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64 Cards in this Set

  • Front
  • Back
What aspect of a CNS tumor is the primary predictor of biologic behavior (prognosis)?
Tumor Histology Appearance
In general, adult CNS tumors are located ______
supratentorial
In general, child CNS tumors are located ______
infratentorial
Most common CNS tumor in a child (<20 yo)?
Astrocytoma
How common are metastatic CNS tumors in children
Rare!

(50% are metastatic in adults)
-altered cognition, personality changes, expressive aphasia (esp. the posterior left anterior frontal gyrus); motor disturbances (esp precentral gyrus --> contrlateral deficits)

-These signs and symptoms would result from a tumor in the ___ lobe
frontal lobe
-seizures; emotional changes; receptive aphasia

-These signs and symptoms would result from a tumor in the ___ lobe
temporal lobe
- sensation abnormalities (contralateral)

--These signs and symptoms would result from a tumor in the ___ lobe
parietal lobe
If a tumor were to cause impaired appreciation of shape, size, weight, texture (astereognosia) you would know it was a ______ -type tumor
cortical-type tumor
Thalamic Syndrome is caused by a CNS tumor on or near the thalamus and causes ______
spontaneous pain
A tumor is causing visual field deficits. You know it is affecting the _____ lobe
occipital lobe
What is the most common primary CNS tumor in adults?
GBM
Most adult CNS tumors are supratentorial and located in the _______
cerebrum
Most child CNS tumors are infratentorial and located in the _____ or _________
cerebellum

4th ventricle
GBM and oligodendroglioma are more commonly seen in what age group?
Adults
Medulloblastoma and pilocytic astrocytoma are more commonly seen in what age group?
Children (20 or younger)
You are looking at a CT/MRI with contrast and notice ring-enhancing lesions. What are those?
**Ring enhancing lesions**

—discovered on CT/MRI with contrast and present as a **white line around a darker region** --> due to enhancement from increased blood flow at the periphery
Name the ring-enhancing lesions of the CNS.

"MAGIC DR"
M-etastatic
A-bscess
G-lioma
I-nfarction
C-ontusion

D-emyelinating Diseases
R-adiation necrosis
You are giving a contrast MRI to a patient that was determined to have a brain lesion. There is no post-contrast enhancement. What does this mean?
No post contrast enhancement = the BBB is intact

-no leakage of contrast into the lesion
Vasogenic vs. Cytotoxic vs. Interstitial Cerebral Edema:

-increased BB permeability -->extracellular

• Damaged vessels-inflammation, neovascularization

• White matter > gray matter; steroids helpful

• Tends to have associated edema**
Vasogenic Edema
Vasogenic vs. Cytotoxic vs. Interstitial Cerebral Edema:

-impaired Na/K ATPase -->intracellular

• Hypoxia/ischemia; intact BBB

• Gray matter = white matter; steroids not helpful
Cytotoxic Edema
Vasogenic vs. Cytotoxic vs. Interstitial Cerebral Edema:

-transependymal fluid shift --> extracellular

• Due to ­ CSF pressure (hydrocephalus)
Interstitial Cerebral Edema
Identify the primary cell types that give rise to CNS neoplasms
• Glial cells
1. Astrocytes**
2. Oligodendrocytes**
3. Ependymal cells**

• Neurons (*Robbins)

• Other neuroepithelial cells – choroid plexus**

• Embryonal (primitive neuroepithelial cells) – Medulloblastoma**

• Cells of the meninges (“arachnoid cap cells”) – Meningioma**

• Hematolymphoid (lymphocytes) – Lymphoma**

• Germ cells** – can arise outside gonads; tend to arise midline
Identify the neuroepithelial cells that can give rise to CNS tumors
• Glial cells
1. Astrocytes**
2. Oligodendrocytes**
3. Ependymal cells**

• Neurons (*Robbins)

• Other neuroepithelial cells – choroid plexus**

• Embryonal (primitive neuroepithelial cells) – Medulloblastoma**

• Cells of the meninges (“arachnoid cap cells”) – Meningioma**
WHO Grade I-IV:

- may be curative with surgery

• Low proliferative activity

• Typically well-circumscribed; good potential for surgical cure
WHO Grade I
WHO Grade I-IV:

-typically survive > 5 yrs

• Low proliferative activity

• Infiltrative; often recur; may progress to higher grade
WHO Grade II
WHO Grade I-IV:

-typically survive ~ 2-3 yrs

• Greater proliferative potential; more aggressive behavior

• Generally treated with adjuvant chemo/XRT
WHO Grade III
WHO Grade I-IV:

-typically < 1 yr survival

• **High proliferative** activity; tumor necrosis; “glomeruloid” endothelial proliferation/neovascularization

• Typically “not curable
WHO Grade IV
A 10-yo boy has had persistent headaches for the past three months. On P.E., he is afebrile. He has an ataxic gait. CT scan of his head shows a 4-cm cystic mass in the right cerebellar
hemisphere. Cerebral ventricles are enlarged. A lumbar puncture is performed: CSF protein is elevated, but glucose is normal. Neurosurgery is performed and the mass is removed and evaluated.

The mass has a thin cyst wall and contains gelatinous material and a mural nodule. Microscopic analysis shows GFAP-positive cells with long, hair-like processes.

The most likely diagnosis is …
Pilocystic Astrocytoma

**MOST COMMON CNS TUMOR OF CHILDREN**
BUZZWORDS:

Cystic with mural nodule

One of most common pediatric brain tumors **CHILDREN **

Cerebellum

Rosenthal fibers—“carrots”
Pilocystic Astrocytoma
 WHO Grade

**Fibrillary** histologic features most common

~ 15% of astrocytomas

Peak ~30-40 y (adults)** YOUNG ADULTS**

**Frontal white matter; disrupts gray-white junction

5 yr survival with total resection + XRT = 70%

**50% will progress to higher grade over time

**Micro**
• No mitoses; hemorrhage is relatively rare

- May see microcalcifications

•↑ p53 staining (mutated form of p53)**
Diffuse Infiltrating Astrocytoma - Adult

(No post-contrast enhancement = BBB intact)
WHO Grade III**

- Increased cellularity, cytologic atypia (pleomorphism)**

-Mitotic figures are present; +/- endothelial proliferation; no necrosis; rare calcification

- Immunoperoxidase stains for proteins present in actively cycling cells can indicate growth fraction of tumor (e.g., MIB-1 stain)

~30% of astrocytomas

Peak age 40-60 yo **OLDER ADULTS**—>likely d/t progression of lower grade lesions)
Anaplastic Astrocytoma
WHO Grade IV**

~ 50% of astrocytomas

**Most frequent primary brain tumor in adults**

Peak age: 45-60 yo **OLDER ADULTS**

-Most common location: deep frontotemporal

-Molecular: ­ EGFR (Chromosome 7)—epidermal GF expression receptor
Glioblastoma Multiforme (GBM)

• Primary GBM – arise de novo (from nothing, brand new tumor, arose as GBM)

**Older patients

-EGFR mutations/amplifications early


• Secondary GBM – arise via progression from lower-grade glioma

**Younger patients

-p53 mutations early & ­PDGF-A receptor signaling late
**Imaging**

• Postcontrast injection CT MR studies, many are characterized by a bright (“enhancing”) ring

• Ring surrounds a region of hypodensity (central necrosis)

**Micro**

•Palisading necrosis— dense cellularity, striking pleomorphism, and zones of coagulative necrosis lined by “palisading” tumor cells

Endothelial Hyperplasia-complex, “glomeruloid” quality of microvascular proliferation
GBM
Micro**

• “Chicken-wire”** vasculature—capillary with branching pattern

• **“Fried egg” cytology—yolk – nucleus, w/ surrounding cytoplasm

• Calcifications (~80%)

• Uniform, round nuclei and clear perinuclear halos
Oligodendroglioma
WHO Grade II – ??? (better 5 yr survival)**

WHO Grade III – anaplastic**

~10% gliomas

Peak age 35-40 yo **YOUNG ADULTS**

- Often mixed with astrocytomas

- Tumors with chromosome 1p/19q deletions**


Micro**

• “Chicken-wire”** vasculature—capillary with branching pattern

• **“Fried egg” cytology—yolk – nucleus, w/ surrounding cytoplasm

• Calcifications (~80%)

• Uniform, round nuclei and clear perinuclear halos
Oligodendroglioma
- WHO Grades I-III

< 5-9% of 1° CNS tumors

**Bimodal age distribution**
(2) peak ages 1-5yo and <35 yo ** TWO TARGET AGE GROUPS**

**Location** varies with age group*

• Children
~4th ventricle, lateral ventricles

• Adults
~Spinal cord, 4th ventricle

- Account for ~60% of intramedullary spinal cord tumors

**Micro**
• True and pseudorosettes
Ependymoma
**Ependymoma**

- Cytoplasmic processes of ependymal tumor cells condense about blood vessels to form _____
pseudorosettes
**Ependymoma**

- True ependymal rosette contains a well-defined
central lumen
This is a tumor of the choroid plexus and may cause noncommunicating hydrocephalus
Choroid Plexus Papilloma
Are choroid plexus papillomas more common in adults or children?

Where are they normally located in adults and children?
Most common in CHILDREN!!

Children = lateral ventricles

Adults = 4th ventricles
Choroid Plexus carcinomas are very rare and occur mostly in ________ (age group)
Children
- WHO Grade IV (highly aggressive)*

• Small, round(ish) blue cells

- One of most common CNS tumors in children--> cerebellum
** CHILDREN**


**Micro**

• Rosettes and pseudorosettes

•**Homer Wright rosettes**
consist of tumor cell nuclei disposed in circular fashion about tangled cytoplasmic processes --> these structures are indicative of differentiation along neuronal lines

• Cellular enlargement, often prominent nucleoli, and pronounced mitotic and apoptotic activity = features of this aggressive tumor
Medulloblastoma

(“medulloblast” – immature cells that can become glia or neurons)
- Generalized or localizing presentation

~ 15% of primary intracranial tumors

- Peak age 40-60 yo ** OLDER ADULTS **

**Monosomy chromosome 22**
(22q12 = NF2 gene --> merlin)

- Incidence increased following radiation and in people with NF2

-Arise from arachnoid cap cells (meningothelial cells)

**Well demarcated, firm/rubbery, broad dural base = characteristic**

• MRI may demonstrates thickened and abnormally enhancing dural “tails” extending from the lesional borders —a finding suggestive, though not diagnostic, of this tumor entity
**Meningioma**


- 90% cranial; 9% spinal; 1% ectopic

- Multiple in 9%
These are uncommon, except when seen in immunocompromised patients**

- Most are of **B-cell origin**

- EBV virus positive
Primary CNS Lymphoma
- Immunocompromised patients

- B-cell origin

- EBV (+)

• Most often high-grade and relatively poorly responsive to chemotherapy

• May respond initially to corticosteroids

• Typically more responsive to radiation tx
Primary CNS Lymphoma
A germ cell tumor in the CNS is most likely a ________

***
testicular seminoma
Where are germ cell tumors in the CNS most likely found?

**
Midline --> pineal, suprasellar regions

germinoma = testicular seminoma
Most common sources of metastatic CNS tumors?

***
- Lung (most commonly)
- Breast
- Melanoma
- Kidney (RCC)
- GI tract
- (Choriocarcinoma ,rare tumor but often w/ mets to brain)
What do metastatic CNS tumors look like?

***
**Most often multiple**

- Ring-enhancing, sharply demarcated lesions

- Surrounded by zone of edema

**Gray-white junction of cerebrum (where blood is richest, thus where they will most likely travel to)
neuromas, Schwannomas, Neurofibromas, Malignant peripheral nerve sheath tumors (MPNST) are all tumors of the _____
PNS
**non-neoplastic** mass lesion, not malignant—associated w/ trauma)

- Tangled mass of axons, reactive proliferations of
Schwann cells, fibroblasts

**Painful **
Neuroma
- WHO Grade I

-see MULTIPLE in patients with NF2

- In general, occur as solitary tumors, firm and encapsulated

• Antoni A (dense fibrillary) tissue
**Verocay bodies**: palisades of elongated, bipolar cells around pink fibrillary stroma

• Antoni B (loose reticulated) tissue—looser stroma, fewer cells, and myxoid change
Schwannomas
Patients with NF2 often have multiple _______
***Schwannomas***

• Bilateral vestibular Schwannomas

**Deletion/mutations in NF2 gene, 22q12 (merlin)**

Patients likely present:
-Tinnitis, gait disturbance, vertigo, nausea
Can schwannomas be removed surgically?
Yes, they are encapsulated!
-WHO Grade I

- Composed of Schwann cells, fibroblasts, collagen, reticulin

**Unencapsulated** and infiltrate nerves—not thought to occur intracranially

• Cannot easily surgically remove neoplasm without damage to nerve
Neurofibromas
Can Neurofibromas be removed surgically?
No, they are uncapsulated and often infiltrate nerves!
50% of people with a Malignant peripheral nerve sheath tumors (MPNST) have what disease??
NF1
- WHO Grade III or IV

- Arise de novo or via progression of nerve sheath tumors

• Neurofibromas, possibly Schwannomas

~ 10-15% of neurofibromas in NF1 become malignant

• ~ 75% recur and cause death

Very difficult to tx/cure
Malignant peripheral nerve sheath tumors (MPNST)
**Chromosome 17**

Inclusion criteria – at least 2 of the following:

• Six café au lait spots

• Two neurofibromas

• One plexiform neurofibroma

• Axillary or inguinal freckling

• Osseous lesion (e.g., sphenoid dysplasia)

• Optic glioma

• Two *Lisch nodules** (iris hamartomas
NF1 = von Recklinghausen’s NF-peripheral

90% of NF (MOST COMMON form)
 Central type
- Less common, with later onset

- AD

- Chromosome 22

- Associated with bilateral vestibular Schwannomas, meningiomas
NF2
Paraneoplastic syndromes:

- Antibodies against presynaptic voltage-gated Ca2+ channels of skeletal muscle, and any electrical membrane

**Small cell lung Ca** -Ab association

- Presents as mysthenia gravis presents**

• More commonly associated with thymic hyperplasia/thymoma
Lambert Eaton Syndrome
Paraneoplastic Syndromes

- Destruction of Purkinje cells -

- Anti-Yo** antibodies

- Breast, ovarian carcinoma
Cerebellar degredation
Paraneoplastic Syndromes:

**Anti-Hu** antibodies (small cell lung Ca, lymphoma)

- Tend to be related to some underlying immunologic mechanism*

- Associated with some solid tumor of an organ—breast, lung, ovary—and sometimes a lymphoma
Sensory Neuropathy