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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).

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PATOLOGIE TUMORALI ENDOCANALARI

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

  2. 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

  3. MRI gold standard!

  4. 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

  5. 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

  6. 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)

  7. 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)

  8. 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

  9. 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

  10. RARELY HEMORRAGE CYSTS

  11. T1-w post-contrast: a) enhancement MODERATE C.E. ALWAYS!! From Rossi et al 2007

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

  13. ARNOLD-CHIARI I MALFORMATION SYRINGOMYELIA

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

  15. 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

  16. Generalfeatures A) Age: 40 yrs Demographic features: B) Gender: M:F=3:1 C) Ethnicity: non racial predilection

  17. Radiologicalfeatures MR Findings ISO- HYPOINTENSE LESION T1-w: • Iso- or slightly hypointense to spinal cord • Hemorrhage

  18. 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

  19. 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

  20. ASTROCYTOMA EPENDYMOMA

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

  22. 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

  23. INTENSE C.E. From Rossi et al 2007

  24. DIFFERENTIAL DIAGNOSIS ARTERO-VENOUS FISTOLA

  25. 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

  26. 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

  27. Generalfeatures A) Age: mean age at presentation 30 ys Demographic features: B) Gender: M = F

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

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

  30. 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

  31. 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

  32. Ependymoma metastasis mass centrally located Astrocytoma Crowley, Neurosurgery 2008 not hypervascular; peritumoral oedema common Renal cell carcinoma

  33. 1)they tend to enlarge the cord either focally or diffusely 2) on T2-w they produce high signal intensity 3) They enhance

  34. Symptoms include pain, weakness and muscle atrophy

  35. 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

  36. 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

  37. 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

  38. Generalfeatures A) Age: 40 ys Demographic features: B) Gender: M = F C) Ethnicity: no racial predilection

  39. Radiologicalfeatures T1-w: Isointense ISOINTENSE LESION T2-w: hyperintense 45%cyst 10% hemorrage “Target sign” HYPERINTENSE LESION WITH TARGET SIGN

  40. Radiologicalfeatures T1-w post-contrast: • Intense c.e. • (uniform or peripheral) C.E.

  41. 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

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

  43. DIFFERENTIAL DIAGNOSIS CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY (CIDP)

  44. 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

  45. Generalfeatures • Age: 50-60 yrs Demographic features: B) Gender: F:M=4:1 (>70-80% female!)

  46. Radiologicalfeatures ISOINTENSE LESION WITH CALCIFICATION MR Findings T1-w: • Isointense • Iso- hyperintense • Hypointense (calcification) T2-w:

  47. Radiologicalfeatures C.E. T1-w post-contrast: • Prominent c.e. • May see enhancing “dural tail”

  48. 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

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