Pediatric Neurosurgical Neuropathology

 Brain tumors are second only to

leukemias in children
 Brain tumors are the most common solid
organ tumor in children
 70% of pediatric brain tumors are in the
posterior fossa
– Pilocytic astrocytoma
– Medulloblastoma
– Ependymoma
CNS 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
Brain tumors: intro

 Intracranial neoplasms
– Primary
– Secondary
» Metastatic
» Local invasion
 Tumors of the spinal cord
Primary 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
Clinical 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
CNS 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
CNS 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
CNS tumors: location
 posterior fossa (in children)
– pilocytic astrocytoma
– medulloblastoma
 brainstem (pons)
– pontine glioma (astrocytoma)
 spinal cord
– low-grade astrocytomas (grade I and II)
Pilocytic 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
Pilocytic astrocytomas

Tumor of cerebellum, often with cyst,

biphasic, Rosenthal fibers, piloid
cells
Astrocytoma - high grade
 Astrocytoma grade II and III are very, very rare in the
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
Glioblastoma (grade IV)

 Less common in children than adults, typical

pathology (necrosis with psuedopallisading)
Pontine glioma

Diffuse expansion of pons, usually high

grade astrocytoma (III-IV)
Medulloblastomas
 PNET of posterior fossa in children
 Histologic appearance:
– Densely cellular “small blue cell tumor”
– Numerous mitoses
– Apoptotic (karyorrhectic) cells
– Endothelial proliferation
– Necrosis
– neuronal or glial differentiation
» Homer Wright rosettes
» GFAP positive cells
 Medulloblastoma

 Mass arising in roof of

fourth ventricle
 Homer Wright rosettes
 Ependymoma

 Mass arising in floor of

fourth ventricle
 Perivascular
pseudorosettes
Meningiomas
 Discrete non-invasive tumor
– Extra-axial, pushes into brain
– Attached to dura
– Hyperostosis or invasion of skull common
 Histologic appearance:
– Fibroblastic or menigothelial cells
– Meningothelial whorls
– Psammoma bodies
 Rare in children, may be intraventricular
(lateral ventricles)
Meningiomas

Extra-axial tumor, meningothelial cells, whorls and

psammoma bodies
 Ganglioglioma

Cerebrum, cervicomedullary, often with cystic component

Increased numbers of neurons (some binucleate) and increased glial cells
(usually astrocytic)
 Craniopharyngioma

 Heterogeneous, cystic mass in suprasellar region

 Basiloid layer, stellate reticulum, “wet” keratin, often
calcified
Choroid plexus papilloma

 Lateral ventricle in children (fourth ventricle

in adults)
 Germ cell tumors
Germinoma Teratoma

 Pineal - 99% males, most are germinomas

 Suprasellar - often mixed germ cell tumor, 50%
female
 Tertomas are rare
Metastatic tumors

 The most common “brain” tumor in adults is

metastatic
 Metastatic tumors are rare in children
 The most common metastatic tumor in children is
osteosarcoma
 Local extension of malignant tumors of vertebral
bodies (Ewing’s sarcoma) or paravertebral soft tissues
(neuroblastoma) are not uncommon
 Other tumors
 Subependymal giant cell astrocytoma (SEGA)
– 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 with
seizures
Hereditary syndromes
 Neurofibromatosis type I
– Café-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