[HOUSE STARK] 2017

INFRATENTORIAL TUMORS
ADDITIONAL NOTES IN PEDIA NEURO (PART 2)
DR. DIAZ  Incoordination
 Ataxia
 Nystagmus
CLASSIFICATION AND GRADING OF INTRACRANIAL NEOPLASM  Diplopia
(WH0)  Blurred Vision
 Torticollis
HISTOLOGICAL GROUPS  Cerebellar Dysfunction
Neuroepithelial
Meningeal CEREBELLAR ASTROCYTOMAS
Sellar Region
Germ Cell Most common primary CNS tumor
Lymphoid-Hematopoeitic Juvenile pilocytic astrocytoma in cerebellum
Cranial/ Spinal Nerve (Grade 1); fibrillary diffuse (Grade 2); anaplastic
(Grade 3); Gliobalstoma mulitforme (Grade 4)
WHO GRADING Peak age 5-9 years
 GRADE 1 – slow growing, non-malignant, with long- RADIOLOGIC FUNCTION: neuromedium enhancing
term survival nodule within the wall of as cystic mass
 GRADE 2 – relatively slow-growing, recurrent, MICROSCOPY: presence of Rosenthal bodies help
progress to higher grade establish the diagnosis
 GRADE 3 – malignant, recur as higher grade Low metastatic potential, rarely spread
 GRADE 4 – very aggressive Complete surgical resection = 80-100% cure

MEDULLOBLASTOMA

Most common malignant tumor

Accounts for 90% of PNET or embryonal tumor
Most common cytogenic abnormality is
chromosome 17p deletions
Median age of 5-7 years, mostly males
Occurs in the midline cerebellar vermis
Children , 4 years have poo outcome because of
incidence of dissemination
Radiosensitive and chemotherapy sensitive

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The tumor may spread contagiously, to the Dense cellularity, high mitotic index, microvesicular
cerebellar peduncle and/or the floor of the fourth proliferation, foci of tumor necrosis
ventricle Over expression of P53 is a poor prognostic sign
Anteriorly, to the brainstem
Inferiorly, to the cervical spine CRANIOPHARYNGIOMA
Superiorly, above the tentorium
It may also spread via the CSF intra-cranially or to WHO Grade 1, histologically benign neuroepithelial
the leptomeninges and spinal cord tumor
Peak age incidence 5-10 years
BRAINSTEM GLIOMA Remnant of Rathke’s Pouch – Pituitary Gland
Minimally invasive, adheres to brain parenchyma,
Peak age 7-9 years engulf brain tissues
Accounts for 80% of brainstem tumors RADIOLOGIC FUNCTION: calcifications with solid and
Constitute 10-20% of childhood primary CNS cystic wall components
tumors Surgery + radiotherapy, chemotherapy has no role
Classic triad – motor weakness, lower cranial
nerve deficits, ataxia PINEAL GLAND TUMORS
4 Types – focal, dorsally exophytic,
cervicomedullary, diffuse intrinsic tumor 2ND most common malignancies after germ cell
Radiotherapy and chemotherapy, no role for tumor in pineal area
surgery PINEOCYTOMA – benign, young adults, present as
hydrocephalus
SUPRATENTORIAL TUMORS PINEOBLASTOMA – most malignant variant, PNET in
pineal area, young children; associated with
 Motor weakness retinoblastoma, CSF seeding
 Sensory changes Multimodal therapy
 Speech disorders
 Seizures
 Change in hand preference
 Hemiparesis

EPENDYMOMA

Third most common primary CNS tumor

Tumor of the ependymal lining of the ventricles –
glial cell differentiated along glial lines
70% in the posterior fossa, in the 4th ventricle
Mean age is 6 years
Younger children have poorer outcome
Multimodal treatment – sensitive to chemotherapy,
surgery

GLIOBALSTOMA MULTIFORME

Subcortical white matter of the cerebral

hemisphere
May infiltrate adjacent cortex, basal ganglia and
contralateral hemisphere
WHO Grade 4
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NEURODIAGNOSTIC PROCEDURES TEMPORAL LOBECTOMY & HEMISPHERECTOMY

EEG
Skull X-ray
Cranial CT Scan
Cranial MRI

MANAGEMENT

Close Observation
Multimodal:
 Surgery
 Radiotherapy
 Chemotherapy

SURGERY

1. Surgery also may help: RADIOTHERAPY

1. Reduce the size of the tumor
2. Relieve symptoms, such as headaches, and
nausea
3. Place a shunt to drain excess CSF, which may
cause including headaches and blurred vision
VENTRICULOPERITONEAL SHUNT
1. EXTERNAL BEAM RADIATION THERAPY (EBRT) –
focus from the source outside the body
2. THREE-DIMENSIONAL CONFORMAL RADIATION
(3D-CRT) – 3D-CRT uses the results of imaging
tests such as MRI and special computers; radiation
beams are then shaped and aimed at the tumor
from different conditions

3. INTENSITY MODULATED RADIATION THERAPY

(IMRT) – advanced form of 3D therapy; shaping the
beams and aiming them at the tumor from several
angles, the intensity (strength) of the beams can be
adjusted to limit the dose reaching the most
sensitive normal tissues

4. CONFORMAL PROTON BEAM RADIATION THERAPY:

Proton beam therapy is related to 3D-CRT; proton
beams on the tumor; less damage

5. STEREOTACTIC RADIOSURGERY/ STEREOTACTIC

RADIOTHERAPY: delivers a large, precise radiation
dose to the tumor area in a single session
(radiosurgery) or in a few session (radiotherapy)

6. BRACHYTHERAPY (INTERNAL RADIOTHERAPY):

Unlike the external radiation approaches above in
brachytherapy a radiation source is put directly
into or near the tumor; most often used along with
external radiation. It provides a high dose of
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radiation at the tumor site, while the external

radiation treats nearby areas with a lower dose.

7. WHOLE BRAIN AND SPINAL CORD RADIATION

THERAPY (CRANIOSPINAL RADIATION): is tests,
such as MRI scan or lumbar puncture, show the
tumor has spread along the covering od the spinal
cord (meninges) or into the surrounding
cerebrospinal fluid, then radiation may be given to
the whole brain and spinal cord

8. GAMMA KNIFE RADIOSURGERY: delivers a pinpoint

dose of radiation to the tumor from hundreds of
angles. It may be used if the tumor’s location makes
it impossible to remove of if the child is not healthy

CHEMOTHERAPY

Chemotherapy is used for many types of brain

tumors including aggressive, high-grade tumors
Chemotherapy can be administered as pills (orally),
intravenously (IV, by vein), injected directly into the
cerebrospinal fluid, or injected directly into the
cavity left after surgical removal of the brain tumor
– Carboplatin, Carmustine (BCNU), Cisplatin,
Cyclophosphamide, Etoposide, Lomustine (CCNU),
Methotrexate, Temozolomide, Thiotepa, Vincristine

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