiological featuresRadiological features

MR FindingsMR Findings

T1-w:a) Usually Isointense with cord

b) Hyperintensity (accumulation of mucin!)

T2-w:a) Hyperintenseb) Hypointensity at

tumor margins (hemosiderin)STIR:a) Hyperintense

T1-w post-contrast:a) Intense c.e.From Rossi et al 2007

From Rossi et al 2007

INTENSE C.E.

ARTERO-VENOUS FISTOLADIFFERENTIAL DIFFERENTIAL

DIAGNOSISDIAGNOSIS

From Rossi et al 2007

SPINAL MENINGIOMA

•Isointense with cord on T1-w and T2-w•More common in thoracic and cervical spine (conus and filum location unusual)

•Hemorrhage uncommon•Bony changes rare

1.c. SPINAL CORD 1.c. SPINAL CORD HEMANGIOBLASTOMAHEMANGIOBLASTOMA

Capillary rich neoplasms

1-5% of all spinal cord neoplasms:

•75% sporadic•25% associated with von Hippel-Lindau (VHL)

•Autosomal dominant (chromosome 3p)•Cerebellar hemangioblastomas, retinal angiomas, pheochromocytoma, renal cell carcinoma, angiomatous or cystic lesions of kidneys, pancreas and epididymis

WHO I

BEST DIAGNOSTIC

CLUE

Intramedullary mass with serpentine “flow voids”

LOCATION

Thoracic > CervicalSubpial (posterior, rarely anterior) often associated with intraspinal cysts

SIZEFew mm to several cm

MORPHOLOGY

Round, well-defined margins

General featuresGeneral features

Demographic features:A) Age: mean age at presentation

30 ysB) Gender: M = F

Radiological featuresRadiological features

MR FindingsMR FindingsT1-w:

a) Isointenseb) Cyst!!

(> 50%)

Small: Large:a) hypo-

isointense

b) “flow voids” (lesion > 2,5 cm)

ISO-

HYPOINTENSE LESION

+ SYRINX

Radiological featuresRadiological features

MR FindingsMR FindingsT2-w:a) Uniformly hyperintense

(small lesions) mixed hyperintense (hemorrhage common)

a) +/- peritumoral oedemab) Syrinx fluid

HYPERINTENSE LESION +

CYST

Radiological featuresRadiological features

a) Subpial nodule

Well-demarcated, intense, homogeneous C.E. (often on surface of dorsal cord)

Cyst/Syrinx: no c.e.

MR FindingsMR FindingsT1-w post contrast:

SUBPIAL NODULE ON THE DORSAL

SURFACE

ARTERIOVENOUS MALFORMATION (AVM)

CAVERNOUS MALFORMATION

•Cord often normal/small, gliotic• focal nodule absent, dorsal veins prominent•M 50-60 y insidious onset of lower extremities weakness

•Pattern of prior hemorrhage, hemosiderin rim•Minimal c.e.

From Chabert E et al. J Neuroradiol 1999; 26:262-268

Ependymoma

Astrocytoma

metastasis

mass centrally located

not hypervascular; peritumoral oedema common

Crowley, Neurosurgery 2008

Renal cell carcinoma

1)they tend to enlarge the cord either focally or diffusely

2) on T2-w they produce high signal intensity

3) They enhance

Demylinating diseases Infections Inflammation

Multiple SclerosisADEM

Cord swelling from extramedullary infectious process (as meningitis producing vascular compromise)AIDS

SarcoidLES

Syringohydromyelia Vascular lesions Causes of subacute necrotizing myelopahty

HemorrageAcute infarctionVenous hypertensionCavernous angiomaArterovenous malformation

ADEMDevic, MS HZV,Rubeola, Mononucleosis, mumpsToxo, TBCLupusSpinal dural AVMVenous hypertension

Symptoms include pain, weakness and muscle atrophy

EPIDURAL FAT

SUBDURAL SPACE

EPIDURAL FAT

SEPTUM POSTICUM

DURA MATER

ARACHNOIDDURAL NERVE ROOT SLEEVE

DENTICULATE LIGAMENT

SPINAL CORD AND

PIA

JUNCTION OF DURAL SLEEVE AND SPINAL NERVE PERINEURIUM

2. INTRADURAL EXTRAMEDULLARY 2. INTRADURAL EXTRAMEDULLARY TUMORSTUMORS

Schwannoma, Neurinoma Neurofibroma

Arise from single nerve, from one side Typically dorsal root Schwann cellsDo NOT envelope the adiacent nerve rootLobulatedSingle

Schwann cells and fibroblasts between dispersed nerve fibers, mps matrixSpreads apart axonsDO envelope the adiacent nerve rootFusiformMultiple

2.a. SCHWANNOMA2.a. SCHWANNOMA

Neoplasm of nerve sheath in PNS

Most common intradural extramedullary mass30% primary spinal tumors

•Mostly sporadic (inactivation of NF2 gene)• Association with NF2, bilateral multiple

WHO grade I

BEST DIAGNOSTIC

CLUE

Well-circumscribed, “dumbbell” shaped, enhancing spinal mass

LOCATION

Thoracic > Cervical = Lumbar

70-75% intradural extramedullary15% completely extradural15% “dumbbell” (both intra- and extradural)

SIZE Few mmGiant schwannoma: > 2 vertebral segments

MORPHOLOGY

Round, lobulated

General featuresGeneral features

Demographic features:

A) Age: 40 ys

B) Gender: M = F

C) Ethnicity: no racial predilection

Radiological featuresRadiological features

T1-w:Isointense

T2-w:a) hyperintenseb) 45%cyst 10% hemorragec) “Target sign”

HYPERINTENSE LESION WITH TARGET SIGN

ISOINTENSE LESION

Radiological featuresRadiological features

T1-w post-contrast:a) Intense c.e.

(uniform or peripheral)

C.E.

2.b. NEUROFIBROMA2.b. NEUROFIBROMA

Localized, diffuse or plexiform neoplasm

of nerve sheath

WHO grade IWHO grade III/IV

•5% of all benign soft tissue tumors

•Association with neurofibromatosis 1 (NF1) 13-65% have spinal neurofibromas

ISOINTENSE LESION

HYPERINTENSE LESION

+ HYPOINTENSE

SEPTATIONS

C.E.

DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSISCHRONIC INFLAMMATORY DEMYELINATING

POLYNEUROPATHY (CIDP)

2.c. MENINGIOMA2.c. MENINGIOMAOrigin from arachnoid

Attached to the dura mater for infiltration

SECOND MOST COMMON

INTRADURAL EXTRAMEDULLAR

Y TUMOR> 25% WHO I

Solitary

Genetic predisposition in women! •Almost all have chromosome 22 abnormalities•Association with neurofibromatosis 2 (NF2)

Meningothelial Fibroblastic

Psammomatousangiomatous

BEST DIAGNOSTIC

CLUE

Enhancing intradural extramedullary mass with “dural tail”

LOCATION

Thoracic (80%)>> Cervical (16%) > Lumbar (4%)Typical intradural extramedullaryPosterolateral (anterior cervical)

SIZE Variable

MORPHOLOGY

Typically round with dural attachment, Ca ++ < 5%

General featuresGeneral features

Demographic features:A) Age: 50-60 yrs

B) Gender: F:M=4:1 (>70-80% female!)

Radiological featuresRadiological features

T2-w:a) Isointenseb) Iso- hyperintensec) Hypointense

(calcification)

MR FindingsMR Findings

ISOINTENSE LESION WITH

CALCIFICATION

T1-w:

Radiological featuresRadiological features

T1-w post-contrast:a) Prominent c.e.b) May see

enhancing “dural tail”

C.E.

NEURINOMA

More anterior

Very hyperintense on T2-w with Target sign

Cystic change, hemorrhage more common

No dural attachment

more frequent enlargment of neural foramen

Differential diagnosis

DROP METASTASIS

EPIDURAL FAT

SUBDURAL SPACE

EPIDURAL FAT

SEPTUM POSTICUM

DURA MATER

ARACHNOIDDURAL NERVE ROOT SLEEVE

DENTICULATE LIGAMENT

SPINAL CORD AND

PIA

JUNCTION OF DURAL SLEEVE AND SPINAL NERVE PERINEURIUM

3. EXTRADURAL TUMORS3. EXTRADURAL TUMORS

LYMPHOMA

From Thurner et al.

METASTASIS

Female (breast and lung)Male (prostate and lung)

Thoracic (70%)> lumbar (20%) > cervical (10%)

DROP METASTASIS

DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSISANGIOMIOLIPOMA

DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSISGRANULOMATOSIS

INTRADURAL ARACHNOID CYST

EPIDURAL EMATOMA

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