Handout for the lectures by James G. Smirniotopoulos, M.D.,
The 29th Annual International Diagnostic Course in Davos, Switzerland
April 6 - 11, 1997

WATCH for the Next Neuroradiology Course in DAVOS, April 2000


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SPINAL MASSES:INTRADURAL LESIONS

SPINAL MASSES Intramedullary -masses intrinsic to the cord Intradural/Extramedullary -masses in the subarachnoid space Extradural -masses outside the thecal sac

LOCALIZATION

SPINAL MASSES Primary Neoplasms Secondary Neoplasms Congenital Traumatic/Degenerative Infectious/Inflammatory Vascular/Ischemic

INTRASPINAL NEOPLASMS WOMEN 5/million/yr MEN 3/million/yr

INTRASPINAL NEOPLASMS INTRAMEDULLARY Expanded Cord effaces the CSF Fusiform (often) Long Segment (often) Tapered (usually)

EXPANDED CORD Primary & Secondary Neoplasms Myelitis, Abscess Granuloma Acute MS Syringohydromyelia (primary/secondary) Infarct, Edema

INTRAMEDULLARY Primary Neoplasms Ependymoma Astrocytoma other Gliomas (incl. GBM, Oligo, etc.) Hemangioblastoma Paraganglioma

EPENDYMOMA Most Common Primary Neoplasm of Cord Two "flavors" - solid and myxopapillary 60% - 70% of all Cord, 90% of CAUDA EQUINA MIDDLE AGE (40'S) YOUNGER for Cauda Equina (20'S) and Cervical spine Hemorrhage/hemosiderin watch for SAH (mimic ANEURYSM) "Cap Sign" dark rim top/bottom of lesion Enhance Heterogenously +/- tumor cyst and/or syringohydromyelia Surgical cure more likely than for astrocytoma

NEUROLOGIC NEOPLASMS EXPANDED CORD Primary & Secondary Neoplasms Myelitis, Abscess Granuloma Acute MS Syringohydromyelia(primary/secondary) Infarct, Edema Hematoma, Contusion, Edema

SYRINGOHYDROMYELIA GR. "SYRINX' a pipe or straw (hollow reed) A Fluid-Filled Cavity within the Cord usually central Hydromyelia = Ependymal-lined (An Enlarged Central Canal) often the Ependyma is destroyed Loss of Pain&Temperature Fibers (Quien es mas macho?)

SYRINGOHYDROMYELIA Idiopathic (Congenital?)do not enhance, smooth Associated w/Chiari Malformations - Chiari 1 (30%) Chiari 2 (60%) (Myelomeningocele) Associated w/Neoplasms

INTRAMEDULLARY Primary Neoplasms Ependymoma Astrocytoma other Gliomas (incl. GBM, Oligo, etc.) Hemangioblastoma Paraganglioma

ASTROCYTOMA 2nd Most Common (30%) Children, Young Adult Unlike Brain, Low grade most common Unlike Brain, Enhance even if Low grade Cervical Cord > Thoracic,Lumbar

INTRAMEDULLARY Primary Neoplasms Ependymoma Astrocytoma other Gliomas (incl. GBM, Oligo, etc.) HemangioblastomaParaganglioma

HEMANGIOBLASTOMA Synonyms: H...endothelioma, Angioblastic meningioma, Lindau Tumor Cell of Origin: Vascular, Endothelial? Associations: von Hippel-Lindau (20%), (multiple in 5%, w/o VHL) Incidence: 1-3% of ALL Intracranial Age: 30-45 (peak 33/35) Sex: 1-2M/1F Location: Subpial CRBLL > Medulla > Spinal Treatment: Surgery, Radiation Prognosis: 92% at 5yrs. (small series)

HEMANGIOBLASTOMA 1-15% of Cord Tumors 85-90% are Intramedullary 10-15% occur in Nerve Roots 50% Thoracic, 15% Cervical 20% Multiple 1/3 of pts. will have VHL Suggestive for Hemangioblastoma cyst (rimmed by enhancement) flow voids (hypervascular) signs of hemorrhage

INTRADURAL- EXTRAMEDULLARY Meningioma Nerve Sheath Tumors: - Schwannoma - Neurofibroma Epidermoid, Lipoma Drop Mets (intracranial primary) Granulomatous, Arachnoiditis Paraganglioma (filum, cauda equina)

INTRADURAL- EXTRAMEDULLARY Meningioma Nerve Sheath Tumors: - Schwannoma - Neurofibroma Epidermoid, Lipoma Drop Mets (intracranial primary) Granulomatous, Arachnoiditis Paraganglioma (filum, cauda equina)

SPINAL MENINGIOMA 1/4 -1/3 OF Spinal Neoplasms F > M (4 : 1, up to 8:1) Arise from Arachnoid Cells (NOT dura) Psammomatous Type Common look for Calcification on CT, XR Hyperdense, Dural Tail? Occasionally (5%) Dumbbell 4/5 Thoracic Spine (most arachnoid)

INTRADURAL- EXTRAMEDULLARY Meningioma Nerve Sheath Tumors: - Schwannoma - Neurofibroma Epidermoid, Lipoma Drop Mets (intracranial primary) Granulomatous, Arachnoiditis Paraganglioma (filum, cauda equina)

NERVE SHEATH TUMORS NERVE SHEATH TUMORS: DISTRIBUTION Intracranial - Schwannoma (sporadic >> NF-2) Spinal - Both (S > N) Dumbell - Both (N > S) PNS - Both Cutaneous - Neurofibroma (NF-1 >> sporadic)

INTRASPINAL NEOPLASMS SCHWANNOMA Benign, Encapsulated 10-20% of Spinal masses F=M (slight Female?) Sporadic >> NF2 Attached to (but may separate from) Nerve Ca++ and Hyperdensity Rare Difficult DDX from Neurofibroma Dorsal, Sensory Root Dumbbell Shape (less than neurofibroma) Multiple in NF2

NEUROFIBROMA Benign, not Encapsulated NF1 >> Sporadic Fusiform, Incorporates Nerve Dumbbell configuration is common extends through the neural foramen Often Multiple (because most likely w/NF1) Look for other signs of NF-1 Scoliosis extraspinal masses

NEUROFIBROMATOSIS NF-1, von Recklinghausen ("peripheral") NF-2, Bilateral Acoustic - MISME ("central") NF-3, Overlap of 1 and 2 NF-5, Segmental/Localized NF-1 (e.g. a quadrant) NF-6, Cafe-au-lait only (w/o CNS/PNS) NF-7, Late Onset (usu w/o CLS, Lisch, etc.) NF-8, Other (N.O.S.)

NEUROFIBROMATOSES Neurofibromatosis Type 1 (NF-1) - von Recklinghausen's Disease - "True" Neurofibromatosis- Prominent Cutaneous Signs - Chromosome 17q Neurofibromatosis Type 2 (NF-2) - Bilateral Acoustic Schwannoma - "Central Neurofibromatosis" - Minimal Skin Manifestations - Chromosome 22q

NEUROFIBROMATOSES NEUROFIBROMATOSIS TYPE 1 (NF-1) -von Recklinghausen Disease -"Peripheral" Neurofibromatosis -Prominent cutaneous signs -Multiple Neurofibromas NEUROFIBROMATOSIS TYPE 2 (NF-2) -Bilateral Acoustic Schwannoma -"Central Neurofibromatosis" -Minimal Skin Manifestations -Multiple Schwannomas/Meningiomas and Ependymomas

NEUROFIBROMATOSES NEUROFIBROMATOSIS 1 Lesions of Astrocytic/Neuronal Origin - glioma (astrocytoma, pilocytic optic nerve > GBM) - hamartoma - heterotopia Neurofibromas Macular Hyperpigmentation (cafe-au-lait spots) NEUROFIBROMATOSIS 2 Lesions of Covering/Lining of CNS - schwannoma (multiple) - meningioma (multiple) - ependymoma (usually cord >> brain) Epidermal - cutaneous "skin tags"

NEUROFIBROMATOSIS - 1 Incidence: 1/2,500 births Inheritance: Autosomal Dominant Age at Presentation: Birth to Death Sx at Presentation: Spots, NFBA Diagnostic Criteria: Cutaneous, PNS Chromosome Abnl.: 17 Ocular Findings: Myelinated retina Cutaneous Findings: cafe-au-lait, neurofibroma CNS Findings: Optic N. Glioma Hamartoma Heterotopia macrocephaly mentation problems (e.g. learning disability)

NEUROFIBROMATOSIS - 1&2

NEUROFIBROMATOSIS TYPE-2 (the MISME syndrome) (Bilateral Vestibular Schwannoma) M - Multiple I - Inherited S - Schwannomas M - Meningiomas E - and Ependymomas

INTRAMEDULLARY Primary Neoplasms Ependymoma Astrocytoma other Gliomas (incl. GBM, Oligo, etc.) Hemangioblastoma Paraganglioma

SPINAL PARAGANGLIOMA Cauda Equina, Conus Hypervascular (flow voids) Signs of Hemorrhage(hemosiderin, methemoglobin)

LOCALIZATION EXPANDED CORD Primary & Secondary Neoplasms Myelitis, Abscess Granuloma Acute MS Syringohydromyelia(primary/secondary) Infarct, Edema Hematoma, Contusion, Edema

NEUROLOGIC NEOPLASMS INTRADURAL- EXTRAMEDULLARY Meningioma Nerve Sheath Tumors: - Schwannoma - Neurofibroma Epidermoid, Lipoma Drop Mets (intracranial primary) Granulomatous, Arachnoiditis Paraganglioma (filum, cauda equina)

NERVE SHEATH TUMORS Schwannoma (Sporadic >> NF-2 >> NF-1) - focal mass, sensory root- cranial and spinal Neurofibroma (usually NF-1 if spinal) - focal mass, dumb-bell lesion Plexiform Neurofibroma (usually NF-1) - diffuse or fusiform enlargement Malignant N. S. Tumor (NF-1 or Sporadic)

END OF HANDOUT