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Neurology - Brain Lesions/Neoplasms (*Peripheral CNS Lymphomas (General…

- - - - *Children Brain Neoplasms
        
        child primary brain tumors are almost all in the brainstem - medulla and cerebllum
        --> medulla/ceerebellum = meduloblastoma
        
        Notes
        
        70% in the posterior fossa (cerebellum area)
        --> think the baby fossa
        
        most in children are intrinsic or primary where adults are usually secondary metastases from else where
        
        Medulloblastoma PND and Glioma - Astrocytomas most common type in children
        
        *Medulloblastoma PNDT
        (Primitive Neuroectodermal Tumors)
        
        very quick
        
        develop hemorragic rossetes around blood vessels
        
        in the cerebellum
        
        Pathophys - Medulloblastoma
        
        most common type in children
        
        come from primitive neuroectodermal cells
        
        develop, grow and spread extremely fast
        --> short history
        
        spread into the brainstem or through the CSF very quickly
        
        5 year survival rate is 50%
        
        Key Features/Presentation - Medulloblastoma
        
        in the cerebellum
        --> this is counter intuitive since it is called medullo-
        
        headache and vomiting in children
        
        develop, grow and spread extremely fast
        --> short history
        
        responds well to chemotherapy and radiation if you catch it quick enough
        
        need to differentiate between Medulloblastoma and Ependymoma since they are both
        
        these form rings around blood vessels like ependyoma rossettes
        
        but medulloblastomas ARE hemorragic
        --> think they BLAST through the blood
        --> ependymal rossettes ARE NOT ---> they are ROSY
        --> key differential factor
        
        Genes/Proteins
        
        WNT and SHH signalling pathways
        
        MYC oncogene and beta catanin (CTNNB1) genes
        --> think of kids watching Mickey mouse = MYC on CTN
        
        MYCC locus oncogene
        --> think Mickey mouse is evil
        --> bad for prognosis
        
        beta catanin (CTNNB1) genes
        --> think beta cat good luck
        --> good for prognosis
        
        one copy of chromosome 17q
      - *Craniopharyngiomas
        
        benign tumor
        
        common lesions in the suprasellar region where the pituitary gland sits
        
        classic presentation with:
        --> hypothalamic and pituitary gland dysfunction
        --> bitemporal hemianopia
        
        Clinical Cases
        
        Clinical Case
        
        Notes:
        
        note that
        
        Clinical Case
        
        *Craniopharyngioma
        
        benign tumour
        
        Case presentation:
        
        Notes:
        
        the pituitary gland sits inside the sella turcica and
        
        the pituitary stalk is held in place by a roof of dura mater over the sella turcica that has a hole in it
        --> diaphragma sellae
        
        Craniopharyngioma present with ant pituitary endocrine dysfunction and bitemporal hemianopia
      - *GLIOMAS
        
        Glioblastomas = by far the most common
        --> most common primary brain tumor in MEN (meningioma ONIONs in WOMEN)
        
        *Astrocytomas
        cystic, solid or semi-solid
        
        General Pathology
        
        common in the cerebellum in kids and in the cerebrum for adults
        
        can be grades 1-4
        --> varies with how bad they are
        
        use ECP for enhancement on images
        
        can be cystic, solid or semi-solid
        --> think of the cyst in a child's cerebellum
        --> these can be very dangerous for children since in the cerebellum can invade the 4th ventricle
        
        Differentials in kids
        
        these are in cerebellum for kids like medulloblastomas
        
        but they are usually cystic, soli or semi-solid
        
        *Oligodendrogliomas
        
        think Oligo to the Front
        
        Oligo LOH
        
        think Oligos everywhere --> OLIGO multiple calcified
        
        General Pathology
        
        come from oligodendrocytes
        --> that make CNS myelin
        
        5 year survival rate is 76%
        
        Key Features
        
        frontal lobe usually
        
        usually grade 2
        
        slow growing and well defined
        
        get seizures and headache
        
        multiple foci of calcification on CT imaging
        --> this is not definite that it is oligo
        --> could be other type, but is common here
        --> look like mexican hats around the oligodendrocytes of the myelin
        
        loss of Heterozygosity LOH in 1p and 19q
        
        multiple foci of calcification on CT imaging
        
        1p/19q oligodendrocyte pattern
        
        should have 2 of each of these in each cell
        
        2 1p and 2 19q
        if there is LOH of this 2 and 2 then most likely oligodendroglioma
        
        *Glioblastomas
        
        most common primary adult brain cancer
        
        cancer of astrocytes
        
        are very large, can casue midline shift
        
        "butterfly glioblastoma" = when a glioblastoma is very large and crosses the corpus callosum
        
        microvascular endothelial large growth
        
        palicading necrosis areas
        
        Prognosis and WHO Grading
        
        ALWAYS Grade 4 Very Malignant
        --> must have a large number of microvascular endothelial proliferation
        --> must have palicading necrosis present (this means the palicading cells surround the necrosis)
        
        usually last less than 10 months
        
        Key features - Glioblastomas
        
        normally see ring enhancement on images
        
        *Butterfly Glioma
        
        primary brian tumor in men almost always are glioblastomas
        
        tumour crosses the midline via the corpus callosum
        
        Other white matter commissures are also occasionally involved.
        
        Pathophys - Glioblastoma
        
        note these come from astrocytes but are the worst kind so they get called glio-
        
        from astrocytes
        
        same tumor that John McCain had and just died from
        
        Gliobblastomas have the worst prognosis and are the most malignant
        
        EGFR mutation = epidermal growth factor receptor
        --> note that EGFR oncogene mutation is also seen in SC Lung cancer, breast and prostate cancers
        --> even though glioblastoma is not METs
        
        EGFR mutation
        
        EGFR = epidermal growth factor receptor
        --> note that EGFR oncogene mutation is also seen in SC Lung cancer, breast and prostate cancers
        --> even though glioblastoma is not METs
      - *Extrinsic Brain Neoplasms
        (Meninges and Nerve Roots)
        
        *Meningiomas
        
        "Meningiomas MOMMYs make MANY ONIONS"
        
        Calcospherites ‘’psammoma bodies’’ = onion skins
        
        "Meningiomas MOMMYs sinuses CRY when they make MANY ONIONS"
        
        "MOMMYs " = 5 times more common in WOMEN in
        --> 2nd most common primary brain tumor behind gliobastomas
        --> thus primary brain tumor (MEN = glioblastoma; Women = Meningioma)
        
        "sinuses CRY " = women cutting onions make them cry = meningiomas
        --> SINUS = venous sinuses (superior sagital sinus) are the most common site for meningiomas
        
        Calcospherites ‘’psammoma bodies’’ = onion skins
        
        Pathophys of Meningiomas
        
        5 times more common in women
        
        note these are the 2nd most common tumor in adults after gliomas
        --> since they affect women so much
        
        when you see women --> think meingioma
        
        usually right next to the saggital sinus (superior), foreman magnum or the olfactory bulbs
        - Calcospherites ‘’psammoma bodies’’ = onion skins
        --> think meningiomas are common in women or mommys
        --> psammoma bodies that are calcified spheres
        
        Prognosis of Meningiomas
        
        graded 1-3
        
        grade 1 very easy to remove as superficial
        
        grade 2 usually recurs or goes to grade 3
        
        grade 3 can kill
        
        usually don't infiltrate the brain since are on the dura and can't get in
        
        Imaging of meningioma
        
        *Schwannomas (Acoustic)
        
        common lesions in the cerebellopontine Angle
        --> called acoustic Swchannomas
        
        USUALLY very BENIGN
        
        grow out of CN 8 = vestibulocochlear
        
        CN 7 = facial nerve usually involved
        --> CN 7 runs right alongside the CN 8 so is usually involved
        
        CN 5 = trigeminal nerve not sually, but can be involved
        --> CN 5 is in the top corner of the cerebellopontine angle/triangle so further away, but next clostest nerve
        
        *cerebellopontine Angle
        
        "Bethoven's VON SCHWAN SONG"
        
        VON RH cafe au lait axilla freckles leads to
        
        Schwannoma in the CN8
        
        NF1 and NF2 =
        (Von Recklinghausens)
        Neurofibromatosis
        
        50% are from random spontaneous mutations
        --> may not be a family Hx
        
        LEADS TO SCHWANOMAS
        
        2 more items...
        
        *Pathophys of Schwannomas
        
        slow growing and benign
        
        most common site is the CN8 = vestibulocochlear auditory nerve
        
        leads to deafness
        --> thought that Bethoven had 2 separate Schwanomas in each CN8
        --> went deaf
        
        mix of compact cells and cystic areas
        
        very distinctive blood vessels
        
        need to check if patient had Von Recklinghausens Neurofibromatosis
        --> Von RH leads to Schwannomas
      - *Ependymomas
        
        think at Ependymal tumor at the END
        of the of the ventricle system = 4th ventricle
        
        General Pathology
        
        most common neoplasm that comes from the spinal cord
        
        ALMOST ALWAYS in the 4th ventricle
        
        makes sense as it is ependymoma
        --> from ependymal cells
        
        can obstruct the ventricle
        
        would present with raised ICP probably
        
        ependymomas tend to grow around blood vessels and form ependymoma rossettes
        
        these ependymal rossettes are NOT hemorragic like medulloblastomas are
        --> key differential factor
        
        ependymoma rossettes
- - - - Clinical Cases
        
        Clinical Case
        
        Clinical Case
        
        Notes:
        
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- - - - Clinical Case
        
        Clinical Case
        
        Notes:
        
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