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lecture :
Brain Tumors
## Brain Tumors Classification

1. **Gliomas**: Tumors originating from glial cells.
   - **Astrocytoma**
   - **Oligodendroglioma**
   - **Ependymoma**
2. **Choroid Plexus Tumors**
3. **Neuronal/Glioneuronal Tumors**: Tumors with neuronal elements.
   - **Gangliogliomas**
   - **Dysembryoplastic neuroepithelial tumors**
4. **Embryonal Tumors**: 
   - **Medulloblastoma**
5. **Meningeal Tumors**
6. **Familial Tumor Syndromes**: Often involve tumors associated with genetic syndromes, such as:
   - **Neurofibromatoses**
   - **Tuberous Sclerosis Complex**
   - **Von Hippel-Lindau Disease**
7. **Metastatic Brain Tumors**:
   - Common primary sites: **lung, breast, skin (melanoma), kidney, gastrointestinal tract**.

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## Peripheral Nerve Damage Patterns

1. **Demyelinating Neuropathy**:
   - Damage targets **Schwann cells** and **myelin sheaths** but **preserves axons**.

2. **Acute Axonal Injury**:
   - Involves degeneration of **distal axons** and their myelin sheaths, leading to **atrophy of affected muscle fibers**.

3. **Regeneration Patterns**:
   - **Axonal Regeneration**: New axons are remyelinated by **Schwann cells**, but internodes are shorter, and myelin is thinner.
   - **Remyelination in Demyelinating Disease**: New internodes are also shorter and thinner.

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## Classification of Peripheral Nerve Tumors

1. **Schwannomas**:
   - Benign tumors originating from Schwann cells.
   - Often part of **Neurofibromatosis type 2 (NF2)** due to mutations in the **NF2 gene** (merlin).
   - Histological features: encapsulated, may show **nuclear palisading** (Antoni A and Antoni B patterns) and **Verocay bodies**.

2. **Neurofibromas**:
   - Benign, heterogeneous tumors containing **Schwann cells**, **perineurial cells**, **fibroblasts**, **mast cells**, and **CD34+ spindle cells**.
   - Associated with **Neurofibromatosis type 1 (NF1)**.
   - Growth patterns:
     - **Superficial cutaneous neurofibromas** (often pedunculated).
     - **Diffuse neurofibromas** (plaque-like).
     - **Plexiform neurofibromas** (associated with nerve roots, typically NF1-associated).

3. **Malignant Peripheral Nerve Sheath Tumors (MPNSTs)**:
   - High-grade tumors, often arising in NF1 patients as a transformation of plexiform neurofibromas.
   - Sporadic cases can also occur.

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## Key Differences: Schwannomas vs. Neurofibromas

| Feature                          | Schwannoma                                           | Neurofibroma                                 |
|----------------------------------|------------------------------------------------------|----------------------------------------------|
| Association                      | Often NF2, not NF1                                   | Typically NF1-associated                     |
| Capsule                          | Usually encapsulated                                 | Encased within the nerve                     |
| Cellularity                      | Biphasic (Antoni A and B areas)                      | Uniphasic, low to moderate cellularity       |
| Nuclear Palisading               | Present                                             | Absent                                       |
| Growth Pattern                   | Eccentric mass around nerve                          | Fusiform, involving whole nerve              |

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## Familial Tumor Syndromes

1. **Neurofibromatosis Type 1 (NF1)**:
   - **Autosomal dominant** disorder (1 in 3000).
   - Associated tumors:
     - Neurofibromas (all types)
     - MPNSTs
     - **Optic gliomas**, other glial tumors
     - **Hamartomas**
     - **Pheochromocytomas**

2. **Neurofibromatosis Type 2 (NF2)**:
   - **Less common** than NF1.
   - Characterized by:
     - Bilateral **eighth nerve schwannomas**
     - **Multiple meningiomas**
     - **Spinal ependymomas**

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This summary covers all essential information about brain and peripheral nerve tumors, demyelinating neuropathy, and familial tumor syndromes. Let me know if there are specific details you’d like to explore further!