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PATOLOGIE TUMORALI ENDOCANALARI. U.O. Neuroradiologia Istituto Neurologico IRCCS Fondazione C. Mondino, Pavia. Dott.ssa Anna Pichiecchio. SPINAL TUMORS. Uncommon lesions Classification based on their location. EXTRADURAL (60% of all spinal tumors).

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Patologie tumorali endocanalari

PATOLOGIE TUMORALI ENDOCANALARI

U.O. Neuroradiologia

Istituto Neurologico IRCCS Fondazione C. Mondino, Pavia

Dott.ssa Anna Pichiecchio


SPINAL TUMORS

Uncommon lesions

Classification based on their location

EXTRADURAL (60% of all spinal tumors)

INTRAMEDULLARY(5%-10% of all spinal tumors)

Metastasis

Extension of primary bone tumors

INTRADURAL-EXTRAMEDULLARY (30% of all spinal tumors)

gliomas

Meningiomas and neurinoma

Leptomeningeal dissemination



1. INTRAMEDULLARY TUMORS

EPIDURAL FAT

DURA MATER

SUBDURAL SPACE

ARACHNOID

JUNCTION OF DURAL SLEEVE AND SPINAL NERVE PERINEURIUM

DURAL NERVE ROOT SLEEVE

EPIDURALFAT

DENTICULATE LIGAMENT

SPINAL CORD AND PIA

SEPTUM POSTICUM


Summary
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 astrocytoma
1.a. SPINAL CORD ASTROCYTOMA

From astrocytes

Most WHO I-II

Most common in CHILDREN

  • 80-90%

  • low grade

  • Pilocitic

  • Fibrillary

  • 20% intraspinal neoplasms

  • 60% primary spinal cord tumors

subarachnoid dissemination may occur!

Association with abnormalities of chromosome 17p (TP53 gene)


Generalfeatures

A) Age: two peaks: 10 - 30 ys

Demographic features:

B) Gender: M > F (1,3:1);

no gender difference in children!

Risk in patients with Neurofibromatosis (NF1 and NF2)


MR Findings

T1-w:

a) Cord expansion

Occasionally multisegmental (holocord more common with pilocytic astrocytomas)

Usually < 4 segments

b) 40% cysts and syringohydromyelia

c) Solidportion (hypo-isointense)

CORD EXPANSION

From Rossi et al 2007


MR Findings

T2-w:

HYPERINTENSE LESION

a) Hyperintense

b) Not as hyperintense if cysts/syrinx

T2* GRE:

  • Rarely hemorrhagic

CYSTS

From Rossi et al 2007



T1-w

post-contrast:

a) enhancement

MODERATE C.E.

ALWAYS!!

From Rossi et al 2007


DIFFERENTIAL DIAGNOSIS

SARCOIDOSIS

INTRAMEDULLARY

EPENDYMOMA

INTRAMEDULLARY HEMANGIOBLASTOMA

From Baker et al 1999



1 b spinal cord ependymoma
1.b. SPINAL CORD EPENDYMOMA

Cellular ependymomas

(lining spinal cord central canal)

Myxopapillary ependymomas

(from ependymal cells of filum terminale)


Cellular ependymoma
CELLULAR EPENDYMOMA

Neoplasm of ependyma lining

spinal cord central canal

  • Associated with:

  • ESA

  • Superficial siderosis

  • NF2 deletion or traslocation chr 22

  • Most common in ADULT (60%) in lower spine

  • [ependymoma: 4% of all primary CNS tumors]

  • 2nd most common

  • in CHILDREN


Generalfeatures

A) Age: 40 yrs

Demographic features:

B) Gender: M:F=3:1

C) Ethnicity: non racial predilection


Radiologicalfeatures

MR Findings

ISO- HYPOINTENSE LESION

T1-w:

  • Iso- or slightly hypointense to spinal cord

  • Hemorrhage


Radiologicalfeatures

MR Findings

HEMOSIDERIN

“CAP SIGN”

T2-w:

a)Hyperintense

b) Polar (rostral or caudal) or intratumoral cysts

(50-90%)

Hemosiderin

“Cap sign”

c) Syrinx

d) Focal hypointensity

e) Surrounding cord oedema


Radiologicalfeatures

MR Findings

HEMOSIDERIN

“CAP SIGN”

STIR:

C.E.

a) Hyperintense

T1-w post-contrast:

a) Intense,well-delineated homogeneous c.e. (50%)

b) Nodular, peripheral, heterogeneous c.e.

c) Minimal or no c.e. rare


ASTROCYTOMA

EPENDYMOMA


Myxopapillary ependymoma
MYXOPAPILLARY EPENDYMOMA

from ependymal cells of filum terminale

  • 27-30% of all ependymomas

  • 90% of all filum terminale tumors


Radiologicalfeatures

MR Findings

T1-w:

  • Usually Isointense with cord

  • Hyperintensity (accumulation of mucin!)

T2-w:

  • Hyperintense

  • Hypointensity at tumor margins (hemosiderin)

STIR:

a) Hyperintense

T1-w post-contrast:

  • Intense c.e.

From Rossi et al 2007


INTENSE

C.E.

From Rossi et al 2007


DIFFERENTIAL DIAGNOSIS

ARTERO-VENOUS FISTOLA


SPINAL MENINGIOMA

From Rossi et al 2007

  • 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 hemangioblastoma
1.c. SPINAL CORD HEMANGIOBLASTOMA

Capillary rich neoplasms

WHO I

  • 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


Generalfeatures

A) Age: mean age at presentation 30 ys

Demographic features:

B) Gender: M = F


Radiologicalfeatures

MR Findings

T1-w:

Small:

Large:

ISO-

HYPOINTENSE LESION

+

SYRINX

  • Isointense

  • Cyst!!

  • (> 50%)

  • hypo- isointense

  • “flow voids” (lesion > 2,5 cm)


Radiologicalfeatures

MR Findings

T2-w:

  • Uniformly hyperintense (small lesions)

  • mixed hyperintense

  • (hemorrhage common)

  • +/- peritumoral oedema

  • Syrinx fluid

HYPERINTENSE LESION

+

CYST


Radiologicalfeatures

MR Findings

T1-w post contrast:

  • Subpial nodule

  • Well-demarcated, intense, homogeneous C.E.

  • (often on surface of dorsal cord)

Cyst/

Syrinx: no c.e.

SUBPIAL NODULE ON THE DORSAL SURFACE


ARTERIOVENOUS

MALFORMATION (AVM)

CAVERNOUS MALFORMATION

  • Pattern of prior hemorrhage, hemosiderin rim

  • Minimal c.e.

  • Cord often normal/small, gliotic

  • focal nodule absent, dorsal veins prominent

  • M 50-60 y insidious onset of lower extremities weakness

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


Ependymoma

metastasis

mass centrally located

Astrocytoma

Crowley, Neurosurgery 2008

not hypervascular;

peritumoral oedema common

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


Symptoms include pain, weakness and muscle atrophy


2. INTRADURAL EXTRAMEDULLARY TUMORS

EPIDURAL FAT

DURA MATER

SUBDURAL SPACE

ARACHNOID

JUNCTION OF DURAL SLEEVE AND SPINAL NERVE PERINEURIUM

DURAL NERVE ROOT SLEEVE

EPIDURALFAT

DENTICULATE LIGAMENT

SPINAL CORD AND PIA

SEPTUM POSTICUM


Neurofibroma

Schwannoma, Neurinoma

  • Arise from single nerve, from one side

  • Typically dorsal root

  • Schwann cells

  • Do NOT envelope the adiacent nerve root

  • Lobulated

  • Single

  • Schwann cells and fibroblasts between dispersed nerve fibers, mps matrix

  • Spreads apart axons

  • DO envelope the adiacent nerve root

  • Fusiform

  • Multiple


2 a schwannoma
2.a. SCHWANNOMA

Neoplasm of nerve sheath in PNS

  • Most common intradural extramedullary mass

  • 30% primary spinal tumors

WHO grade I

  • Mostly sporadic (inactivation of NF2 gene)

  • Association with NF2, bilateral multiple


Generalfeatures

A) Age: 40 ys

Demographic features:

B) Gender: M = F

C) Ethnicity: no racial predilection


Radiologicalfeatures

T1-w:

Isointense

ISOINTENSE LESION

T2-w:

hyperintense

45%cyst 10% hemorrage

“Target sign”

HYPERINTENSE LESION WITH TARGET SIGN


Radiologicalfeatures

T1-w post-contrast:

  • Intense c.e.

  • (uniform or peripheral)

C.E.


2 b neurofibroma
2.b. NEUROFIBROMA

Localized, diffuse or plexiform neoplasm of nerve sheath

  • WHO grade I

  • WHO 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 DIAGNOSIS

CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY (CIDP)


2 c meningioma
2.c. MENINGIOMA

Origin from arachnoid

Attached to the dura mater for infiltration

SECOND MOST COMMON INTRADURAL EXTRAMEDULLARY TUMOR

> 25% WHO I

  • Genetic predisposition in women!

  • Almost all have chromosome 22 abnormalities

  • Association with neurofibromatosis 2 (NF2)

  • Meningothelial

  • Fibroblastic

  • Psammomatous

  • angiomatous

  • Solitary


Generalfeatures

  • Age: 50-60 yrs

Demographic features:

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


Radiologicalfeatures

ISOINTENSE LESION WITH CALCIFICATION

MR Findings

T1-w:

  • Isointense

  • Iso- hyperintense

  • Hypointense (calcification)

T2-w:


Radiologicalfeatures

C.E.

T1-w post-contrast:

  • Prominent c.e.

  • May see enhancing “dural tail”


Differential diagnosis

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



3. EXTRADURAL TUMORS

EPIDURAL FAT

DURA MATER

SUBDURAL SPACE

ARACHNOID

JUNCTION OF DURAL SLEEVE AND SPINAL NERVE PERINEURIUM

DURAL NERVE ROOT SLEEVE

EPIDURALFAT

DENTICULATE LIGAMENT

SPINAL CORD AND PIA

SEPTUM POSTICUM


METASTASIS

LYMPHOMA

From Thurner et al.



DIFFERENTIAL DIAGNOSIS

ANGIOMIOLIPOMA


DIFFERENTIAL DIAGNOSIS

GRANULOMATOSIS


INTRADURAL ARACHNOID CYST

EPIDURAL EMATOMA



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