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Medicine Simplified DBMCI

Brain Tumour (Ref. Harrison 20th ed. pg. 643)

Table: Malignant intracranial tumours


Histological type Common site Malignancy Age
Glioma (Astrocytoma) Cerebral hemisphere + + - + + + Adult
Cerebellum +-++ Childhood
++-+++ Adult
Brain stem +-++ Childhood/Adolescence
Oligodendroglioma Cerebral hemisphere + + - + + + Adult
Medulloblastoma Posterior fossa +++-++++ Childhood
Ependymoma Posterior fossa +++-++++ Childhood/adolescence
Microglioma (Cerebral lymphoma) Cerebral hemisphere + + - + + + Adult

Table: Benign intracranial tumours


Histological type Common site Age
Meningioma Cortical dura Adult
Parasagittal
Sphenoid ridge
Suprasellar
Olfactory groove

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Neurofibroma Acoustic neuroma Adult
Craniopharyngioma Suprasellar Childhood/adolescence
Pituitary adenoma Pituitary fossa Adult
Colloid cyst Third ventricle Any age

Extra Edge: Facts to Remember:


1. The most common tumors in children are: Leukemia
2. The most common solid tumour in children are: CNS tumors
3. The most common intracranial neoplasm in adult is astrocytoma.
4. Meningioma do not metastasize.
5. 'Most common Primary Intracranial Neoplasms in adults
a. Astrocytomas (Type of Glioma and includes Glioblastoma Multiforme) 30%
b. Meningiomas 15%
6. Glial tumors Astrocytomas b. Oligodendroglioma c. Ependymomas

Note: Most common glial tumors is astrocytomas.

Cerebellar Astrocytomas
1. Cerebellar Astrocytomas are equally common in both males & females.
2. The predominant group of astrocytomas in childhood are low grade astrocytomas (Nelsons)
3. Juvenile Pilocytic Astrocytoma (JPA) is the most common astrocytoma in children
4. Juvenile Pilocytic Astrocytoma is a low grade tumor and is classified as a WHO grade I tumor
5. Cerebellar Astrocytomas (Juvenile JPA) most commonly develop during the first two decades of life (WHO
classification of Tumors of CNS)
6. Cerebellar Astrocytomas have a very good prognosis
7. With complete surgical resection the overall survival for Juvenile Pilocytic Astrocytomas approaches 80% - 100%

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Astrocytomas
A. Non infiltrating (low grade) Benign: (WHO grade I)
1. Pilocytic astrocytoma
2. Pleomorphic xanthoastrocytoma
3. Sub ependymal giant cell astrocytoma
B. Infiltrating (WHO Grade II, III, IV)
1. Low grade astrocytomas (WHO Grade II)
a. Fibrillary b. Protoplasmic c. Gemistocytic
2. High Grade (WHO Grade III/IV)
a. Anaplastic Astrocytoma (WHO III) b. Glioblastoma Multiforme (WHO IV)

Important points
1. Astrocytoma (Cerebellar)
a. Second Most common posterior fossa tumor in children is Cerebellar Astrocytoma
b. Second Most common brain tumor in children is also Cerebellar Astrocytoma.
c. Astrocytoma also has the best prognosis in children.
d. Tumor with best prognosis in children is also Astrocytoma.

2. Brain Stem Gliomas: These are the third most frequent post fossa tumors in children
3. Craniopharyngioma: These are the most common supratentorial tumor in children.

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Medulloblastomas
Medulloblastomas are highly radiosensitive tumors of the CNS and more radiosensitive than all gliomas (Astrocytoma,
Ependymoma, Glioblastoma Multiforme) whereas Glioblastomas are the least radiosensitive'

Medulloblastoma
a. The most common posterior fossa tumor in children. (NEET 2019)
b. Tumor which spreads along CSF pathways is medulloblastoma. (NEET 2019)
c. Tumor which is capable of metastasizing to extracranial sites is medulloblastoma. (NEET 2017)

Important Points
1. Medulloblastoma is a highly malignant primary brain tumor that originates in the cerebellum or posterior fossa.
2. Originally considered to be a glioma, medulloblastoma is now known to be of the family of cranial primitive
neuroectodermal tumors (PNET).
3. Medulloblastoma is the most common PNET originating in the brain.
4. All PNET tumors of the brain are invasive and rapidly growing tumors that, unlike most brain tumors, spread
through the CSF and frequently metastasize to different locations in the brain and spine (bone) (NEET 2018).

Recent Advances (AIIMS May 2016)


Chang classification staging system.
1. M0 - No gross subarachnoid or hematogenous metastasis
2. M1 - Microscopic tumor cells found in CSF
3. M2 - Gross nodular seeding in cerebellum, cerebral subarachnoid space, or in the third or fourth ventricles
4. M3 - Gross nodular seeding in spinal subarachnoid space
5. M4 - Extra neuraxial metastasis.

Radiosensitive Brain Tumors in Children


1. Medulloblastomas and other PNET (Most radiosensitive)
2. Gliomas (low grade astrocytoma), ependymoma

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3. Pineal blastoma
4. Craniopharyngioma
5. Germ cell tumors (pure Germinoma-highly radiosensitive)

Cerebellar Hemangioblastoma
Cerebellar hemangioblastoma (NEET 2017) is a benign neoplasm that occurs either sporadically or as part of Von Hippel-
Lindau disease (NEET 2019)

Clinically presentation is with:


1. Cerebellar dysfunction
2.Hydrocephalus
3. Polycythemia (the tumor produces erythropoietin).

Subependymal Giant cell Astrocytomas (SEGCA)


1. They are low grade non filtrating (benign) variants of astrocytomas.
2. They are characteristically associated with tuberous sclerosis (NEET 2018)
3. The most common site of SEGCA is the ependymal wall of lateral ventricle near the foramen of Munro and
these tumors tend to grow towards the foramen of Munro (leading to obstructive hydrocephalus)
4. Pathologically lesions are composed of Giant cells
5. Candle dripping radiographic appearance is characteristic (NEET 2018)
6. These tumors predominantly occur is children (lst decade)

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7. Total Gross resection is the treatment of choice

Recent Advances:
Chemotherapy for brain Tumour
1. Carmustine (CCNS) lomustine (BCNS): than side effects of these drugs = delayed bone marrow suppression.
2. Temozolomide: Its main indication is anaplastic astrocytoma which is refractory to conventional treatment.

Hereditary Syndromes Associated with Brain Tumors


Syndrome Nervous System Neoplasms
Neurofibromatosis type 1 (von Recklinghausen's Disease) Neuroma, schwannoma, meningioma, optic glioma
Neurofibromatosis type 2 Acoustic neuroma

von Hippel-Lindau Hemangioblastoma of retina, cerebellum and spinal


cord; pheochromocytoma
Tuberous sclerosis Astrocytoma
Li-Fraumeni Malignant glioma
Gorlin (basal cell nevus syndrome) Medulloblastoma
Turcot Medulloblastoma, malignant glioma
Multiple endocrine neoplasia 1 (Werner syndrome) Pituitary adenoma, malignant schwannoma

Brain Herniation
Herniation or coning is said to be occurring when part of the brain is forced through a rigid hole.
1. Uncal herniation
The uncus and the temporal lobe are forced through the cerebellar tentorium (tentorium that separates the
cerebrum from the cerebellum)

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a. It causes: Sequential compression occurs of the


i. Ipsilateral third nerve ii. Contralateral brainstem (later)
b. Physical signs
Signs a). I/L hemiplegia b). Progressive ptosis & third n palsy
Still Later a). Tetraparesis b). B/L fixed dilated pupils c). Erratic respiration d). Death

2. Central herniation
The diencephalon i.e. the thalamus and related structures that lie between upper brainstem and cerebral
hemispheres are forced through the tentorium
a. It causes: Sequential compression occurs of the
i. Upper midbrain (first) ii. Pons (later) iii. Medulla (finally)
b. Physical signs
i. Early a). Erratic respiration b). Small reactive pupils
c). Increased limb tone d). Bilateral extensor plantar
3. Cingulate Herniation –
It occurs by frontal mass cingulate gyrus. (Medial frontal lobe) is forced down under falx cerebri, later on ACA
obstruction causing stroke and abulia (lack of decision).

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