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PNS {patho} (Neurodegenerative Diseases (Alzheimer disease, Parkinson…

- - - - -Usually solitary
        -More in adults
        -Usually benign
        -Anywhere in body (superficial/deep)
      - Cerebellopontine tumor ---->vestibular Schwannoma
        (ACOUSTIC NEUROMA) → Deafness
    - - more difficult to excise
      - Diffuse or nodular ---->in skin
      - Involve axons (mix schwann+neuronal)
    - - very common in the entire body
      - familial predis
      - Encapsulated (peripheral,spinal,cranial) Acoustic Neuroma
      - Excision is easy
  - - - -In children the majority are infratentorial -In adults the majority are supratentorial
      - -20% of CAs in children.
        -1%-2% of all carcinomas -50%-70% are primary tumors
      - can be ^^^
      - Approached by: 1)History
        2) Physical exami
        3) Neurological exami
        4) LP for cytology
        5) CT
        6) MRI
        7) Brain angiography
        8) Biopsy (the last)
    - - cell phones (carcinogenic)
      - radiation
      - familial
        (mostly AD to ts gene inactv)
        
        Tuberous sclerosis
        
        Von Hippel-Lindau (hemangioblastoma, renal carcinoma/cysts)
        
        Neurofibromatosis Type I & Type II (schwannomas///meningioma ///ependymomas)
        
        present with;
        
        Neurofibroma with Sarcomatous transformation.
        
        Glioma of optic nerve,
        
        Pigmented nodules of iris
        
        Café-au-lait spots.
        
        Li-Fraumeni (p53 mutation)
      - Viral & Chemical & Immunosuppression
    - - Gliomas:
        
        Oligodendroglioma (G2/G3If Anaplastic)
        
        Histo
        
        -FRIED-EGG appearance
        -Small uniform cells
        -Minimal mitosis
        
        CCC
        
        -Better prog than astrocytoma at same grade
        -1p/19q co-deletion (imp in dx)
        
        Ependymoma (G2/G3If Anaplastic)
        
        CCC
        
        -children
        -MRI/CT :uniformly enhancing mass
        -can mets (by CSF)
        -can obstructive hydrocephalus
        
        Site:
        -4th ven <20 years
        -Lumbosacral, 3rd ven or lateral ven >20 years
        
        Features:
        
        true rosettes
        
        Myxopapillary
        -loose and mucoid
        -commonly in spinal cord (Lumbosacral r)
        
        Perivascular pseudorosettes
        
        Astrocytoma
        
        CCC
        
        -commonest glioma
        -Mutations: P53, IDH1/2, RB...
        -Positive immunostaining for IDH1 (imp) in identifying low grade.
        -Ki-67 done for all cases .
        -All astrocytomas are GFAP+
        
        -WHO grading :
        1) Nuclear pleomorph
        2) Mitotic
        3) Vascular proliferation
        4) Necrosis
        
        G1 (Pilocytic)
        
        Composed of :bipolar cells, Rosenthal fibers, eosinophilic granular
        
        Site :cerebellum, 3rd ventricles, optic pathways, spinal cord
        
        -(Necrosis and mitosis) are rare - Negative for IDH mutations
        -Mostly in children
        
        G2 (Diffuse)
        
        -Commonest 80%
        -Well-differentiated/low grade
        -fibrillary network
        -more in cerebrum
        
        G3 (Anaplastic)
        
        -Adult tumor
        
        Supratentorial but can occur anywhere in the brain
        
        No **microvascular prolif /palisad necrosis**
        
        More pleomorphic/cellular
        
        G4 (Glioblastoma)
        
        -Microvascular proliferation and palisading necrosis
        -pleomorphic tumor with mitosis
        -Supratentorial with edema
      - Neuronal
        
        Central neurocytoma (low grade intraven)
        
        Ganglioglioma (with seizures/ mostly temporal lobe)
        
        Dysembryoplastic (low grade childhood/ nodular tumor/ with seizures)
      - Embryonal (Primitive)
        
        (G4) Medulloblastoma
        
        CCC
        
        -MYC is a poor prog factor
        -WNTis a favorable prog factor
        
        -20% of pediatric T
        -small cell (blue cell) tumor -subtentorial
        
        Any midline cerebellar or roof of 4th ventricle tumor in a child is a medulloblastoma until proven otherwise.
        
        -located laterally
        -Hydrocephalus & increase in ICP early
        
        Micro
        
        1) small undiffer blue hyperchrom cells
        2) mitoses
        3) Homer-Wright Rosettes
        4) Neurofibrillary
      - Other Parenchymal (Lymphoma///Germ Cell T)
        
        Germ Cell
        
        Germinoma Most comm / 90% cases in first 2 decad / midline in location
        
        Lymphoma
        
        1primary (Most common in imunsup pts / High grade B cell L / multiple peri-ventricular nodular T / Poor resp to chemo)
        
        2nd (mostly spread from peripheral to CNS by meninges)
      - Nerve Sheath (Schwannoma
        //// Neurofibroma)
      - Metastatic
        
        multiple /edema / Broder zone&Grey-white.junc /
        
        Origin
        
        ● Lung (most common)
        
        ● Breast
        
        ● Gastrointestinal
        
        ● Kidney
        
        Leptomeningeal Mets (8%pt / spread by Haematogenous or growth in peripheral nerves )
      - MENINGIOMA
        
        Subtypes
        
        ● Syncytial (most common)
        
        ● Fibroblastic
        
        ● Transitional
        
        ● Psammomatous (Psammoma bodies)
        
        ● Secretory
        
        grades
        
        G1
        
        -Majority / benign
        -higher recurrence rate
        
        G 2-Atypical
        
        (>)mitosis
        -Brain invase
        -Chordoid (>)3features.atypia
        
        G 3 -Malignant
        
        (>>)mitosis
        -Frank sarcomatous
        -rhabdoid/papillary
        -rarely mets out
      - Spinal cord tumors:
        
        ● Extra/intra-spinal ;metastatic, lymphoma
        
        ● Intradural:
        
        − Extramedullary: schwannoma, meningioma
        
        − Intramedullary: ependymoma, astrocytoma