Neuropathology Practice Questions

Q: All of the following are true about medulloblastoma except:

Radiotherapy is the main treatment modality. Medulloblastoma is a highly malignant, Grade 4 embryonal tumor of the cerebellum [1]. The primary treatment strategy is **maximal safe surgical resection**, followed by craniospinal irradiation and chemotherapy (depending on the child's age and risk stratification) [1]. Therefore, **Option D is the correct answer** because surgery, not radiotherapy, is the cornerstone and initial "main" treatment modality. **Analysis of Options:** * **Option A:** Medulloblastoma is the most common malignant brain tumor in children, with a peak incidence between 3–7 years of age [2]. * **Option B:** In children, the tumor typically arises in the **midline (vermis)** of the cerebellum, often projecting into the fourth ventricle. (In adults, it more commonly occurs in the cerebellar hemispheres). * **Option C:** To avoid the devastating neurocognitive side effects of craniospinal radiation on the developing brain, radiotherapy is generally avoided in children **under 3 years of age**. In these cases, intensive chemotherapy (including agents like Vincristine, Cisplatin, or Lomustine/Carmustine) is used to delay or replace radiation. **High-Yield Clinical Pearls for NEET-PG:** * **Histology:** Characterized by small round blue cells and **Homer-Wright rosettes** (pseudorosettes). * **Spread:** Known for "drop metastasis" via CSF to the spinal cord [1]. * **Molecular Subtypes:** WNT (best prognosis), SHH, Group 3 (worst prognosis), and Group 4 [1]. * **Genetics:** Often associated with **Isochromosome 17q**. * **Imaging:** Appears as a hyperdense mass on CT and shows restricted diffusion on MRI due to high cellularity. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1314-1315. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 725-726.

Q: Which tumor is associated with extracranial spread?

Medulloblastoma. **Explanation:** **Medulloblastoma** is the correct answer because it is one of the few primary central nervous system (CNS) tumors known for its potential to metastasize outside the neuraxis. While CNS tumors rarely spread beyond the brain and spinal cord due to the blood-brain barrier and the absence of traditional lymphatic drainage, Medulloblastoma can spread extracranially, most commonly to the **bone (osteoblastic lesions)** and **bone marrow**. This usually occurs via ventriculoperitoneal (VP) shunts or following surgical intervention. **Analysis of Options:** * **Ependymoma:** These typically spread via the cerebrospinal fluid (CSF) "drop metastasis" to the spinal cord but rarely ever leave the CNS. * **Glioblastoma Multiforme (GBM):** Although highly aggressive and locally invasive, GBM almost never spreads outside the cranium. On the rare occasions it does, it usually involves the lungs or cervical lymph nodes, but this is significantly less common than in Medulloblastoma. * **Choroid Plexus Papilloma:** These are generally benign (WHO Grade I) tumors. While they can seed within the ventricular system via CSF, extracranial spread is virtually non-existent. **High-Yield Clinical Pearls for NEET-PG:** * **Homer-Wright Rosettes:** A classic histological finding in Medulloblastoma (also seen in Neuroblastoma). * **Drop Metastasis:** Medulloblastoma frequently spreads through the CSF to the cauda equina; hence, "craniospinal axis radiation" is often required [1]. * **Molecular Subtypes:** WNT (best prognosis), SHH, Group 3 (worst prognosis), and Group 4 [1]. * **Location:** Always arises in the **cerebellum** (vermis in children, hemispheres in adults) [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1314-1315.

Q: Which of the following is NOT true about pilocytic astrocytoma?

It is highly malignant.. **Explanation:** Pilocytic Astrocytoma (PA) is the most common primary brain tumor in children [3]. The correct answer is **Option A** because PA is characteristically a **Grade I (benign)** tumor according to the WHO classification, not highly malignant [3]. It is slow-growing and carries an excellent prognosis with surgical resection. **Analysis of Options:** * **Option A (Correct):** PA is a low-grade, benign tumor [3]. High-grade (malignant) astrocytomas include Anaplastic Astrocytoma (Grade III) and Glioblastoma (Grade IV) [2]. * **Option B (Incorrect):** This is a true statement. The **cerebellum** is the most common site [2], though it can also occur in the optic pathway (optic glioma) and third ventricle. * **Option C (Incorrect):** This is a true statement. Radiologically, PA typically presents as a **"cystic lesion with a mural nodule,"** a classic finding on MRI. **High-Yield NEET-PG Pearls:** 1. **Microscopy:** Look for a "biphasic" pattern consisting of dense fibrillary areas and loose microcystic areas. 2. **Pathognomonic Markers:** * **Rosenthal Fibers:** Thick, eosinophilic, corkscrew-shaped inclusions (found in the fibrillary areas). * **Eosinophilic Granular Bodies (EGBs):** Small proteinaceous droplets. 3. **Genetics:** Strongly associated with **BRAF gene** alterations (KIAA1549-BRAF fusion) and **Neurofibromatosis Type 1 (NF1)** [1]. 4. **IHC:** It is strongly **GFAP positive**, confirming its astrocytic origin. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 724-725. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 725-726. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1319-1320.

Question 1

Which of the following brain tumors does not spread via cerebrospinal fluid?

Accepted Answer

Craniopharyngioma

Answer

Germ cell tumors

Answer

Medulloblastoma

Answer

CNS lymphoma

Question 2

In Alzheimer's disease, which neurotransmitter is deficient in the cortex?

Accepted Answer

Acetylcholine

Answer

Serotonin

Answer

Dopamine

Answer

Noradrenaline

Question 3

What protein is deposited in familial amyloid neuropathy?

Accepted Answer

Mutated transthyretin

Answer

Normal transthyretin

Answer

Mutated beta-2 microglobulin

Answer

Normal beta-2 microglobulin

Question 4

All of the following are true about medulloblastoma except:

Accepted Answer

Radiotherapy is the main treatment modality

Answer

Occur predominantly in children

Answer

Most common site is the vermis

Answer

Carmustine and vincristine are used for recurrences and in children <3 years to avoid radiation therapy

Question 5

Which of the following cell types does not participate in repair after brain infarction?

Accepted Answer

Fibroblasts

Answer

Microglia

Answer

Astrocytes

Answer

Endothelium

Question 6

Which tumor is associated with extracranial spread?

Accepted Answer

Medulloblastoma

Answer

Ependymoma

Answer

Glioblastoma multiforme

Answer

Choroid plexus papilloma

Question 7

Neurofibrillary tangles are characteristic pathological findings in which of the following conditions?

Accepted Answer

Dementia of Alzheimer's Type

Answer

Neurosyphilis

Answer

Shy-Drager syndrome

Answer

Lewy body disease

Question 8

Which of the following is NOT true about pilocytic astrocytoma?

Accepted Answer

It is highly malignant.

Answer

It is usually located in the cerebellum.

Answer

It is often cystic.

Answer

All of the above are true.

Question 9

Medulloblastoma exclusively occurs in which location?

Accepted Answer

Cerebellum

Answer

Medulla

Answer

Cerebral hemisphere

Answer

Spinal cord

Question 10

Hemangioblastomas associated with Von Hippel-Lindau (VHL) disease are most commonly found in which location?

Accepted Answer

Cerebellum

Answer

Liver

Answer

Kidneys

Answer

Pancreas

Neuropathology — MCQs

Neuropathology — MCQs

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