Tumors Flashcards
1
Q
Types of gliomas
A
- astrocytoma (juv. PA, low-grade, anaplastic)
- ependymoma
- oligodendroglioma
+ GBM
2
Q
What’s a tumor of neurons called?
A
Neuronal tumor
3
Q
What’s a mixed cell tumor of neuronal and glial cells called?
A
Ganglioglioma
4
Q
Nerve tumors
A
Schwannoma and Neurofibroma
5
Q
Other tumors that can occur intracranially
A
- meningioma
- hemangioma
- choroid plexus tumors
- pineal tumor
- germinoma (from nests of germ cell tumors)
- primary CNS lymphoma
- sellar tumors (ie pituitary)
- suprasellar tumors (ie craniopharyngioma)
- cystic lesions
6
Q
How to distinguish primary vs. metastatic cancer in brain?
A
- find extracranial cancer (whole body scan)
- metastatic will often be multiple lesions (ring-enhancing)
- mets will have significant edema, out of proportion to the nodule size
7
Q
Signs and symptoms of brain tumors
A
- tumor HA
- focal signs: papilledema; seizures; focal neuro deficits
8
Q
What labs to do when a patient has a brain tumor?
A
x
9
Q
Treatment plan for patients with brain tumor
A
treating symptoms: - steroids - if seizures: anticonvulsants (*use non-CYP inducers; don't use prophylactically) definitive treatment: - observation - surgery - radiotherapy - chemo
10
Q
Most common primary brain neoplasia
A
- Infiltrative Astrocytoma - 42%
- GBM - 40%
- all the rest are
11
Q
EBV is linked to
A
CNS lymphoma in transplant patients
12
Q
Brain lesions in Tuberous sclerosis (TSC on 9/16)
A
- Subependymal giant cell astrocytoma
- cortical tubers
- glioma
13
Q
NF1 vs. NF2 tumors
A
NF1 (17) - optic nerve gliomas, glioblastoma, neurofibromas in skin derived from NCCs
NF2 (22) - meningioma, schwannoma (acoustic neuroma), ependymoma
14
Q
Li Fraumeni tumors
A
- glioma (MC astro), medulloblastoma
- p53 mutation
15
Q
Feature of tumor headache
A
- 2/2 increased ICP or local irritation
- may show laterality, resembles migraine
- suspect tumor especially if it’s worse in the AM because overnight the veins get congested from lying down
- suggests tumor if vomiting immediately follows acute onset HA (suggesting ICP)
16
Q
Types of herniation
A
- subfalcine - ACA
- diencephalic - drowsiness, impaired gaze, Horner’s
- uncal - ipsi CN3, hemiparesis
- upward through tentorium - ipsi CN3, contra hemiparesis
- tonsillar - BP changes, weakness, respiratory disturbance, Horner’s
17
Q
What brain tumor type can be diagnosed with lumbar puncture?
A
Primary CNS lymphoma - malignant cells can be in CSF
18
Q
T1 vs. T2 weighted imaging
A
T1 = water is dark (so is tumor) T2 = water/tumor is bright
19
Q
Normally, edematous contrast enhancing tumors are fast-growing and malignant. What are 2 contrast-enhancing BENIGN tumors?
A
- Pilocytic astrocytoma
2. Meningioma
20
Q
Gliomas Grades 1-4
A
Grade 1: JPA; indolent, usually non-infiltrative;
Grade 2: low-grade astro
Grade 3: anaplastic astro
Grade 4: GBM
21
Q
Temozolamide
A
- alkylating agent, causes DNA damage (that is repaired by MGMT)
- SOC for malignant glioma
- given during and after radiation
- prolongs median survival by 3mo
22
Q
Meningioma
A
- diffuse enhancement
- slow-growing
- dural tail
- benign tumors, but may want to remove them +/- radiotherapy (higher grade would need radiotherapy too)
23
Q
Pituitary tumor
A
- mostly derived from anterior pituitary
- micros are usually functional; macros are usually non-functional
- sx: HA, vision, endo
- Tx: observe, surgery, RT, chemo
24
Q
Acoustic neuroma
A
- Schwann cells; most common site is CN8, also CN5 or others
- also commonly present at the cerebellopontine angle
- may present with hearing loss, ataxia, trigeminal neuralgia/neuropathy, and ataxia
- if bilateral –> NF2
25
PCNSL
- steroids are oncolytic; avoid
- from trafficking B-LCs
- prognosis: mo for steroids, 1yr for RT, 3-4yr for chemo+RT (MTX works well)
- recall link between EBV and immunosuppression
26
Brain metatasases
- as devastating as high grade glioma but more common
- mostly from the LUNG; also breast, melanoma, colon, RCC
- will see single/multiple discrete enhancing nodules, edema out of proportion to nodules
- tx: WBRT with goal to prolong life w/o incurring neuro deficit; untx'd survival is ~4wk, tx may prolong to 12mo
27
Ring-enhancing lesion in the brain - what could it be?
- metastasis
- abscess
- CVA
- high grade glioma
- lymphoma
- demyelination (open ring enhancement)
28
Spinal cord tumors
- can be extradural or intradural; if intradural can be intramedullary (astro, ependymo, hemangio) or extramedullary (schwanno, neurofibro, menigio)
- present as pain, weakness (UMN/LMN findings), paresthesias
- tx: surgery, RT, chemo
29
Risk factors for pediatric brain tumors
1. ionizing radiation
2. other tumors (esp. kidney, RB)
3. immune suppression (Wiskott-Aldrich, Ataxia-telan; acquired ID)
4. familial conditions
30
Clinical presentation of PBTs
Depends on location:
- supratentorial --> localized sx, seizures, hemiparesis
- midline --> endocrinopathies, VF defect, signs of inc. ICP/hydrocephalus
- infratentorial --> inc. ICP
- brainstem --> CN deficits, hydrocephalus
31
How to diagnose PBT
- H&P
- imaging
- biopsy
- CSF cytology/look for mets (+/- bone scan)
32
How to treat PBT
- surgery (histo dx and reduce tumor burden; won't work for brainstem gliomas/DIPG)
- radiation: >7yo, min. >3yo
- chemo: for high-grade tumors or optic
33
Types of PBTs
- astrocytoma (LG is MC; JPA can be anywhere)
- medulloblastoma
- supratentorial PNETs
- pineoblastomas
- brainstem glioma
- diffuse intrinsic pontine glioma
- ependymoma
- neurofibroma
*high grade gliomas and GBM are RARE in kids
34
Most common anatomic sites of PBTs (in order)
1. Infratentorial
2. Supratentorial
3. Midline
35
Ependymoma
- from epithelial cells of ventricular system
| - in kids it's usually IC
36
Medulloblastoma
- MC malignant PBT (primary)
- invasive, rapidly growing tumors of primitive nerve cells (or cerebellar stem cells)
- occurs infratentorial/posterior fossa
- metastasize via CSF
- heterogenous enhancement on MRI
- Homer-Wright rosettes
- if WNT mutation good prog; if Shh may be good/intermediate
37
Craniopharyngioma
- rare, usually suprasellar neoplasm, which may be cystic, that develops from nests of epithelium derived from Rathke's pouch
- Rathke's pouch is an embryonic precursor of the anterior pituitary
38
If the kid is
supratentorial
- glioma
- teratoma
- PNET
- choroid plexus
39
If the kid is 1-11yo, the tumor is most likely where and what type?
infratentorial
- medulloblastoma
- ependymoma
- brainstem glioma
40
If the kid is >11yo, the tumor is most likely where and what type?
Infra/Supratentorial
- glioma
- PNET
- medulloblastoma
- germinoma
41
Turcot syndrome
- medulloblast-omas
- GBM
- associated with germline APC mutation
42
Cowden syndrome
- dysplastic gangliocytoma of the cerebellum
| - PTEN mutation leads to hyperactivity of mTOR
43
Classic triad for symptoms of inc. ICP
- morning headache
- N/V
- lethargy
* infants: bulging fontanel and sundowning
44
How to histologically recognize PA?
- long red strands
- eosinophilic Rosenthal fibers
- hyalinization of blood vessels
- pilocytic refers to the hairlike projections from neoplastic cells
45
Mutation in the MAPK path and what tumors they cause
- BRAF activating mutation --> JPA in cerebrum
- RAF fusion/activation --> JPA in cerebellum
- loss of NF1 inhibition on KRAS --> NF
- BRAF v600e GOF mutation --> extracerebellar
46
Brainstem glioma
- uniformly fatal in ~2yr
- non operable
- radiation, supportive care helps
47
Diffuse intrinsic pontine glioma
- dismal prognosis
- CST signs (hemiparesis), ataxia, CN6-8 deficits
- on MRI will see obscuration of pons w/o enhancement and engulfing basilar artery - characteristic MRI signs so no biopsy needed
- non-operable; radiation helps
48
Macro vs. Microadenoma of the Pituitary
Macro: >10mm; Micro:
49
Pituicytoma
- rare, low-grade glioma derived from pituicytes of the posterior pituitary
- non-secreting
- present as mass lesion similarly to meningioma
