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182 Cards in this Set
- Front
- Back
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Which is more prevalent: intracranial or spinal tumors?
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Intracranial (10-17/100,000 vs 1-2)
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What is the ratio of primary to metastatic tumors in the CNS?
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50% : 50%
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What percent of childhood cancers are CNS?
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20%
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Childhood CNS tumors vs Adult?
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Childhood: 70% are infra-tentorial
Adult: 70% are supra-tentorial |
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Main Clinical Findings with CNS Tumors
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HA**
Seizures** Vomiting Altered Mental Status Focal Neurological Deficits (tumor location) |
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Main Radiological Findings w/ CNS Tumors?
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Location
Mass Effect (tumor + edema) Diagnostic Patterns Secondary Effects |
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What are Diagnostic Patterns?
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Ring Enhancement
Cyst w/ mural node |
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What are secondary effects of CNS tumors?
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Bleed
Herniation Hydrocephalus (pediatric) |
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What is not likely to happen with Primary CNS Tumors? and why?
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Distant Metastasis
Not accessible to modes of metastasis other than possibly CSF or subarachnoid seeding |
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What is the Direct Effect of benign CNS tumors?
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Infiltration of functional brain in non-resectable areas
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what is the indirect effect of benign CNS tumors?
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When mass effect leads to herniation
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What are the primary roles of the pathologist in relation to CNS tumors?
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Histo Typing
Histo Grading |
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1 step to CNS Classification?
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Primary or Metastatic
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Two common types of systemic cancer to metastasize to CNS?
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Carcinoma of Lung and Breast
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Classifying Primary CNS Tumors
2 broadest categories |
Parenchymal (brain or SC)
Meningeal (meningiomas) |
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Two types of Parenchymal CNS Tumors?
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Neuroepithelial
Non-Neuroepithelial |
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Three Types of Neuroepithelial, Parenchymal CNS Tumors?
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Glial
Neuronal Embryonal/Primitive |
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2 Types of Non-Neuroepithelial, Parenchymal Tumors
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Lymphoma
Germ Cell |
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WHO Histo Grading Criteria
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Degree of Differentiation
Cellularity Nuclear Pleomorphism Mitotic Activity MICROVASCULAR PROLIFERATION Necrosis |
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Three Types of Gliomas
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Astrocytoma
Oligodendroglioma Ependymoma |
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Most common type of Primary CNS Tumors?
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Gliomas
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Different Grades of Astrocytomas
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Grade I: Pilocytic
Grade II: Diffuse/Fibrillary Grade III: Anaplastic Grade IV: Glioblastoma |
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Different Grades of Oligodendrogliomas?
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Grade II: Oligodendroglioma
Grade III: Anaplastic |
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Different Grades of Ependymomas?
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Grade I: Myxo-papillary
Grade II: Ependymoma Grade III: Anaplastic |
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More specifically, what is the most common primary CNS Tumor?
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Glioblastoma
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Approximate Median Survival
Oligodendroglioma vs. Astrocytoma |
O: 10yrs
A: 7 yrs |
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Approximate Median Survival
Anaplastic Astrocytoma Glioblastoma Multiforme |
III: 4
IV: 1 |
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Percents of Primary CNS tumors based on location
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85% Intracranial
15% Spinal |
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Location of Intracranial Primary CNS Tumors in Adults
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70% Supratentorial
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Common-->Rare
Supratentorial Primary CNS Tumors in Adults |
Gliomas
Meningiomas Schwannomas |
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Location of Intracranial, Primary, CNS tumors in kids
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70% infratentorial
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3 types of Primary CNS tumors in kids?
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Pilocytic Astrocytoma
Medulloblastoma Ependymoma |
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Common to Rare Spectrum of Spinal Primary CNS Tumors
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Schwannoma
Meningioma Gliomas (ependymoma>Astrocytoma) (reverse order of intracranial) |
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Diagnostic Immunohistochemical Markers:
Glial Tumors |
Glial Fibrillary Acidic Protein (GFAP)
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Diagnostic Immunohistochemical Markers:
Neuronal tumors |
synaptophysin
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Diagnostic Immunohistochemical Markers:
Schwann Cell |
S-100
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Diagnostic Immunohistochemical Markers:
Lymphoid |
LCA
CD20 (B) |
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Prognostic Immunohistochemical Markers of proliferation
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Ki67 (MIB-1)
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Prognostic Immunohistochemical Markers for Glioblastomas?
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p53
EGFR |
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What is the most common pediatric CNS tumors?
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Pilocytic Astrocytoma
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Common sites of Piloctyic Astrocytoma
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Cerebellum (most common)
Hypothalamic Optic Nerve |
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Typical morphology of Piloctyic Astrocytoma
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Circumscribed
Indolent (so close to benign) |
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Imaging of Piloctyic Astrocytoma
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*Cyst w/ Mural Nodule*
Maybe Focal Enhancement |
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Prognosis for Piloctyic Astrocytoma
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Depends on surgical accessibility
(so hypothalamic is bad) |
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Microscopic Diagnostic Factors of Piloctyic Astrocytoma
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Biphasic Tumor: compact/spongy
Bipolar (long, hair-like cells) Rosenthal Fibers (compact area) Eosinophilic Granular Bodies (spongy bodies) |
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What can be seen microscopically with Piloctyic Astrocytoma, but is not prognostic?
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Microvascular Proliferation (usually pericystic)
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DIFFUSE (FIBRILLARY) ASTROCYTIC TUMORS
Age? |
Mostly Adults (30-40), but any age
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DIFFUSE (FIBRILLARY) ASTROCYTIC TUMORS
Location? |
Mostly Cerebral Hemispheres, but any location
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DIFFUSE (FIBRILLARY) ASTROCYTIC TUMORS
Symptoms |
HA (inc ICP)
Seizures Focal Deficits |
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DIFFUSE (FIBRILLARY) ASTROCYTIC TUMORS
% of astrocytic tumors |
10-15%
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Common sites of Piloctyic Astrocytoma
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Cerebellum (most common)
Hypothalamic Optic Nerve |
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Typical morphology of Piloctyic Astrocytoma
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Circumscribed
Indolent (so close to benign) |
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Imaging of Piloctyic Astrocytoma
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*Cyst w/ Mural Nodule*
Maybe Focal Enhancement |
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Prognosis for Piloctyic Astrocytoma
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Depends on surgical accessibility
(so hypothalamic is bad) |
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Microscopic Diagnostic Factors of Piloctyic Astrocytoma
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Biphasic Tumor: compact/spongy
Bipolar (long, hair-like cells) Rosenthal Fibers (compact area) Eosinophilic Granular Bodies (spongy bodies) |
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What can be seen microscopically with Piloctyic Astrocytoma, but is not prognostic?
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Microvascular Proliferation (usually pericystic)
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DIFFUSE (FIBRILLARY) ASTROCYTIC TUMORS
Age? |
Mostly Adults (30-40), but any age
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DIFFUSE (FIBRILLARY) ASTROCYTIC TUMORS
Location? |
Mostly Cerebral Hemispheres, but any location
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DIFFUSE (FIBRILLARY) ASTROCYTIC TUMORS
Symptoms |
HA (inc ICP)
Seizures Focal Deficits |
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DIFFUSE (FIBRILLARY) ASTROCYTIC TUMORS
% of astrocytic tumors |
10-15%
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DIFFUSE ASTROCYTOMA (II)
Imaging? |
MRI=no contast enhancement
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DIFFUSE ASTROCYTOMA (II)
Gross Morphology? |
Poorly defined outlines
Obliterated gray-white junction |
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DIFFUSE ASTROCYTOMA (II)
Microscopic Findings |
Mildly Increased Cellurlarity, nuclear atypia
Closely mimics gliosis Usually infiltrative (around neurons) Microcysts +/- |
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Favorable Prognostic Factors for Astrocytomas
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Age <40
High Karnofsky Status Oligodendroglial Component |
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Unfavorable Prognostic Factors for Astrocytomas
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Large Tumor (>6cm, crosses midline)
Critical Location (poor resectability, neuro deficits at presentation) Gemistocytes >20% |
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ANAPLASTIC ASTROCYTOMA (III)
Peak Age? |
10 years after grade II (so 40-50?)
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ANAPLASTIC ASTROCYTOMA (III)
Imaging? |
Contrast Enhancement
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ANAPLASTIC ASTROCYTOMA (III)
Microscopic Findings |
Increased Cellularity w/ nuclear pleomorphism
*Mitotic Activity*** *Tumor shouldn't have necrosis or microvascular prolif* |
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Most common primary brain tumor?
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Glioblastoma
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GLIOBLASTOMA (IV)
peak age? |
50-70
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GLIOBLASTOMA (IV)
Imaging |
Irregular or *Ring Enhancement**
Perilesional Edema + Mass Effect Corpus Callosum Involvement (butterfly glioma*) |
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GLIOBLASTOMA (IV)
Gross Morphology |
Irregular
Hemorrhage Necrosis |
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GLIOBLASTOMA (IV)
Microscopic Findings |
Obvious Anaplastic Features +
Necrosis +/- pseudopalisading Significant Microvascular Prolif Gemistocytic Cells more common |
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What the crap is pseduopalisading?
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its when the cells appear to line up next to each other around a necrotic area
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What is the difference between a Primary GLIOBLASTOMA and a Secondary one?
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Primary means it progressed so fast it first presented at a GLIOBLASTOMA. Secondary means it progressed there from another grade
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which is more common? primary or secondary?
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Primary 95%
Secondary 5% |
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Primary vs Secondary GLIOBLASTOMA
Mean Age? |
Primary: 62
Secondary 45 |
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Primary vs Secondary GLIOBLASTOMA
Mean duration of onset? |
primary < 6 months
secondary: 5 yrs |
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Primary vs Secondary GLIOBLASTOMA
MRI and Gross Morphology |
Primary: Larger and more Necrosis
Secondary: variable |
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Primary vs Secondary GLIOBLASTOMA
Genetics? |
Primary: EGFR amplification
Secondary: P53 mutation |
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Primary vs Secondary GLIOBLASTOMA
Median Survival? |
Primary < 6 months
Secondary < 12 months |
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OLIGODENDROGLIOMA
Age? |
Adults between 4th and 5th decade
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OLIGODENDROGLIOMA
Location? |
Usually cerebral hemispheres
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OLIGODENDROGLIOMA
Grossly? |
Soft
Gelatinous Few Cysts Calcification Infiltrates Gray and White Matter |
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OLIGODENDROGLIOMA
Genetics? |
LOH of 1p and 19q
(except in peds) |
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OLIGODENDROGLIOMA
Microscopic Findings? |
Relatively Homogeneous
Round, uniform nuclei Perinuclear Halo Micro-calcification Infiltrates Gray matter |
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OLIGODENDROGLIOMA
Grades? |
most are grade II
fewer are grade III |
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OLIGODENDROGLIOMA
Favorable prognostic factors |
Younger Age
High Karnofsky Status More complete Resection LOH of 1p and 19q |
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OLIGODENDROGLIOMA
Unfavorable prognostic factors |
Ring Enhancement
Histologic Anaplasia (grade III) p53, p16 mutations |
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EPENDYMOMA
Location? |
Kids: Intracranial (>4th ventricle)
Adults: Intraspinal (central canal) |
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EPENDYMOMA
MRI and Gross Morphology |
Intraventricular (sometimes parenchymal)
Demarcated, but when in 4th ventricle can compress BS |
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EPENDYMOMA
Microscopic Findings |
Perivascular Pseudorosettes
Ependymal True Rosettes |
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EPENDYMOMA
Grading |
Most are grade II
Fewer grade III |
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EPENDYMOMA
Grade III differences |
High Cellularity
Mitoses |
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What is a perivascular pseudorosette?
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rosette formation area around a centrally located vessel.
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What is a ependymal true rosette?
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rosette formation attempting to form a ventricle
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EPENDYMOMA
Variants? |
Myxopapillary Ependymoma (grade I)
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Where is the Myxopapillary Ependymoma found?
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Filum Terminale
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EPENDYMOMA
Poor Prognosticators |
Age < 3
Location (posterior fossa near BS) Histological Anaplasia (III) CSF Spread |
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Grade IV Embryonal Tumors
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1. Medulloblastoma
2. Supratentorial Primitive Neuroectodermal Tumors (sPNET) 3. Atypical Teratoid Rhaboid Tumor (AT/RT) |
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Most common Embryonal Tumor in kids?
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Medulloblastoma
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MEDULLOBLASTOMA
Age and Location |
First 2 decades
Cerebellum (infratentorial) |
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MEDULLOBLASTOMA
Typical clinical findings |
Increased ICP (CSF obstruction)
Gait Abnormalities |
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MEDULLOBLASTOMA
Grossly? |
usually a midline mass (vermis)
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MEDULLOBLASTOMA
Microscopically? |
Small, Embryonal, blue cell tumor
Super Cellular Horner Wright Rosettes +/- neuronal differentiation |
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MEDULLOBLASTOMA
5yr Survival |
60-70%
(they respond well to radiation) |
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MEDULLOBLASTOMA
bad prognosticator |
CSF Spread
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MEDULLOBLASTOMA
Marker? |
Synaptophysin
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MEDULLOBLASTOMA
Favorable Prognosticators? |
Histo: Neuronal Differentiation
Molecular: Beta catenin, TrkC (don't worry) |
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MEDULLOBLASTOMA
Unfavorable Prognosticators |
Age < 3
Incomplete Resection Metastatic at presentation Anaplastic (histo) Molecular Stuff Supratentorial |
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Types of Neuronal Neoplams?
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Ganglioglioma
Dysembryoplastic neuroepithelial tumor (DNT) Central Neurocytoma |
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Ganglioglioma
Age, location, presentation |
Pediatric
Temporal Lobe Seizures |
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Ganglioglioma
Grading |
Usually Grade I (benign)
Rarely Grade III needing RT |
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Dysembryoplastic Neuroepithelial Tumor
age, location, presentation |
Pediatric
Temporal Lobe Seizures |
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Dysembryoplastic Neuroepithelial Tumor
Grading and Prognosis |
Usually Grade I (benign)
Excellent prognosis |
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Central Neurocytoma
age, location |
Young Adults
Lateral Ventricles |
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Central Neurocytoma
Histo? |
Looks like Oligodendrogliomas
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Central Neurocytoma
Grading? |
Mostly Indolent (grade II)
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What marker for all the neuronal tumors?
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synaptophysin
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Which Primary CNS Tumors are infiltrative?
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Astrocytomas
Oligodendrogliomas Medulloblastomas |
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Which Primary CNS Tumors are Expansile?
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pilocytic astrocytomas
ependymoma |
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Which Primary CNS Tumors can spread in the CSF malignantly?
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Glioblastoma
Medulloblastoma |
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Which Primary CNS Tumors can spread via CSF just based on location?
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pilocytic astrocytoma
ependymoma |
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Best Rx for Primary CNS Tumors?
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Minimal Safe resection and radiation.
Chemo just for some genetically favored ones |
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What type of lymphoma is Primary CNS Lymphoma?
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NHL
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Clinically what could be Primary CNS Lymphoma's claim to fame?
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Most common CNS tumor in T-cell immuno-suppressed patients (HIV, etc)
AIDS defining illness |
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Primary CNS Lymphoma
Grossly |
Solitary/Multiple
Hemorrhage Necrosis |
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Primary CNS Lymphoma
microscopically |
Large B-Cell NHL
Angiocentric Growth |
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Primary CNS Lymphoma
response to Rx? |
Poor compared to nodal lymphoma
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Primary CNS Lymphoma
metastasis? |
Node, Marrom, etc
but rare and late |
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Germ Cell Tumors
Incidence? |
In west: 0.5% of all primary intracranial tumors
In Asia: 2-3% |
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Germ Cell Tumors
Age? |
Kids and young adults
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Germ Cell Tumors
Sites? |
Pineal
Suprasellar |
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Germ Cell Tumors
microscopically |
Germinoma
Teratoma |
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MENINGIOMA
defiintion |
usually benign (I) derived from mengiothelial calls of arachnoid
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MENINGIOMA
Age and Gender |
Adults
More Females |
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MENINGIOMA
Sx's |
non localizing syndroms
slow growth |
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MENINGIOMA
Grossly |
Lobulated
Attached to Dura Thicken or Invade Skull Usually sharp interface with brain |
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MENINGIOMA
Genetically |
Loss of 22q
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MENINGIOMA
subtypes |
Subtypes: meningothelial, fibroblastic, transitional
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MENINGIOMA
Grading |
Typically Grade I
Grade II: atypical mitoses, clear cell Grade III: median survival < 2 yrs, anaplastic |
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MENINGIOMA
prognosis? |
Extent of Resection
Histological Grade Brain Invasion Progestone receptor status |
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MENINGIOMA
microscopic |
Meningothelial Whorl
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METASTATIC TUMORS
most common in who? |
elderly
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METASTATIC TUMORS
most common starts? |
Leukemias and lymphomas (yougn pts)
Carcinoma of Lung Carcinoma of Breast Malignant Melanoma |
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METASTATIC TUMORS
grossly |
Sharp Interface w/ brain
Leptomeningeal carcinomatosis |
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METASTATIC TUMORS
microscopic? |
resemble tumors at primary sites
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Neurocutaneous Syndromes
AKA? |
Phakomatoses
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Neurocutaneous Syndromes
etiology? |
INHERITED
Auto Dom |
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Neurocutaneous Syndromes
Characterized by? |
Hamartomas
Neoplams (particularly in CNS) |
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Types of Neurocutaneous Syndromes?
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Neurofibromatosis Type 1 and 2 (NF1 and NF2)
Tuberous Sclerosis Von Hippel Lindau Syndrome (VHL) |
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TYPE 1 NEUROFIBROMATOSIS (NF1)
AKA |
Von Reckinghousen's
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TYPE 1 NEUROFIBROMATOSIS (NF1)
inheritance and frequency? |
Auto Dom
1 in 3000 |
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TYPE 1 NEUROFIBROMATOSIS (NF1)
Main Lesions |
Neurofibroma (solitary or plexiform)
Optic Gliomas (pilocytic astrocytoma) Pigmented nodules of Iris (Lisch nodules) Hyperpigmented Cutaneous Macules (cafe au lait spots) |
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TYPE 1 NEUROFIBROMATOSIS (NF1)
When is there a higher risk of malignant transformation? |
Plexiform neurofibroma
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TYPE 1 NEUROFIBROMATOSIS (NF1)
Tumor Suppressor Gene? |
NF1 (neurofibromin)
17q11 |
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TYPE 1 NEUROFIBROMATOSIS (NF1)
why don't all carriers of mutated gene present the same? |
incomplete penetrance
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TYPE 2 NEUROFIBROMATOSIS
inheritance and frequency |
Auto Dom
1 in 50,000 (rarer) |
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TYPE 2 NEUROFIBROMATOSIS
Neoplastic Lesions |
Bilateral Acoustic Schwannomas (most common)
Meningiomas Spinal Ependymoma |
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TYPE 2 NEUROFIBROMATOSIS
Non-neoplastic Lesions |
Schwannosis (nodules in cord)
Meningioangiomatosis Glial Hamartomas |
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TYPE 2 NEUROFIBROMATOSIS
Genetics? |
NF2 (merlin) gene on 22q12
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What does a Plexiform Neurofibroma transform malignantly into?
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Malignant Peripheral Nerve Sheath Tumor
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How else can you get Malignant Peripheral Nerve Sheath Tumor?
|
they can arise de-novo
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Mutations associated with transformations to Malignant Peripheral Nerve Sheath Tumor?
|
NF1
p53 p16 |
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Malignant Peripheral Nerve Sheath Tumor
Grossly? |
Invasion
Necrosis |
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Malignant Peripheral Nerve Sheath Tumor
marker? |
S100
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TUBEROUS SCLEROSIS
characterized by? |
hamartomas and benign lesions in many locations
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TUBEROUS SCLEROSIS
typical presentation? |
seizures
mental retardation |
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TUBEROUS SCLEROSIS
Typical Sites? |
Brain
Skin Visceral |
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TUBEROUS SCLEROSIS
What is typically seen in brain |
Cortical Tubers
**Subependymal Giant Cell Astrocytoma (SEGA) in ventricles** |
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TUBEROUS SCLEROSIS
Cortical Tubers: grossly? |
Enlarged Gyri w/ indistinct gray/white junction
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TUBEROUS SCLEROSIS
Cortical Tubers: microscopically? |
big, bizzare, haphazardly arranged neurons
Balloon Cells Gliosis |
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What is the most common CNS neoplasm in TS?
|
SEGA
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How does SEGA typically present?
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Worsening Seizures
Increased ICP |
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TUBEROUS SCLEROSIS
Genetics? |
TSC1 (hamartin) 9q34
TSC2 (Tuberin) 16q13 |
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VON HIPPEL LINDAU SYNDROME
inheritance |
Auto Dom
VHL Gene on chromosome 3 |
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VON HIPPEL LINDAU SYNDROME
Typical neoplasms? |
Hemangioblastomas in cerebellum and retina (BENIGN!)
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VON HIPPEL LINDAU SYNDROME
Imaging? |
Cyst w/ Mural Nodule (like pilocytic astrocytoma)
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VON HIPPEL LINDAU SYNDROME
Microscopically? |
Mimics metastatic renal cell carcinoma...may make erythopoeitin-->polycythemia
-Stromal cells w/ vacuolated cytoplasm |
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Effects of Radiation on the Brain:
Early |
Edema
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Effects of Radiation on the Brain:
Latter (6month-1year) |
Radiation Necrosis due to vascular damage
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Effects of Radiation on the Brain:
Remote |
Intellectual Decline (kids)
Radiation Induced Tumors (gliomas, meningiomas, sarcoma) |
