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Clincial Neuro
Tumors of the Nervous System
84
Biology
Graduate
01/04/2008

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Term
80% of Adult primary brain tumors are
Definition
Fibrillary Astrocytomas
Term
Name the 8 types of Gliomas
Definition
1. Fibrillary Astrocytomas
2. Pilocytic Astrocytomas
3. Brain stem Gliomas
4. Pleomorphic Xanthoastrocytoma
5. Oligodendrogliomas
6. Ependymomas
7. Choroid Plexus Papillomas
8. Golloid cyst of the Third Ventricle
Term
Fibrillary astrocytomas originate where?
Definition
Cerebrum
Term
Describe the histology of Fibrillary Astrocytomas
Definition
Cells w/ Astrocytic nuclei
Term
How does a low grade Fibrillary astrocytomas look like
Definition
Gray/white infiltrative tumor w/ hypercellularity & nuclear pleomorphism
Term
Name the two types of Fibrillary Astrocytomas
Definition
1. Anaplastic Astrocytoma
2. Glioblastoma Multiforme
Term
Describe an anaplastic Astrocytoma
Definition
It'a a fibrillary astrocytoma w/ mitoses and vascular cell proliferation present w/ increased nuclear anaplasia
Term
What is the most malignant Astrocytoma
Definition
Glioblastoma multiforme
Term
in Glioblastoma multiforme there are areas of the necrosis present w/ ?
Definition
Serpingious patter (psudopalisading) & cystic change
Term
Which growth factor is produced by malignant astrocytes that results in vascular and endothelial proliferation
Definition
VEGF
Term
What is a glomeruloid body?
Definition
Glioblastoma multiforme cell masses that protrude into the vascular lumina
Term
A low grade Fibrillary astrocytoma has what molecular characteristics
Definition
p53 inactivated and overexpression of PDGF-A receptor
Term
in fibrillary astrocytomas when is there a inactivation of RB and p16 tumor suppressor genes?
Definition
When it progresses to Glioblastoma multiforme
Term
Describe a primary (de novo) glioblastoma. What percentage of patients have these
Definition
no p53 mutation, amplification of epidermal growth factor receptor (EGFR) and 1/3 of patients
Term
Describe a secondary glioblastoma
Definition
p53 mutation is present and amplification of epidermal growth factor
Term
In what demographic are pilocytic astrocytomas present?
Definition
children and young adults
Term
where are pilocytic astrocytomas found in the brain? What is it's presentation
Definition
cerebellum and frequent cystic presentation
Term
Brain stem gliomas are present in what two groups
Definition
Children and adolescents
Term
___% of brain stem gliomas progress to glioblastomas
Definition
50
Term
Where are pleomorphic xanthoastrocytoma found? In what demographic?
Definition
Temporal lobe of children and young adults
Term
Give a clinical finding of Pleomorphic xanthoastrocytoma
Definition
frequent seizures
Term
Describe the morphology of Pleomorphic xanthoastrocytoma
Definition
1. Neoplastic astrocytes w/ lipid disposition
2. Chronic inflammatory infiltrates
Term
This tumor is found in middle age patients and has a better prognosis than astroctomas
Definition
oligodendrogliomas
Term
What are the clinical features of oligodendrogliomas
Definition
history of seizures and other neurological symptoms
Term
Where in the brain does oligodendrogliomas originate
Definition
Cerebral hemispheres
Term
describe an oligodendrogliomas
Definition
1. Gelatinous gray masses w/ cyst and hemorrhage; 90% have calcified
2. Sheet of cells w/ spherical nuclei & cytoplasmic halo
Term
Where do Ependymomas arise from
Definition
ependymal layers of the ventricular system
Term
Where is the primary site for ependymomas? in what decade does this happen in? Where is the most common site after this?
Definition
4th ventricle is the common primary site in the first 2 decades followed by the SC
Term
Posterior fossa ependymomas is present in
Definition
hydrocephalus
Term
Ependymomas disseminates through the ___
Definition
CSF
Term
Give the morphological characteristics of ependymomas
Definition
1. Regular nuclei w/ granular chromatin
2. May form ependymal rosettes or perivascular pseudorosettes
Term
Give the characteristics of Myxopapillary ependmyomas
Definition
1. Benign and arise from the SC
2. Tumor cells in the papillae around the vessels & CT
3. Contains acid and neutral mucopolysaccharides
Term
Describe subependymomas
Definition
1. Solid and can be calcified
2. Slow growing nodules that attached to ventricular lining and protruding into ventricle
Term
What are the two types of ependymomas
Definition
Myxopapillary ependymomas and subependymomas
Term
Describe a choroid plexus papilloma
Definition
Looks like a normal choroid plexus w/ papillae covered by cuboidal or columnar cilitaed epithelium
Term
How does a choroid plexus papilloma present
Definition
as hydroocephalus from either a direction obstruction or overproduction of CSF
Term
What is the primary site for choriod plexus papilloma and in what age group
Definition
Lateral ventricles in children and 4th ventricles in adults
Term
This glioma is associated w/ papvavirus
Definition
Choriod plexus papilloma
Term
What's a colloid cyst of the thrid ventricle
Definition
non-neoplastic cyst containing proteinaceous material at the foramen of munro
Term
List the 5 Neuronal tumors
Definition
1. Ganglioglioma
2. Gangliocytoma
3. Cerebral Neuroblastoma
4. Neurocytoma
5. Dysembryoplastic Neuroepithelial tumor
Term
Which Neuronal tumor as a temporal lobe origin
Definition
Ganglioglioma
Term
Describe a Ganglioglioma
Definition
1. Irregular clustered neural cells w/ glial component
2. Random neural distribution w/ frequent binucleated neurons
Term
Gangliocytoma are composed of
Definition
mature neurons
Term
Cerebral neuroblastoma is a rare agressive _____ tumor containing small cells with _______-_______ rosettes
Definition
childhood tumor
Homer-wright
Term
Where are you most likely to find Neurocytoma and what do they look like
Definition
adjacent to the foramen of Munro
Regular nuclei resembling oligodendrogliomas
Term
How does Dysembryoplastic neuroepithelial tumor present
Definition
Childhood cerebral tumor w/ seizures
Term
Medulloblastoma is responsible for __% of __________ brain tumors in the _______
Definition
20% of childhood brain tumors in the cerebellum
Term
Medulloblastoma disseminates through the ___ and can occluded it causing ______
Definition
CSF causing hydrocephalus
Term
Is medulloblastoma malignant or bening
Definition
malignant
Term
How does a medulloblastoma look histologically
Definition
Cellular and anaplastic w/ sparse cytoplasm, hyperchromatic nuclei and abundant mitoses
Term
Primary brain lymphoma is ____ and involves _____ & ______
Definition
rare and involves nodes and marrow
Term
Primary brain lymphoma is assoicated w/ which virus
Definition
Epstein barr
Term
What's the prognosis for primary brain lymphomas? can they treated w/ chemo?
Definition
very poor and are resistance to chemo
Term
Where do Germ cell tumors occur
Definition
midline
Term
Germ cell tumors occur in _______ & ______ _____ and 10% of brain tumor cases in ____
Definition
adolescents and young adults
japan
Term
Germ cell tumors occur in these areas on the brain
Definition
suprasellar and pineal
Term
Pineal parenchymal tumors arise from
Definition
pineal stroma
Term
What is a pinalomas
Definition
Pinealocyte derived tumors that is well-differentiated w/out necrosis or mitoses
Term
Pinealoblastomas occur in ______ are highly aggressive, spread via the CSF and are composed of ______ _____ ____ _____ w/ mitosis & ______
Definition
children
densely packed small cells
necrosis
Term
What kind of tumor are meningiomas and they arise from where
Definition
benign and arise from arachnoid meningothelial cells
Term
Meningiomas are seen mostly in what gender
Definition
Female
Term
The symptoms of meningioma are due to
Definition
compression of the underlying brainor nonlocalizing
Term
What are the characteristics of a meningioma
Definition
Plaque like growth that spread along the dura surface & into the underlying bone
Term
this kind of tumors comprise half of the intracranial tumors
Definition
Metastatic
Term
What kind of tumors are the common source of metastatic tumor and from what areas
Definition
Choriocarcinomas
1. Lung
2. Breast
3. Skin melanomas
4. Kidney
5. Gastro-intestinal tract
Term
Name the 3 types of Peripheral Nerve sheath tumors and from what kind of nerves do they arise from
Definition
1. Schwannomas
2. Neurofibromas
3. Malignant
arise from peripheral nerves
Term
What are the two growth patterns of Schwannomas
Definition
Antoni A & Antoni B
Term
Describe an Antoni A growth pattern. How about a Antoni B growth pattern
Definition
Antoni A: Cellular areas arrange in facicles, interspersed w/ nuclear free processes termed "verocay bodies"
Antoni B: Loose mesh of cells w/ microcystic degernation & myxoid changes
Term
What's the most common intracranial location for schwannomas? How does it present
Definition
Cerebronpontine angle and presents w/ hearing loss and tinnitis
Term
What's an acoustic neuroma
Definition
Schwannomas
Term
Neurofibromas are composed of what? how do they present?
Definition
composed of spindle cells in fat or dermis and present as nodules
Term
These tumors arise on nerve trunks
Definition
Plexiform neurofibroma
Term
Plexiform neurofibrom can infiltrate nerves but cannot
Definition
be seperated from them
Term
Malignant schwannomas arise from malignant ________ ________ _______ and not from malignant transformation of _______ _______
Definition
transformation plexiform neurofibromas
Benign Schwannomas
Term
What are the 4 types of Phakomatoses
Definition
1. Neurofibromatosis type one
2. Neurofibromatosis type two
3. Tuberous Sclerosis
4. von-Hippel-Lindau disease
Term
What are the characteristics of Neurofibromatosis type one
Definition
Multiple neurofibromas, acoustic gliomas, pigmented nodues of iris (Lisch nodules), cutaneous hyperpigmented macules (cafe au lait spots)
Term
What kind of inheritance is neurofibromatosis type one
Definition
autosomal dominant
Term
NF type one has a mutation where? what's the chromsome number
Definition
NF1 tumor suppressor gene on chromosome 17
Term
What are the characteristics of neurofibromatosis type two
Definition
bilateral acoustic schwannomas, multiple meningiomas
Term
What type of inheritance does NF type two have? Where is the mutation at? what chromosome?
Definition
autosomal dominant mutation in NF2 gene on chromsome 22
Term
this tumor is characterized by benign tumors in the brain, hamartomas termed cortical tubers and various cyst and tumors in different organs
Definition
Tuberous sclerosis
Term
what's unqiue to Tuberous Sclerosis
Definition
Subependymal giant cells
Term
Von Hippel-Lindau Disease is characterized by
Definition
intracerebellar capillary hemangioblastomas, cyst in organs and renal cell carcinoma
Term
von-Hippel-Lindau is autosomal ______ and the mutation is in ___ tumor suppressor gene on chromosome __ affecting the protein _____
Definition
dominant
VHL
3
pVHL
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