PATOLOGIE TUMORALI ENDOCANALARI Dott.ssa Anna Pichiecchio U.O. Neuroradiologia Istituto Neurologico IRCCS Fondazione C. Mondino, Pavia SPINAL TUMORS Uncommon lesions Classification based on their location EXTRADURAL INTRAMEDULLARY (60% of all spinal tumors) (5%-10% of all spinal tumors) Metastasis Extension of primary bone tumors INTRADURALEXTRAMEDULLARY (30% of all spinal tumors) Meningiomas and neurinoma Leptomeningeal dissemination gliomas 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 contrast 541 15 225 304X212 1. INTRAMEDULLARY TUMORS DURA MATER ARACHNOID DURAL NERVE ROOT SLEEVE DENTICULATE LIGAMENT SPINAL CORD AND PIA EPIDURAL FAT SUBDURAL SPACE JUNCTION OF DURAL SLEEVE AND SPINAL NERVE PERINEURIUM EPIDURAL FAT SEPTUM POSTICUM 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 •20% intraspinal neoplasms •60% primary spinal cord tumors 80-90% low grade Pilocitic Fibrillary subarachnoid dissemination may occur! Association with abnormalities of chromosome 17p (TP53 gene) General features A) Age: two peaks: 10 - 30 ys Demographic features: BEST DIAGNOSTIC CLUE LOCATION SIZE B) Gender: M > F (1,3:1); no gender difference in children! Enhancing infiltrating cord mass in child No capsule or cleavage Thoracic >Cervical > Lumbar Usually 1-3 cm, less than 4 segments MORPHOLOGY fusiform expansion of cord, eccentric , posterior Risk in patients with Neurofibromatosis (NF1 and NF2) 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 MR Findings T2-w: a) Hyperintense HYPERINTENSE LESION b) Not as hyperintense if cysts/syrinx T2* GRE: a) Rarely hemorrhagic CYSTS From Rossi et al 2007 RARELY HEMORRAGE CYSTS 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 ARNOLD-CHIARI I MALFORMATION SYRINGOMYELIA 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 •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 General features A) Age: 40 yrs Demographic features: B) Gender: M:F=3:1 C) Ethnicity: non racial predilection BEST DIAGNOSTIC CLUE LOCATION SIZE Circumscribed, enhancing cord mass with hemorrhage Conus> Cervico-Thoracic Multisegmental (typically 3-4 segments) Well-circumscribed, symmetric cord expansion, MORPHOLOGY occasional exophytic component Radiological features MR Findings T1-w: a) Iso- or slightly hypointense to spinal cord b) Hemorrhage ISOHYPOINTENSE LESION Radiological features MR Findings T2-w: a)Hyperintense b) Polar (rostral or caudal) or intratumoral cysts (50-90%) HEMOSIDERIN “CAP SIGN” Hemosiderin “Cap sign” c) Syrinx d) Focal hypointensity e) Surrounding cord oedema Radiological features HEMOSIDERIN “CAP SIGN” MR Findings STIR: a) Hyperintense T1-w post-contrast: a) Intense,welldelineated homogeneous c.e. (50%) b) Nodular, peripheral, heterogeneous c.e. c) Minimal or no c.e. rare C.E. ASTROCYTOMA EPENDYMOMA Astrocytoma Ependymoma cervical Eccentric posterior - conus central hemorragic hyercellularity Pseudocapsule (plane) Infiltrative (poor borders) 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: a) Usually Isointense with cord b) Hyperintensity (accumulation of mucin!) T2-w: a) Hyperintense b) Hypointensity at tumor margins (hemosiderin) STIR: a) Hyperintense T1-w post-contrast: a) 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 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 General features Demographic features: BEST DIAGNOSTIC CLUE LOCATION SIZE A) Age: mean age at presentation 30 ys B) Gender: M = F Intramedullary mass with serpentine “flow voids” Thoracic > Cervical Subpial (posterior, rarely anterior) often associated with intraspinal cysts Few mm to several cm MORPHOLOGY Round, well-defined margins Radiological features MR Findings T1-w: Small: a) Isointense b) Cyst!! (> 50%) Large: a) hypoisointense b) “flow voids” (lesion > 2,5 cm) ISOHYPOINTENSE LESION + SYRINX Radiological features MR Findings T2-w: a) Uniformly hyperintense (small lesions) mixed hyperintense (hemorrhage common) a) +/- peritumoral oedema b) Syrinx fluid HYPERINTENSE LESION + CYST Radiological features MR Findings T1-w post contrast: Cyst/ Syrinx: Well-demarcated, no c.e. a) Subpial nodule intense, homogeneous C.E. (often on surface of dorsal cord) 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 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 Venous hypertension 2. INTRADURAL EXTRAMEDULLARY TUMORS DURA MATER ARACHNOID DURAL NERVE ROOT SLEEVE DENTICULATE LIGAMENT SPINAL CORD AND PIA EPIDURAL FAT SUBDURAL SPACE JUNCTION OF DURAL SLEEVE AND SPINAL NERVE PERINEURIUM EPIDURAL FAT SEPTUM POSTICUM Schwannoma, Neurinoma Arise from single nerve, from one side Typically dorsal root Schwann cells Do NOT envelope the adiacent nerve root Lobulated Single Neurofibroma Schwann cells and fibroblasts between dispersed nerve fibers, mps matrix Spreads apart axons DO envelope the adiacent nerve root Fusiform Multiple 2.a. SCHWANNOMA Neoplasm of nerve sheath in PNS Most common WHO grade I intradural extramedullary mass 30% primary spinal tumors •Mostly sporadic (inactivation of NF2 gene) • Association with NF2, bilateral multiple 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 Thoracic > Cervical = Lumbar LOCATION 70-75% intradural extramedullary 15% completely extradural 15% “dumbbell” (both intra- and extradural) SIZE Few mm Giant schwannoma: > 2 vertebral segments MORPHOLOGY Round, lobulated Radiological features T1-w: Isointense ISOINTENSE LESION T2-w: a) hyperintense b) 45%cyst 10% hemorrage c) “Target sign” HYPERINTENSE LESION WITH TARGET SIGN Radiological features T1-w post-contrast: a) Intense c.e. (uniform or peripheral) C.E. 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 Origin from arachnoid Attached to the dura mater for infiltration SECOND MOST COMMON INTRADURAL EXTRAMEDULLARY TUMOR > 25% WHO I Meningothelial Fibroblastic Psammomatous angiomatous Genetic predisposition in women! •Almost all have chromosome 22 abnormalities •Association with neurofibromatosis 2 (NF2) Solitary General features Demographic features: A) Age: 50-60 yrs B) Gender: F:M=4:1 (>70-80% female!) BEST DIAGNOSTIC CLUE LOCATION SIZE Enhancing intradural extramedullary mass with “dural tail” Thoracic (80%)>> Cervical (16%) > Lumbar (4%) Typical intradural extramedullary Posterolateral (anterior cervical) Variable MORPHOLOGY Typically round with dural attachment, Ca ++ < 5% Radiological features ISOINTENSE LESION WITH CALCIFICATION MR Findings T1-w: a) Isointense T2-w: b) Iso- hyperintense c) Hypointense (calcification) Radiological features C.E. T1-w post-contrast: a) Prominent c.e. b) 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 3. EXTRADURAL TUMORS DURA MATER ARACHNOID DURAL NERVE ROOT SLEEVE DENTICULATE LIGAMENT SPINAL CORD AND PIA EPIDURAL FAT SUBDURAL SPACE JUNCTION OF DURAL SLEEVE AND SPINAL NERVE PERINEURIUM EPIDURAL FAT SEPTUM POSTICUM METASTASIS LYMPHOMA Female (breast and lung) Male (prostate and lung) Thoracic (70%)> lumbar (20%) > cervical (10%) From Thurner et al. DROP METASTASIS DIFFERENTIAL DIAGNOSIS ANGIOMIOLIPOMA DIFFERENTIAL DIAGNOSIS GRANULOMATOSIS INTRADURAL ARACHNOID CYST EPIDURAL EMATOMA Grazie per l’attenzione