2020 - 12 - 17 - Neoplastic and Paraneoplastic Disorders of the CNS - Καλλιγιάννη

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Καλλιγιάννη Μαριβίκη

Ειδικευόμενη Νευρολογίας ΠΑΓΝΗ


 Glial cells: astrocytoma, oligodendroglioma,
glioblastoma
 Neurons: neurocytomas, gangliomas,
gangliocytomas
 Neural progenitor cells: medulloblastoma,
neuroblastoma
 Meninges: meningioma
 Choroid plexus: papilloma/ carcinoma
 Ependyma: ependymoma
 Pineal gland: pinoblastoma, pineocytoma
 Pituitary: adenoma
 Hematologic system: primary CNS
lymphoma, intravascular lymphoma
 Neoplastic disease can affect NS in several
ways
 Directly due to primary NS tumors or metastases to
NS structures
 Indirectly due to
 Tumors of non-neurologic origin impinging upon NS
structures
 Toxicities of chemo and RT
 Paraneoplastic syndromes
 Depends on the speed of growth and location
 Focal signs if the tumor affects an eloquent
area (e.g. motor, speech, visual, cerebellar)
 Generalized
 Seizures
 Headaches
 Increased intracranial pressure
 Hearing loss/ tinnitus/ imbalance:
cerebellopontine angle tumor
 Bitemporal hemianopia: pituitary tumors/
craniopharyngioma
 Unilateral visual disturbance: optic glioma/
optic nerve sheath meningioma/ olfactory
groove meningioma
 Multiple cranial neuropathies: skull base
lesion/ brainstem lesion/ leptomeningeal
metastases
 Isocitrate Dehydrogenase (IDH1/ IDH2)
 Early event of gliomatogenesis
 Commonly found in lower grade gliomas
 Diffuse gliomas harbouring IDH1/2 ➔ better
prognosis
 BRAF
 1p/19q
 H3Lys27Met
 MGMT
Anaplastic pleomorphic xanthoastrocytoma

Pilocytic astrocytoma
 Neuroectodermal origin
 Molecular and histologic classification

subependymoma ependymoma
 <5% genetic
predisposition
syndromes
 Ionising radiation
(dose-dependent)
 Weak association
 Occupation
(white collar,
farming)
 Head trauma
 NOC exposure
 Metastases
 Infections
 Inflammatory disorders
 Vascular lesions
 Non-Hodgkin lymphoma
 98% B-cell lymphoma
 1%-2% of all primary brain tumors (tends to
increase due to growing AIDS population)
 Peak age 60 (immunocompetent)/ 30
(immunocompromised)
 Highly infiltrative
 Predilection for sites that contact
subarachnoid and ependymal surfaces + deep
gray nuclei
 Secondary CNS lymphoma
 Butterfly GBS
 Demyelination (ADEM/ tumefactive MS)
 Abscess
 Toxoplasmosis
 Neurosarcoidosis
 Metastatic disease
 Meningioma
 15% of all intracranial tumors
 Meningothelial cells
 Falx/ convexity/ petrous ridge/olfactory groove/
sphenoid wing/ parasellar region/ posterior fossa
 Calcification is common
 Primitive neuroectodermal tumor (PNET)
 Medulloblastoma
 Pineoblastoma
 Schwannoma
 Tumors of the sella and parasellar region
 Haematogenous spreading from a primary tumor
to the brain microvasculature
 Mostly from: lung, breast, melanoma, colorectal
cancer, renal cell carcinoma
 Incidence X10 than primary CNS tumors
 20%-30% of adults with systemic cancer will
develop brain metastasis
 Primary CNS tumors (esp. malignant glioma/
lymphoma)
 Abscess
 Infections
 Inflammatory disorders
 Demyelinating diseases
 Vascular lesions
 Primary
 GBM
 Anaplastic astrocytome
 sPNET
 Ependymoma
 Germinoma
 Choroid plexus carcinoma
 Pineoblastoma/ pineocytoma
 Metastatic disease (+/- parenchymal disease)
 Breast
 Lung
 Melanoma
 hematological
 MRI (+Gd)/ DWI/ DTI
 MR perfusion
 MRS
 Systemic cancer screening
 Chest/ abdomen CT
 Surgery
 RT
 Chemotherapy
 Seizure control
 Corticosteroids (symptomatic peritumor
vasogenic edema)
 Venous thromboembolism
 Back pain
 Subacute myelopathy
 Vertebral fracture
 Metastasis more common than primary tumor
 Classified by compartment
 Intramedullary (metastases/ ependymoma/
astrocytoma/ glioblastoma/ lymphoma)
 Extramedullary intradural (metastases/
meningioma/ schwannoma/ neurofibroma)
 Extramedullary extradural (bone metastases/
primary bone tumor)
 Primary tumors (schwannoma/ optic n.
glioma)
 Skull base tumors
 Metastases to the orbit/ cavernous sinus/ skull
base
 Tumors of the facial structures
 Tumors of intracranial structures
 Perineural spread
+Cranial neuropathies (usually VII): eg. BRAF inh
 Headache/ aseptic meningitis
 Encephalopathy
 Seizures
 CNS demyelination
 Vascular event
 Movement disorder/ cerebellar
 Spinal cord syndromes
 Radiation
leukoencephalopathy
 Radiation vasculopathy
 SMART syndrome
(stroke-like migraine
attacks after RT)
 RT-induced CN palsies
 Optic neuropathy
 RT-induced myelopathy
 Plexopathies
 Ischemic stroke
 Hyperoagulability
 Disseminated intravascular coagulation
 Tumor embolism e.g. atrial myxoma

 Intracerebral hemorrhage
 Metastases (most common: melanoma, renal, thyroid)
 Thrombocytopenia
 Coagulopathy (DIC)

 CTV
 Hypercoagulability
 Tumor compression
Thank you for your attention

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