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31 Cards in this Set
- Front
- Back
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What is the classic traid?
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headache, vomiting and papilledema
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s/s increased intracranial pressure (ICP) including vomiting, lethargy, poor feeding and macrocephaly
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Sx of open fontanelle
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Gaze palsy, upper motor neuron signs -pareses, hyperreflexia, clonus
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brainstem lesions
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Focal deficits: sensory changes, speech disorders, seizures and reflex abnormalities
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supratentorial lesion
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MRI better than CT for....
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pituitary, suprasellar, optic pathway and infratentorial tumors
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Most common pediatric brain tumor category
Compromised 40% of primary CNS malignancies Low grade predominate (indolent course) |
Astrocytomas
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Most common form of astrocytoma in pediatrics
20% of all brain tumors Classically cerebellum Can also be seen in the hypothalamic/3rd ventricle region, optic nerve/chiasm tract On neuroimaging classically (but not exclusively) appears as a contrast enhancing nodule within a cystic mass |
Juvenile Pilocytic Astrocytoma
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Histologically see Rosenthal fibers which are condensed masses of glial filaments
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Juvenile Pilocytic Astrocytoma
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Low metastatic potential
Rarely invasive Very rarely can undergo malignant transformation into more aggressive tumor type 15% of patients with Neurofibromatosis Type 1 will develop JPA type lesion of the optic chiasm |
Juvenile Pilocytic Astrocytoma
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Histologically shows diffuse infiltration of tumor cells with normal neural tissue
Potential for anaplastic progression Lack of contrast enhancement on neuroimaging 15% of all pediatric brain tumors |
Fibrillary Infiltrating Astrocytoma
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Anaplastic astrocytoma
Glioblastoma multiforme Rare ( combined 7 – 10% of pediatric brain tumors) |
High grade Astrocytoma
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When is chemo especially considered over radiation?
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When patient is <5 yrs old
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10% of pediatric brain tumors
70% present in the posterior fossa MRI: well circumscribed tumor with variable patterns of contrast enhancement. May show cystic structures |
Ependymomas
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Histologically show perivascular pseudorosettes and elongated cells known as “tadpole”, “ice cream” or “carrot” cells
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ependymal tumors
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Primary treatment modality is surgery with the extent of surgical resection being a strong prognostic indicator of outcome
With gross total resection plus radiation long term survival only approaches 40% Younger age = poorer outcome Posterior fossa location = poorer outcome |
Ependymal tumors
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2-4 % of CNS tumors
Most common CNS tumor of infancy An intraventricular neoplasm arising from the choroid plexus epithelium Infant usual present with signs of increased ICP |
Choroid Plexus Tumor
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20 -25 % of pediatric CNS tumors
High grade Includes Medulloblastoma Supratentorial PNET Ependymoblastoma Medulloepithelioblastoma Atypical teratoid/rhabdoid tumor (ATRT) |
PNET
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Cerebellar
Male predominance Median age 5- 7 years Most are midline cerebellar vermis lesions On neuroimaging show a solid, contrast enhancing lesion located in the posterior fossa causing 4th ventricle obstruction and hydrocephalus |
Medulloblastoma
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Histologically show small, round, blue cells in a monomorphic sheet (most common)
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Medulloblastoma
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Usually present with sings of increased ICP: ataxia, balance problems owing to location
Younger age = worse prognosis Prognosis poor if signs of dissemination at diagnosis (including positive CSF cytology alone) Prognosis worse for incomplete resection |
Medulloblastoma
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Surgery
Radiation Chemotherapy Overall survival 60 -70% High incidence of neurologic morbidity due to craniospinal irradiation in young patients |
Medulloblastoma
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2-3% of brain lesions
< 10 years of age Histologically similar to medulloblatoma Poor prognosis |
Supratentorial PNET
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Very aggressive
< 5 years of age Very poor outcomes Cytogenetically show a deletion of chromosome 22Q |
ATRT
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Very rare
Highly malignant Seen in infancy and early childhood |
Ependymoblastoma and medullomyoblastoma
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7 -10 % of pediatric brain tumors
Solid and cystic components Suprasellar location May shoe calcifications on CT with solid and cystic wall components CT PIC OF CRANIO |
Craniopharyngioma
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Surgery is the primary treatment modality
High incidence of pituitary problems, growth failure, and visual loss due to location No significant role for chemotherapy ? Radiation in large or complex lesions |
Craniopharyngioma
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Uncommon in pediatrics
Derived from the cells of the meninges Typically “benign”, but can cause problems due to mass effect on surrounding brain structures |
Meningioma
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Usually midline/pineal/suprasellar
1-2 % of CNS lesions 10 -12 years old Male predominance Can be multifocal 5 -10 % of the time Asian predominance |
Germ Cell Tumors
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Association with the protein markers α-fetoprotein and β human chorionic gonadotropin (as in peripheral germ cell tumors)
Sometimes used in diagnosis and to monitor treatment response Surgery, radiation and chemo all treatment modalities |
Germ Cell Tumors
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What are the most common metastatic pediatric brain lesions?
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ALL and non-hodgkin lymphoma
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collections of myeloid leukemic cells can be seen in leukemia
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Chloromas
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