Term
| What are the Definitive Risk factors associated with CNS tumors? |
|
Definition
Ionizing Radiation
Immunosuppression
(Iatrogenic, chemotherapy, AIDS, Genetic) |
|
|
Term
| What possible risk factors are associated with CNS tumors? |
|
Definition
Electromagnetic Fields (cell phones)
Diet
Occupation
Head Injury
Infections (dysticercosis, Zoster, SV-40) |
|
|
Term
| What tumor type and chromosome/gene is Li-Fraumeni syndrome? |
|
Definition
Glioma, medulloblastoma
17p13, TP53 |
|
|
Term
| What Tumor type and chromosome/gene is associated with Tuberous sclerosis? |
|
Definition
Subependymal giant cell, astrocytoma, cortical tubers, glioma
9q34, 16p13
TSC1, TSC2 |
|
|
Term
| What type of Neurofibromatosis is associated with Glioma (optic nerve) astrocytoma and glioblastoma? |
|
Definition
type 1
Gene: NF1
chromosome: 17q11 |
|
|
Term
| What type of tumors is NF2 associated with? |
|
Definition
Meningioma
Schwannoma (bilateral acoustic neuroma)
ependymomas |
|
|
Term
| What Hereditary syndrome is associated with Pituitary tumors? |
|
Definition
Multiple Endocrine Neoplasia Type 1
(MEN1) |
|
|
Term
| What chromosome and gene are Retinoblastomas associated with? |
|
Definition
|
|
Term
| What Hereditary tumor is associated with Hemangioblastomas? |
|
Definition
Von Hippel-Lindau disease
3p25-20, VHL gene |
|
|
Term
| What are headaches cause by CNS tumors due to? What type of symptoms present? |
|
Definition
Raised intracranial pressure or local irritation
Worse on awakening with improvement within 1 hr
New onset in middle-age or elderly
Change in character or severity of headache |
|
|
Term
| What are the general signs and symptoms of CNS Tumors? |
|
Definition
Headache (worse in the morning)
Vomiting (more often on awakening)
Mental status changes (depression, irritability, apathy) |
|
|
Term
| What do focal signs of CNS tumors results from? |
|
Definition
| direct involvement of elopuent brain structures |
|
|
Term
| What focal signs and symptoms are associated with CNS tumors? |
|
Definition
Papilledema (more common in children and adults)
Seizures (Partial or generalized)
Focal neurologic deficits |
|
|
Term
| What can obstruction of CSF pathways cause? |
|
Definition
| Hydrocephalus -> Increased ICP |
|
|
Term
| How are tumors diagnosed? |
|
Definition
History and Physical Exam
MRI with and without contrast
CT
Tissue Biopsy
Lumbar Puncture |
|
|
Term
| What type of MRI shows CSF, Tumor and Edema as Hypointense (Dark)? |
|
Definition
|
|
Term
| With injectd contrast how does a tumor appear with T1 MRI? |
|
Definition
Hyperintense
contrast leaks across disrupted BBB into tumors |
|
|
Term
| How does CSF/edema/tumors appear in T2 weighted MRI? |
|
Definition
|
|
Term
| How is T2 weighted MRI used to differentiate edema from inflitrating tumor? |
|
Definition
Edema spares the cortex
Tumor does not spare the cortex |
|
|
Term
| What are the major subtypes of Gliomas? |
|
Definition
Astrocytoma
Oligodendroglioma
Ependymoma |
|
|
Term
| What is the most common primary CNS Tumor in adults? |
|
Definition
|
|
Term
| Who are low grade tumors more commonly seen in? |
|
Definition
|
|
Term
| Who are high grade tumors more commonly seen in? |
|
Definition
|
|
Term
| What are the grade ranges from low to high based on? |
|
Definition
| behavior, imaging, and histopathological characterisitics |
|
|
Term
| How is a Grade I Glioma characterized? |
|
Definition
Least Malignant
Grow Slow
well differentiated
No nuclear atypia
Nonenhancing of T1 c contrast
Surgery alone effective
Good Probability of cure with surgery |
|
|
Term
| How is Grade II Glioma characterized? |
|
Definition
Slow Growing
More abnormal histological appearance
Can invade
May recur, sometimes higher grade
Nonenhancing on T1 with contrast
Low Probability of Cure
Survival over many years |
|
|
Term
| How is Grade III Glioma characterized? |
|
Definition
Malignant
Actively reproducing abnormal cells
Tend to Recur, often higher grade
Enhancing on T1 c contrast
Very low to No Probabilty of Cure
Survival 2-3 years |
|
|
Term
| How is Grade IV Glioma characterized? |
|
Definition
Reproduce rapidly
Bizarre histological appearance
Widely infiltrate
Angiogenesis
Necrosis in center
Survival in Months |
|
|
Term
| Why are steroids used for Brain Tumor treatment? |
|
Definition
Reduces vasogenic edema and improves intranial pressure
Dramatic but temporary improvement in neurologic symptoms |
|
|
Term
| What type of Anticonvulsants are used in tumor patients? When are they used? |
|
Definition
Non-hepatic enzyme inducing drugs
Given for focal or generalized seizures caused by the tumor
No prophylactic treatment necessary |
|
|
Term
| When can observation be used as a treatment plan? |
|
Definition
| Reserved for Non life threatening, slow growing, asymptomatic or symptoms can be controlled medically |
|
|
Term
| When is Surgery the 1st line of treatment? What can it provide? |
|
Definition
Accessible Tumors
Establishes pathological diagnosis, Improves ICP and seizure control, Prolongs Survival |
|
|
Term
| When is Radiotherapy used for Tumor treatment? |
|
Definition
Adjuct to surgery for High-grade gliomas
Needed for eradication of infiltrative micro-populations of cells missed with resection |
|
|
Term
| What can Radiation treatment be associated with? |
|
Definition
| Long-term risk of cognitive decline, stroke and secondary cancer |
|
|
Term
| What is the chemotherapy standard of care for malignant glioma? |
|
Definition
| Single agent temozolomide |
|
|
Term
| What Biological agent is an anti-angiogenesis agent? |
|
Definition
Bevacizumab
(antibody to vascular endothelial growth factor) |
|
|
Term
| What is the 2nd most common primary CNS tumor? |
|
Definition
|
|
Term
| What are the subtypes of Meningiomas? |
|
Definition
Low Grade (most common)
Atypical
Malignant |
|
|
Term
| What tumors have a greater indicence in women and peak at 45 years? |
|
Definition
|
|
Term
| Where are the common locations of Meningiomas? |
|
Definition
Convexity
parasagittal
optic sheath |
|
|
Term
| What are meningiomas derived from? |
|
Definition
arachnoid membrane
(arachnoid cap cells) |
|
|
Term
| What are the Imaging characteristics of Meningiomas? |
|
Definition
Dark of T1 (hypointense)
Bright on T2 (hyperintense)
Enhance with contrast
May see calcifications on CT
Dural Tail |
|
|
Term
| What is the 3rd most common primary CNS tumor? |
|
Definition
|
|
Term
| Where are Pituitary tumors most commonly derived? |
|
Definition
| From the Anterior Pituitary |
|
|
Term
| How are Pituitary Tumors Classified? |
|
Definition
Microadenomas
<1cm - most are hormone secreting
Macroadenomas
>1cm most don't secrete hormones, compress, cause hypofunction |
|
|
Term
| What are the Signs and Symptoms of Pituitary Tumors? |
|
Definition
Headache
Endocrine Dysfunction
Visual Field Defects |
|
|
Term
| How are Pituitary Tumors diagnosed? |
|
Definition
MRI with/without contrast
Serum hormone levels
Visual Fields Testing |
|
|
Term
| What drugs are dopamine agonists for prolactinomas? |
|
Definition
| Bromocriptine and Pergolide |
|
|
Term
| What drug is used for GH secreting Tumors? |
|
Definition
Octreotide
(Somatostatin analogue) |
|
|
Term
| Where do Acoustic Neuromas originate? |
|
Definition
Schwann cells surrounding the vestibular parotion of CN VIII
Most commonly in the internal auditory canal or cerebellopontine angle |
|
|
Term
| What do Acoustic Neuromas present with? |
|
Definition
Hearing Loss
Tinnitus
Headache
dizziness
facial numbness or weakness |
|
|
Term
| What are Bilateral acoustic neuromas pathognomic of? |
|
Definition
|
|
Term
| What does Primary CNS Lymphoma commonly affect? |
|
Definition
| Leptomeninges and Deeper periventricular brain parenchyma |
|
|
Term
| What is Diagnostic of Primary CNS Lymphoma? |
|
Definition
MRI w/ Contrast - multiple uniformly enhancing masses with little or no edema
LP - monoclonal population of B-cells in the CSF |
|
|
Term
| If there is ocular involvement in Primary CNS Lymphoma what test can be done? |
|
Definition
| Vitrectomy for tissue diagnosis |
|
|
Term
| What is the Prognosis of Primary CNS Lymphoma based on different Treatments? |
|
Definition
Steroids alone - months
Radiation - 1 year
Chemotherapy and Radiation - 3-4 years
Cure possible in younger patients |
|
|
Term
| What is the most common CNS Tumor? |
|
Definition
|
|
Term
| What Tumors metastasize to the brain? |
|
Definition
Small/Non-Small cell lung cancer (50%)
Breast Cancer (15-20%)
Melanoma (10%)
Colon Cancer (5%)
Renal Cell Carcinoma |
|
|
Term
| What is the most common brain metastases in Males? |
|
Definition
| Small/Non-small cell lung cancer |
|
|
Term
| What is the most common brain matastases in females? |
|
Definition
|
|
Term
| What is the Prognosis for Brain matastases? |
|
Definition
Untreated - 4 weeks
Steroids only - roughly 8 weeks
Other Treatment - may increase up to 12 months |
|
|
Term
| What types of Lesions can produce Ring enhancing lesions in the Brain? |
|
Definition
Metastasis
Abscess
CVA
High Grade Glioma
Lymphoma
Demyelination |
|
|
Term
| What are the Signs and Symptoms of Spinal Cord Tumors? |
|
Definition
1) Pain
2) Weakness plus UMN/LMN motor findings
3) Paresthesias |
|
|
Term
| How are Spinal Cord Tumors Classified? |
|
Definition
Base on Origin
(primary or Metastatic)
Based on Location
(Extra-dural, Intra-dural extramedullary, Intra-dural intramedullary) |
|
|
Term
| What are the majority of Extradural tumors from? |
|
Definition
Spinal metastases
(breast, lung, prostate, renal, lymphoma, sarcomas) |
|
|
Term
| What are Intradural Extramedullary tumors? |
|
Definition
Schwannomas
Neurofibromas
Meningiomas |
|
|
Term
| What are Intradural intramedullary tumors? |
|
Definition
Astrocytomas (most common in peds)
Ependymoma (most common in adults)
Hemangioblastoma |
|
|
Term
| What are the treatment options for Spinal Cord Tumors? |
|
Definition
Surgery - more challangeing
Radiation
Chemotherapy - less well studied |
|
|
Term
| What is the 2nd most common cancer of childhood? |
|
Definition
CNS tumors
(after hematologic malignancies) |
|
|
Term
| What is the most common solid tumor in the pediatric population? |
|
Definition
|
|
Term
| How are Pediatric CNS tumors classified? |
|
Definition
| underlying histopathological features and/or anatomic location |
|
|
Term
| What is the most common malignant brain tumor of childhood? |
|
Definition
|
|
Term
| Where are most (50-60%) of Pediatric Brain Tumors located? |
|
Definition
|
|
Term
| Where do young children have a relatively high occurrence of malignancies? |
|
Definition
| cerebellum and brain stem |
|
|
Term
| What is the tumor distribution for children <1 year? |
|
Definition
Supratentorial
Gliomas
Teratomas
PNET
Choroid Plexus |
|
|
Term
| What is the tumor distribution for Ages 1-11? |
|
Definition
Infratentorial > Supratentorial
Medulloblastoma
Ependymoma
Brain Stem Glioma |
|
|
Term
| What is the Tumor Distribution for >11years? |
|
Definition
Infratentorial = Supratentorial
Gliomas
PNET/Medulloblastoma
Germ cell |
|
|
Term
| What syndrome has a germline mutation of the PTCH receptor and associated with medulloblastoma? |
|
Definition
|
|
Term
| What Tumors are Li-Fraumeni Syndrome associated with? |
|
Definition
|
|
Term
| What syndrome is associated with Optic pathway gliomas? |
|
Definition
|
|
Term
| What tumors are associated with Neurofibromatosis 2? |
|
Definition
|
|
Term
| What syndrome is associated with Subependymal Giant Cell Astrocytoma? |
|
Definition
|
|
Term
| What tumors are associated with Von Hippel-Lindau Syndrome? |
|
Definition
|
|
Term
| What germline mutation and tumors are associated with Turcot's Syndrome? |
|
Definition
APC gene
Gliomas and Medulloblastoma |
|
|
Term
| What Non-genetic causes are associated with tumors in pediatrics? |
|
Definition
Radiation Exposure
Other Cancers
Immunodeficiency
(Wiskott-Aldrich Syndrome, Ataxia-Telangiectasia, Acquired immunodeficiency) |
|
|
Term
| Where would a tumor be located if thought, reasoning, behavior, or memory is impaired? |
|
Definition
|
|
Term
| Where would a Tumor be located if Right visual fields or Emotions are impaired? |
|
Definition
|
|
Term
| Where would a tumor be located if speech and motion sensation are impaired? |
|
Definition
|
|
Term
| Where would a tumor be located if a patient is unable to process abstract concepts? |
|
Definition
|
|
Term
| Where would a tumor be located if Spatial relationships are impaired? |
|
Definition
|
|
Term
| Where would a tumor be located if Balance and coordination are impaired? |
|
Definition
|
|
Term
| Where would a tumor be located if hearing is impaired? |
|
Definition
|
|
Term
| What Localized findings would be present with a Supratentorial tumor? |
|
Definition
|
|
Term
| What Clinical manifestations would present with a Midline tumor? (Hypothalamic/Optic, Craniopharyngioma, Pineal) |
|
Definition
Endocrinopathies
--Diabetes insipidus
--Growth disorders
--Decreased vision
--Visual field deficits
Signs or Increase ICP |
|
|
Term
| What Clinical manifestations present with Infratentorial tumors? |
|
Definition
| Signs and Symptoms related to Increased Intracranial Pressure |
|
|
Term
| What Clinical Manifestations are associated with Brain Stem Tumors? |
|
Definition
Cranial Nerve Deficit:
--Double Vision
--Slurred Speech
--Swallowing disorders
--Crossed-weakness
Occasional hydrocephalus |
|
|
Term
| What is the ICP classic Triad? |
|
Definition
AM Headaches
Nausea and Vomiting
Lethargy |
|
|
Term
| What do around 1/3 of posterior fossa tumor patients require? |
|
Definition
| Permanent VP shunts following surgical debulking |
|
|
Term
| What type of Tumors is Radiotherapy appropriate for Pediatrics? |
|
Definition
Low Grade Astrocytoma
High Grade Astrocytoma
Medulloblastoma/PNET
Ependymoma
Germ Cell Tumors
Craniopharyngioma
Brainstem Glioma |
|
|
Term
| What tumor is characterized by corticospinal long tract signs (weakness/hemiparesis), ataxia, and difficulty with lateral gaze (abducens paresis)? |
|
Definition
| Diffuse intrinsic pontine gliomas |
|
|
Term
| When should Chemotherapy be used in pediatric cancer patients? |
|
Definition
Intermediate and High Risk Medulloblastoma
High Grade Gliomas/PNET/ATRT
Germ Cell Tumors
Low Grade Astrocytomas
(Optic pathways/hypothalamus) |
|
|
