PATOLOGIE TUMORALI ENDOCANALARI Dott.ssa Anna Pichiecchio U.O. Neuroradiologia Istituto Neurologico IRCCS Fondazione C. Mondino, Pavia

Classification based on their location 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 Generalmente divisi in base alla localizzazione si tratta di una generalizzazione perche’: un a stessa patologia puo’ risiedere in due comparti contemporaneamente opure due lesioni con patologia identica alla base possono insorgere in comparti differenti Cmq utile Meningiomas and neurinoma Leptomeningeal dissemination

Sag T1-w fat suppression post contrast MRI gold standard! TR TE FOV Matrix Sag T2-w 3500 120 275 304X225 Sag T1-w 400 10 Sag T2 STIR 2500 (TI=170) 70 256X190 Ax T2-w 4500 230 288X200 Ax T1-w 541 15 225 304X212 Sag T1-w fat suppression post contrast Ax T1-w post contrast In tutto l’ambito della patologia spinale la patolgoia tumorale è quella in cui l’rm has the msot impact Allo stato attuale è la modality of choice Estensione localizzazione e struttura interna della massa

1. INTRAMEDULLARY TUMORS EPIDURAL FAT SUBDURAL SPACE SEPTUM POSTICUM DURA MATER ARACHNOID DURAL NERVE ROOT SLEEVE DENTICULATE LIGAMENT SPINAL CORD AND PIA JUNCTION OF DURAL SLEEVE AND SPINAL NERVE PERINEURIUM

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 From astrocytes Most WHO I-II Most common in CHILDREN 80-90% low grade Pilocitic Fibrillary 20% intraspinal neoplasms 60% primary spinal cord tumors Il potenziale maligno è piu’ basso rispetto agli astrocitomi cerebrali e la grandezza del tumore non riflette la malignità subarachnoid dissemination may occur! Association with abnormalities of chromosome 17p (TP53 gene)

Demographic features: General features A) Age: two peaks: 10 - 30 ys Demographic features: B) Gender: M > F (1,3:1); no gender difference in children! BEST DIAGNOSTIC CLUE Enhancing infiltrating cord mass in child No capsule or cleavage LOCATION Thoracic >Cervical > Lumbar SIZE Usually 1-3 cm, less than 4 segments MORPHOLOGY fusiform expansion of cord, eccentric , posterior Risk in patients with Neurofibromatosis (NF1 and NF2)

b) 40% cysts and syringohydromyelia MR Findings T1-w: a) Cord expansion Usually < 4 segments Occasionally multisegmental (holocord more common with pilocytic astrocytomas) b) 40% cysts and syringohydromyelia c) Solid portion (hypo-isointense) CORD EXPANSION From Rossi et al 2007

b) Not as hyperintense if cysts/syrinx 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

RARELY HEMORRAGE CYSTS Ci possono essere cisti intratumorali o rostrali e caudali, queste ultime tendono ad essere benigne , possono essere proteinacee o emorragiche ma in genere non hanno cellule tumorali per cui non prendono contrasto e per questo non vanno escisse ma drenate Mentre cisti intratumorali prendono contyrasto Sono tumori infiltranti molto difficili spesso non resecabili

a) enhancement T1-w post-contrast: MODERATE C.E. ALWAYS!! Enghancement è il general rule anche se spesso non è granche’ rispetto all’intensità di enhancement dell’ependimoma From Rossi et al 2007

DIFFERENTIAL DIAGNOSIS INTRAMEDULLARY HEMANGIOBLASTOMA SARCOIDOSIS INTRAMEDULLARY EPENDYMOMA INTRAMEDULLARY HEMANGIOBLASTOMA From Baker et al 1999

ARNOLD-CHIARI I MALFORMATION SYRINGOMYELIA Dd con siringhe benigne complesse per margini indistinit segnale disomogeneo non sempre simile al CSF sempre fare contradsto in una siringa a meno che non sia una chiari I in cui lìetiologia della siringa è ovvia

1.b. SPINAL CORD EPENDYMOMA Cellular ependymomas (lining spinal cord central canal) Myxopapillary ependymomas (from ependymal cells of filum terminale)

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

Demographic features: General features A) Age: 40 yrs Demographic features: B) Gender: M:F=3:1 C) Ethnicity: non racial predilection BEST DIAGNOSTIC CLUE Circumscribed, enhancing cord mass with hemorrhage LOCATION Conus> Cervico-Thoracic SIZE Multisegmental (typically 3-4 segments) MORPHOLOGY Well-circumscribed, symmetric cord expansion, occasional exophytic component Piu’ focali !!! Anche se possono essere estesi per piu’ segmenti Nonstante non siano capsulati sono circoscritti non infiltranti e benigni istologicamente in alcuni casi tot resecabili

Radiological features ISO- HYPOINTENSE LESION MR Findings ISO- HYPOINTENSE LESION T1-w: Iso- or slightly hypointense to spinal cord Hemorrhage

Radiological features 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

Radiological features MR Findings HEMOSIDERIN “CAP SIGN” STIR: a) Hyperintense C.E. 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 cervical conus Eccentric posterior central - hemorragic hyercellularity Infiltrative (poor borders) Pseudocapsule (plane)

MYXOPAPILLARY EPENDYMOMA from ependymal cells of filum terminale 27-30% of all ependymomas 90% of all filum terminale tumors

Radiological features 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 Per propensione per cono possono essere sia intramid che extramidollari intradurali From Rossi et al 2007

DIFFERENTIAL DIAGNOSIS ARTERO-VENOUS FISTOLA Cord often normal/small, gliotic talora ingrandito sa STASI, EDEMA, poi ischemia focal nodule absent, dorsal veins prominent M 50-60 y insidious onset of lower extremities weakness

Isointense with cord on T1-w and T2-w 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 Capillary rich neoplasms WHO I 1-5% of all spinal cord neoplasms: 75% sporadic 25% associated with von Hippel-Lindau (VHL) Lesioni vascolarizzate costiutito da denso network di capillari e canali vascolari Autosomal dominant (chromosome 3p) Cerebellar hemangioblastomas, retinal angiomas, pheochromocytoma, renal cell carcinoma, angiomatous or cystic lesions of kidneys, pancreas and epididymis

Demographic features: General features A) Age: mean age at presentation 30 ys Demographic features: B) Gender: M = F BEST DIAGNOSTIC CLUE Intramedullary mass with serpentine “flow voids” LOCATION Thoracic > Cervical Subpial (posterior, rarely anterior) often associated with intraspinal cysts SIZE Few mm to several cm MORPHOLOGY Round, well-defined margins

Radiological features MR Findings T1-w: Small: Large: ISO- HYPOINTENSE LESION + SYRINX Isointense Cyst!! (> 50%) hypo- isointense “flow voids” (lesion > 2,5 cm)

Radiological features MR Findings T2-w: Uniformly hyperintense (small lesions) mixed hyperintense (hemorrhage common) +/- peritumoral oedema Syrinx fluid HYPERINTENSE LESION + CYST

Radiological features SUBPIAL NODULE ON THE DORSAL SURFACE 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. Hannpo quindi componente cisticA E COMPONENTE SOLIDA CHE ENANHA SUBPIAL NODULE ON THE DORSAL SURFACE

Possono esser multiple ed eccentriche e quindi apparire come extramidollarti

CAVERNOUS MALFORMATION ARTERIOVENOUS MALFORMATION (AVM) CAVERNOUS MALFORMATION Pattern of prior hemorrhage, hemosiderin rim Minimal c.e. AVM una delle poche soddisfaz del neuroradiologo visto che si tratano bene AC simili all’encefalo 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 Astrocytoma mass centrally located Metastasi arrivano per disseminazione ematogena oppure per crescita lungo gli spazi di v-R per disseminazione liquorale Ricordiamo che metastatizzano allo spine oligodendro, gbl, linfoma melanoma e oltre a lung breast e gastric 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 In relatà ci sono caratteristiche precipue di ognuno di loro, ma il punto non è distinguerle istopatologicamente bensi’ nella DD Non alla enlarged cords are neoplasms

Symptoms include pain, weakness and muscle atrophy Demylinating diseases Infections Inflammation Multiple Sclerosis ADEM Cord swelling from extramedullary infectious process (as meningitis producing vascular compromise) AIDS Sarcoid LES Syringohydromyelia Vascular lesions Causes of subacute necrotizing myelopahty Hemorrage Acute infarction Venous hypertension Cavernous angioma Arterovenous malformation ADEM Devic, MS HZV,Rubeola, Mononucleosis, mumps Toxo, TBC Lupus Spinal dural AVM Non alla enlarged cords are neoplasms molte cause infiammatorie e demilinizzanti possono allargarlo, quindi specialmente in pz giovani cobn sintomi acuti o subacuti nel dubbio fare RM encefalo nel caso ci siano elsioni addizionali che indirizzano. In piu’ un consiglio è che si puo’ suggerire l’ipotesi istopatologica ma talora è difficle o impossobile , l’aspetto piu’ importante è identificare che lo spinal cord is abnormal

2. INTRADURAL EXTRAMEDULLARY TUMORS EPIDURAL FAT SUBDURAL SPACE SEPTUM POSTICUM DURA MATER ARACHNOID DURAL NERVE ROOT SLEEVE DENTICULATE LIGAMENT SPINAL CORD AND PIA JUNCTION OF DURAL SLEEVE AND SPINAL NERVE PERINEURIUM

Arise from single nerve, from one side Typically dorsal root 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 Incapsulati i primi, ben circoscritti ma non incapsulati i secondi , nei secondi l’elemento cospicuo è il collagene hanno poca propensione a emorragia , modificaz vascolari o grasse a differenza dei primi

Neoplasm of nerve sheath in PNS 2.a. SCHWANNOMA Neoplasm of nerve sheath in PNS Most common intradural extramedullary mass 30% primary spinal tumors WHO grade I Le lesioni solitarie sono in genere schwannomi Mostly sporadic (inactivation of NF2 gene) Association with NF2, bilateral multiple

Demographic features: General features A) Age: 40 ys Demographic features: B) Gender: M = F C) Ethnicity: no racial predilection BEST DIAGNOSTIC CLUE Well-circumscribed, “dumbbell” shaped, enhancing spinal mass LOCATION Thoracic > Cervical = Lumbar 70-75% intradural extramedullary 15% completely extradural 15% “dumbbell” (both intra- and extradural) SIZE Few mm Giant schwannoma: > 2 vertebral segments MORPHOLOGY Round, lobulated Frequentemente associati a emorragia, camb vascolari, cisti e degeneraz grassa

Radiological features HYPERINTENSE LESION WITH TARGET SIGN T1-w: Isointense ISOINTENSE LESION T2-w: hyperintense 45%cyst 10% hemorrage “Target sign” Target sign alto segnale periferico e basso segnale al centro PER AREE piu’ dense di collagene e cellule di schwann è un aspetto di benignità Formae è allagrato e si vede il tumore passare attraverso HYPERINTENSE LESION WITH TARGET SIGN

Radiological features T1-w post-contrast: Intense c.e. (uniform or peripheral) Ottimo dettaglio della compressione del midollom C.E.

Localized, diffuse or plexiform neoplasm of nerve sheath 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

Quando hanno un’apparenza diffusa multinodualre si definiscono plessiformi

HYPOINTENSE SEPTATIONS ISOINTENSE LESION HYPERINTENSE LESION + HYPOINTENSE SEPTATIONS C.E.

DIFFERENTIAL DIAGNOSIS CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY (CIDP) Polineuropatia sensitivo motoria causata da ripeutiti successivi episodi di demilinizzazione e rimeilinizzazione con un aspeto a cipolla del nervo Similissima ma non c’e’ il contesto di NF1 Pathology Hallmarks of CIDP: Enlarged nerves with "onion bulb" formations, demyelination Clinical Issues Mixed sensorimotor neuropathy Symmetric proximal and distal weakness, sensory loss Clinical course: Chronic progressive, step-wise progressive, or recurrent behavior T1WI C+: Mild to moderate nerve enhancement cauda equina

SECOND MOST COMMON INTRADURAL EXTRAMEDULLARY TUMOR 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

Demographic features: General features Age: 50-60 yrs Demographic features: B) Gender: F:M=4:1 (>70-80% female!) BEST DIAGNOSTIC CLUE Enhancing intradural extramedullary mass with “dural tail” LOCATION Thoracic (80%)>> Cervical (16%) > Lumbar (4%) Typical intradural extramedullary Posterolateral (anterior cervical) SIZE Variable MORPHOLOGY Typically round with dural attachment, Ca ++ < 5%

Radiological features ISOINTENSE LESION WITH CALCIFICATION MR Findings Ben circostritti capsulati Originano dal legamento denticolato e generalmente sono sottodurali e possonmo comprimere il midollo T1-w: Isointense Iso- hyperintense Hypointense (calcification) T2-w:

Radiological features 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

DROP METASTASIS Margini irregolari del midollo o noduli lungo le radici le M pilai posso dare enhancement lineare lungo il cord DD infezioni o infiammz tipo sarcoidosi il CSF puo’ esser piu’ iso che ipo Fare RM encefalo Leucemie e linfomi son i preferenziali di parameningeal metastasis

3. EXTRADURAL TUMORS DURA MATER ARACHNOID SPINAL CORD AND PIA EPIDURAL FAT SUBDURAL SPACE SEPTUM POSTICUM DURA MATER ARACHNOID DURAL NERVE ROOT SLEEVE DENTICULATE LIGAMENT SPINAL CORD AND PIA JUNCTION OF DURAL SLEEVE AND SPINAL NERVE PERINEURIUM

Female (breast and lung) Male (prostate and lung) METASTASIS LYMPHOMA Female (breast and lung) Male (prostate and lung) Thoracic (70%)> lumbar (20%) > cervical (10%) Ricordiamo che metastatizzano allo spine oligodendro, gbl, linfoma melanoma e oltre a lung breast e gastric From Thurner et al.

DROP METASTASIS

DIFFERENTIAL DIAGNOSIS ANGIOMIOLIPOMA

DIFFERENTIAL DIAGNOSIS GRANULOMATOSIS

INTRADURAL ARACHNOID CYST EPIDURAL EMATOMA

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