pediatric tumors
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Pediatric Neurosurgical NeuropathologyPediatric Neurosurgical Neuropathology
Brain tumors are second only toleukemias in children
Brain tumors are the most common solidorgan tumor in children
70% of pediatric brain tumors are in theposterior fossa
Pilocytic astrocytoma
Medulloblastoma
Ependymoma
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CNS tumors: pediatric vs. adultCNS tumors: pediatric vs. adult
Adults: 70% of tumors are supratentorial
meningioma
pituitary adenoma
High grade astrocytoma Anaplastic astrocytoma (grade III)
Glioblastoma multiforme (grade IV astrocytoma)
Pediatric: 70% in posterior fossa
pilocytic astrocytoma (cerebellar astrocytoma)
medulloblastoma
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Brain tumors: introBrain tumors: intro
Intracranial neoplasms
Primary
Secondary Metastatic
Local invasion
Tumors of the spinal cord
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Primary brain tumors: introPrimary brain tumors: intro
Primary brain tumors are rare 2.5% of all cancer deaths
Second most common type of tumor in children
There are over 100 different brain tumors
Most common types Astrocytomas
Grades I-IV
Medulloblastomas
primitive neuroectodermal tumor-PNET
Meningiomas
Pituitary adenomas
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Clinical presentationClinical presentation
Clinical symptoms depend upon: Age, location, and type of tumor and grade
Symptoms may include: Increased intracranial pressure
secondary to obstruction of CSF at aqueduct
hydrocephalus (infants), headache, papilledema, vomiting
seizures
focal neurological deficits
hormonal changes (pituitary adenoma) visual changes (diplopia, field defects)
Pituitary adenoma - pressure on optic chiasm
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CNS tumors: diagnosisCNS tumors: diagnosis
Symptoms prompt neuroimaging
CT and MRI
intra-axial vs. extra-axial Location of tumor
contrast enhancement typical of high grade
also in some low grade, i.e., pilocytic astrocytomas
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CNS tumors: locationCNS tumors: location
Extra-axial
meningiomas
Cerebral hemispheres
grade II-III astrocytomas, GBM Crossing corpus callosum - GBM
optic nerve - pilocytic astrocytoma (NF-1)
Sella - Pituitary adenoma
Peri-III ventricle - Pilocytic astrocytoma, GBM
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CNS tumors: locationCNS tumors: location
posterior fossa (in children)
pilocytic astrocytoma
medulloblastoma
brainstem (pons)
pontine glioma (astrocytoma)
spinal cord
low-grade astrocytomas (grade I and II)
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Pilocytic astrocytomasPilocytic astrocytomas
Most common in children Grade I astrocytoma
Cerebellum (posterior fossa), optic nerve Thalamic, spinal cord, cerebral
Discrete, well circumscribed mass Often with associated cystic area
Contrast enhancing
Histologic appearance:
Biphasic: piloid cells and microcystic areas Rosenthal fibers
no mitoses
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Pilocytic astrocytomasPilocytic astrocytomas
Tumor of cerebellum, often with cyst,biphasic, Rosenthal fibers, piloidcells
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AstrocytomaAstrocytoma -- high gradehigh grade
Astrocytoma grade II and III are very, very rare inthe pediatric population
Grade IV - glioblastoma multiforme
Diffusely infiltrating glial tumor of cerebral
hemispheres Contrast enhancing tumor
Histological appearance: Densely cellular, with marked nuclear pleomorphism
Numerous mitoses Endothelial proliferation
Necrosis with pseudopallisading
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Glioblastoma (grade IV)Glioblastoma (grade IV)
Less common in children than adults, typicalpathology (necrosis with psuedopallisading)
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Pontine gliomaPontine glioma
Diffuse expansion of pons, usually highgrade astrocytoma (III-IV)
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MedulloblastomasMedulloblastomas
PNET of posterior fossa in children
Histologic appearance:
Densely cellular small blue cell tumor
N
umerous mitoses Apoptotic (karyorrhectic) cells
Endothelial proliferation
Necrosis
neuronal or glial differentiation Homer Wright rosettes
GFAP positive cells
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MedulloblastomaMedulloblastoma
Mass arising in roof offourth ventricle
Homer Wright rosettes
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EpendymomaEpendymoma
Mass arising in floor offourth ventricle
Perivascularpseudorosettes
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MeningiomasMeningiomas
Discrete non-invasive tumor
Extra-axial, pushes into brain
Attached to dura
H
yperostosis or invasion of skull common Histologic appearance:
Fibroblastic or menigothelial cells
Meningothelial whorls
Psammoma bodies Rare in children, may be intraventricular
(lateral ventricles)
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MeningiomasMeningiomas
Extra-axial tumor, meningothelial cells, whorls andpsammoma bodies
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GangliogliomaGanglioglioma
Cerebrum, cervicomedullary, often with cystic component
Increased numbers of neurons (some binucleate) and increased glial cells(usually astrocytic)
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CraniopharyngiomaCraniopharyngioma
Heterogeneous, cystic mass in suprasellar region Basiloid layer, stellate reticulum, wet keratin, often
calcified
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Choroid plexus papillomaChoroid plexus papilloma
Lateral ventricle in children (fourth ventriclein adults)
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Germ cell tumorsGerm cell tumors
Pineal - 99% males, most are germinomas Suprasellar - often mixed germ cell tumor, 50%
female
Tertomas are rare
Germinoma Teratoma
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Metastatic tumorsMetastatic tumors
The most common brain tumor in adults ismetastatic
Metastatic tumors are rare in children
The most common metastatic tumor in children isosteosarcoma
Local extension of malignant tumors of vertebralbodies (Ewings sarcoma) or paravertebral soft tissues(neuroblastoma) are not uncommon
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Other tumorsOther tumors S
ubependymal giant cell astrocytoma (S
EGA) Intraventricular tumor in Tuberous sclerosis
Desmoplastic infantile ganglioglioma (DIG)
Superficial cerebral tumor in infants
Dysembryoplastic neuroepithelial tumor (DNET)
Hamartomatous lesion associated with seizures Atypical teratoid rhabdoid tumor (ATR, AT/RT)
Infants, posterior fossa, very malignant
Eosinophilic granuloma
A type of Langerhans cell histiocytosis
Single discrete osteolytic lesion in skull Meningioangiomatosis
Hamartomatous superficial cerebral lesion associated withseizures
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Hereditary syndromesHereditary syndromes
Neurofibromatosis type I Caf-au-lait spots
Dermatofibromas, multiple
optic nerve gliomas, bilateral
plexiform neurofibroma Malignant peripheral nerve sheath tumor
Neurofibromatosis type II
bilateral acoustic neuroma
multiple meningiomas
ependymomas