CNS Tumors — MCQs
What is not seen in tuberous sclerosis?
Which of the following statements about meningiomas is true?
Which of the following is the most frequent primary malignant tumor of the CNS?
A patient presents with headache, confusion, and a diagnosis of a brain tumor. The family history reveals brain and kidney tumors. What is the most likely diagnosis?
Which one of the following is the most common CNS tumor associated with type I neurofibromatosis?
Most common tumor in the part of the brain shown (arrow) among children is
Most common site for medulloblastoma is-
Most common supratentorial tumor in children
Which of the following CNS tumor shows increased growth during pregnancy?
CNS tumor seen in Von Hippel Lindau syndrome is:
CNS Tumors Indian Medical PG Practice Questions and MCQs
Question 1: What is not seen in tuberous sclerosis?
- A. Giant Cell Astrocytoma
- B. White matter migration lines
- C. Sub-ependymal nodules
- D. Ependymoma (Correct Answer)
Explanation: ***Ependymoma*** - **Ependymomas** are primary central nervous system tumors that arise from **ependymal cells**, typically found in the ventricles and spinal cord. - While they are brain tumors, they are **not characteristic lesions** associated with **Tuberous Sclerosis Complex (TSC)**. *Giant Cell Astrocytoma* - **Subependymal giant cell astrocytomas (SEGAs)** are a classic feature of **Tuberous Sclerosis Complex (TSC)**, often growing within the ventricles. - They can cause **hydrocephalus** and are a significant source of morbidity in TSC patients. *White matter migration lines* - **Radial glial migration lines**, or **cerebral white matter lesions**, are due to abnormalities in neuronal migration during development and are a common finding in **Tuberous Sclerosis Complex (TSC)**. - These are typically seen as **linear hypdensities** on neuroimaging. *Sub-ependymal nodules* - **Subependymal nodules (SENs)** are small, benign lesions located just beneath the ependyma of the lateral ventricles in **Tuberous Sclerosis Complex (TSC)**. - They are a diagnostic hallmark of TSC and can sometimes develop into **Subependymal Giant Cell Astrocytomas (SEGAs)**.
Question 2: Which of the following statements about meningiomas is true?
- A. Approximately 5% of meningiomas are malignant.
- B. Arise from the dural layer
- C. Meningiomas are more common in women due to hormonal influences.
- D. 95% cure rate following total surgical resection of benign meningiomas (Correct Answer)
Explanation: ***95% cure rate following treatment*** - Meningiomas generally have a **high cure rate of approximately 95%** following surgical resection, especially when they are completely excised [1]. - They are typically **benign tumors**, resulting in favorable outcomes with appropriate management [1]. *Arise from arachnoid layer* - Meningiomas actually arise from **meningothelial cells** of the **arachnoid layer**, but this statement does not fully explain their pathogenesis. - This mischaracterization does not provide an accurate understanding of the tumor's origin and biology. *50% are malignant* - Most meningiomas are benign; only a small percentage, about **1-5%**, are classified as malignant. - Thus, stating that **50% are malignant** significantly overestimates the incidence of aggressive forms. *More common in men* - Meningiomas are more prevalent in **women**, especially those aged between 30-70 years, with a female-to-male ratio of approximately **3:1**. - This option is incorrect as it misrepresents the demographic distribution of the disease. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1316-1317.
Question 3: Which of the following is the most frequent primary malignant tumor of the CNS?
- A. Glioblastoma multiforme (Correct Answer)
- B. Oligodendroglioma
- C. Medulloblastoma
- D. Meningioma
Explanation: ***Glioblastoma multiforme*** - **Glioblastoma multiforme (GBM)** is the most common and aggressive primary malignant brain tumor in adults [1]. - It is a **grade IV astrocytoma**, characterized by rapid growth, necrosis, and microvascular proliferation [1]. *Oligodendroglioma* - **Oligodendrogliomas** are primary glial tumors but are less common than GBM. - They typically have a more indolent course than GBM and are often characterized by **IDH mutations** and **1p/19q co-deletion**. *Medulloblastoma* - **Medulloblastoma** is the most common malignant brain tumor in children, but it is rare in adults [2]. - It arises in the **cerebellum** and is a type of embryonal tumor [2]. *Meningioma* - **Meningiomas** are the most common primary brain tumors overall, but they are typically **benign** and originate from the meninges. - While they can be symptomatic due to compression, they are not primarily malignant in the way GBM is. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1308-1310. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 725-726.
Question 4: A patient presents with headache, confusion, and a diagnosis of a brain tumor. The family history reveals brain and kidney tumors. What is the most likely diagnosis?
- A. Neurofibromatosis
- B. Li-Fraumeni syndrome
- C. VHL syndrome (Correct Answer)
- D. Churg-Strauss syndrome
Explanation: ***VHL syndrome*** - **Von Hippel-Lindau (VHL) syndrome** is an inherited disorder characterized by the growth of tumors and cysts in various parts of the body, including the **brain (hemangioblastomas)** and **kidneys (renal cell carcinoma)**. - The presentation of a brain tumor, kidney tumors, and a positive family history for both organs strongly points to VHL syndrome. *Neurofibromatosis* - **Neurofibromatosis (NF)** typically presents with **cafe-au-lait spots**, neurofibromas, optic gliomas, and Lisch nodules. - While it involves brain tumors, kidney tumors are not a primary feature of NF. *Li-Fraumeni syndrome* - **Li-Fraumeni syndrome** is associated with an increased risk of various cancers, including **sarcomas**, **breast cancer**, **adrenocortical carcinomas**, and **leukemia**. - While brain tumors can occur, the specific combination of brain and kidney tumors with a clear family history is less characteristic of Li-Fraumeni than VHL syndrome. *Churg-Strauss syndrome* - **Churg-Strauss syndrome (Eosinophilic Granulomatosis with Polyangiitis)** is a systemic vasculitis characterized by **asthma**, **eosinophilia**, and **granulomatous inflammation**. - It does not involve the development of brain or kidney tumors.
Question 5: Which one of the following is the most common CNS tumor associated with type I neurofibromatosis?
- A. Optic nerve glioma (Correct Answer)
- B. Meningioma
- C. Acoustic schwannoma
- D. Low grade astrocytoma
Explanation: ***Optic nerve glioma*** - **Optic nerve gliomas** are the most frequently encountered central nervous system tumors in patients with **Type 1 neurofibromatosis (NF1)**, occurring in about 15% of individuals. - They are typically low-grade **astrocytomas** and can cause vision loss and proptosis depending on their size and location. *Meningioma* - While more common in **Type 2 neurofibromatosis (NF2)**, meningiomas can occur in NF1, but are not the most common CNS tumor. - Meningiomas are tumors that arise from the **meninges**, the membranes surrounding the brain and spinal cord. *Acoustic schwannoma* - **Bilateral acoustic schwannomas (vestibular schwannomas)** are the hallmark feature of **Type 2 neurofibromatosis (NF2)**, not NF1 [1]. - These tumors arise from the Schwann cells of the **vestibulocochlear nerve** and can cause hearing loss and balance issues [1]. *Low grade astrocytoma* - While optic nerve gliomas are a type of low-grade astrocytoma, this option is too general; **optic nerve glioma** is the specific and most common presentation in NF1. - Other forms of low-grade astrocytomas can occur in NF1 but are not as universally characteristic as optic nerve gliomas.
Question 6: Most common tumor in the part of the brain shown (arrow) among children is
- A. DNET
- B. Ependymoma
- C. Astrocytoma (Correct Answer)
- D. Medulloblastoma
Explanation: ***Astrocytoma*** - The image shows a **brainstem lesion**, which is a common location for astrocytomas in children. - **Pilocytic astrocytoma** is the most common brain tumor in children, often presenting in the cerebellum, brainstem, or optic pathways. *DNET (Dysembryoplastic Neuroepithelial Tumor)* - DNETs are typically found in the **temporal lobe** and are associated with a history of seizures. - While benign, their characteristic location and symptoms do not align with a brainstem mass as the primary finding. *Ependymoma* - Ependymomas frequently arise in the **fourth ventricle** in children, often causing hydrocephalus. - Although they can occur in the brainstem, astrocytomas are generally more common in this specific region in pediatric cases. *Medulloblastoma* - Medulloblastomas are nearly always located in the **cerebellum** (infratentorial region) and are known for their aggressive nature and propensity for CSF dissemination. - The lesion shown appears to be within the brainstem, making medulloblastoma less likely given its typical cerebellar location.
Question 7: Most common site for medulloblastoma is-
- A. Cerebellum (Correct Answer)
- B. Pituitary
- C. Cerebrum
- D. Pineal gland
Explanation: ***Cerebellum*** - **Medulloblastoma** is a highly malignant primary brain tumor that characteristically arises in the **cerebellum** [1]. - It is the most common malignant brain tumor in children, typically originating from the **roof of the fourth ventricle**. *Pituitary* - The **pituitary gland** is mostly associated with **adenomas**, which are benign tumors arising from anterior pituitary cells. - Tumors like **craniopharyngiomas** can also be found in the sellar region, but medulloblastomas do not originate here. *Cerebrum* - The **cerebrum** is the most common site for **gliomas** (e.g., glioblastoma multiforme) and metastatic tumors in adults. - Medulloblastoma specifically originates from primitive neuroectodermal cells in the posterior fossa [1]. *Pineal gland* - The **pineal gland** is associated with **pinealomas** (e.g., pineoblastoma, pineocytoma) and **germinomas** [2]. - These are distinct from medulloblastomas in their cellular origin and typical anatomical location.
Question 8: Most common supratentorial tumor in children
- A. Astrocytoma
- B. Oligodendroglioma
- C. Meningioma
- D. Craniopharyngioma (Correct Answer)
Explanation: ***Craniopharyngioma*** - **Craniopharyngiomas** are the **most common supratentorial tumors in children**, accounting for approximately 5-10% of all pediatric intracranial tumors. - They arise from **Rathke's pouch remnants** near the pituitary gland and hypothalamus in the **sellar/suprasellar region**. - Present with **visual disturbances, endocrine dysfunction, and increased intracranial pressure**. - Bimodal age distribution with peaks at **5-14 years** and 50-74 years. *Astrocytoma* - While **astrocytomas** are the **most common primary CNS tumors in children overall**, they are predominantly located in the **posterior fossa (infratentorial)**, particularly as **cerebellar astrocytomas** [1], [2]. - In the **supratentorial compartment specifically**, astrocytomas are less common than craniopharyngiomas in the pediatric population. - They arise from **astrocytes** and range from low-grade (pilocytic) to high-grade (glioblastoma) [1]. *Oligodendroglioma* - **Oligodendrogliomas** are **rare in children** and more prevalent in adults (peak 40-50 years). - They originate from **oligodendrocytes** and are typically **slow-growing tumors**. - Account for only 1-2% of pediatric brain tumors. *Meningioma* - **Meningiomas** are **very rare in children** (less than 2% of pediatric brain tumors) and are primarily tumors of adulthood. - They arise from **arachnoid cap cells** of the meninges and show increasing incidence with age. - When they occur in children, they are often associated with **neurofibromatosis type 2** [2]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 725-726. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1319-1320.
Question 9: Which of the following CNS tumor shows increased growth during pregnancy?
- A. Oligodendroglioma
- B. Meningioma (Correct Answer)
- C. Craniopharyngioma
- D. Glioblastoma Multiforme
Explanation: ***Meningioma*** - **Meningiomas** often express receptors for **estrogen** and **progesterone**, leading to increased growth rates during the **estrogen-rich environment of pregnancy**. - This tumor's growth during pregnancy can exacerbate neurological symptoms or lead to the discovery of previously asymptomatic meningiomas. *Oligodendroglioma* - **Oligodendrogliomas** are a type of glioma that do not typically show an increased growth rate in response to hormonal changes during pregnancy. - Their growth is generally independent of **sex hormones**, and their progression is determined by other genetic and molecular factors. *Craniopharyngioma* - **Craniopharyngiomas** are benign epithelial tumors that arise from Rathke's pouch remnants and primarily affect children, though they can occur in adults. - Their growth is not significantly influenced by **hormonal changes** related to pregnancy. *Glioblastoma Multiforme* - **Glioblastoma multiforme (GBM)** is a highly aggressive and fast-growing primary brain tumor, but its growth is not directly stimulated by the hormonal changes of pregnancy. - While GBM can rapidly progress during pregnancy, this is due to its inherent aggressive nature rather than hormonal effects on tumor cells.
Question 10: CNS tumor seen in Von Hippel Lindau syndrome is:
- A. Meningioma
- B. Glioma
- C. CNS lymphoma
- D. Cerebellar hemangioblastoma (Correct Answer)
Explanation: ***Cerebellar hemangioblastoma*** - **Cerebellar hemangioblastomas** are characteristic CNS tumors associated with **Von Hippel-Lindau syndrome** due to mutations in the VHL gene [1]. - Patients typically present with symptoms related to raised **intracranial pressure** or focal neurological deficits depending on the tumor's size and location. *Meningioma* - Meningiomas are **benign tumors** arising from the meninges and are generally not a primary feature of **Von Hippel-Lindau syndrome** [1]. - They are more commonly associated with **Neurofibromatosis type 2** if linked to a genetic syndrome [1]. *Glioma* - Gliomas, which include astrocytomas, oligodendrogliomas, and ependymomas, are a diverse group of brain tumors that are not typically or specifically linked to **Von Hippel-Lindau syndrome** [1]. - While central nervous system tumors, they lack the specific association seen with **hemangioblastomas** in VHL. *CNS lymphoma* - **CNS lymphoma** is primarily associated with **immunocompromised states**, such as HIV/AIDS, or occurs as a primary brain tumor in older adults [1]. - It has no known direct association with **Von Hippel-Lindau syndrome** [1]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 724-728.
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