CNS Tumors Flashcards
CNS tumors are the ____ most common malignancy in children
CNS tumors are the 2nd most common malignancy in childhood, and the most common solid cancer of childhood
In adults 70% is ______tentorial
In children, 70% is _____tentorial
In adults 70% is SUPRAtentorial
In children, 70% is INFRAtentorial
WHAT TYPE OF TUMOR GROWTH IS THIS?
EXPANSILE
SHARP border between the tumor and surrounding tissue
WHAT TYPE OF TUMOR GROWTH IS THIS?
INFILTRATIVE
SINGLE TUMOR CELLS percolate thorugh brain parenchyma and surround normal structures
Primary tumors arise from brain ____ or ________.
classified according to cell type of origin
In adults, most common (3):
In children, most common (4):
Secondary tumors are….
PRIMARY tumors arise from brain parenchyma or its coverings
in ADULTS: MENINGIOMA, SCHWANNOMA, GLIOBLASTOMA
(msg)
in children: pilocytic, astrocytoma, ependymoma, medulloblastoma
(pame)
Secondary tumors are METASTATIC, spread to brain through the bloodstream
***more common that primary tumors
Astrocytoma:
Age groups affected:
Location:
Sx:
MOST COMMON GLIOMA
Age groups affected: ALL
Location: ANYWHERE
Sx: depends on location and grade
Astrocytoma: Grade 1
example:_____, (major locations, age group affected, MRI findings, major histological features)
Growth:
Prognosis and Rx:
Astrocytoma: Grade 1 - least malignant
example: PILOCYTIC astrocytoma
* @ cerebellum and brain stem of children and young adults*
* MRI: cyst with solid mural nodule that enhances on scan w/contrast*
* Histo: piloid cells, biphasic architecture (compact and microcytic areas), rosenthal fibers*
Growth: slow growing
Prognosis and Rx: low proliferative potential and possibility of cure follwoing surgical resection alone
What type of cell is represented in the H&E stain?
Pilocytic astrocytoma (Astro Grade 1)
Piloid cells with hair-like processes
What does the H&E stain represent?
Pilocytic astrocytoma (astro grade 1)
biphasic architecture = compact (left arrow) and microcytic (right arrow) areas
What does the H&E stain represent
Pilocytic Astrocytoma (grade 1)
Rosenthal fibers = eosinophilic corkscrew inclusions
Astrocytoma grading
Grade II: NO ……, infiltrative in nature and _____ level of proliferation
Grade III: _____ [benign/malignant] tumors, without _____ proliferation or _______. RX: _______ and/or ________
Grade IV: ie) __________, most malignant astrocytoma. _____ progression and prognosis _______.
Astrocytoma grading
Grade II: NO mitosis, necrosis or vascular proliferaiton. Infiltrative in nature with LOW level of proliferation
Grade III: MALIGNANT tumors, no microvascular proliferation, no necrosis . RX: adjuvant radiation and/or chemotherapy
Grade IV: GLIOBLASTOMA, most malignant astrocytoma. RAPID progression and poor prognosis/fatal outcome
Glioblastoma:
Arises in: _______
Infiltration:
Typical lesion on MRI: ______, because it can______
Contrast CT:
Gross:
Histo:
Prognosis:
Glioblastoma
Arises in: cerebral hemispheres
Infiltration: diffusely infiltrative, can be multicentric
MRI: BUTTERFLY LESION because it can cross the corpus callosum
Contrast CT: RING ENHANCING LESIONS w/ edema, mass effect
Gross: central/yellow necrosis, red/brown hemorrhage, cystic changes, poorly defined borders
Histo: ENDOTHELIAL cell hyperplasia, pleomorphic malignant astrocytes, HI mitosis, area of necrosis with pseudopalisading of cells at the periphery (pictured), GFAP positive
POOR PROGNOSIS
Oligodendroglioma:
rare _____ tumor/glioma of ______ with _____ prognosis in comparision to astrocytoma of comparable grade
Loc:
associated sx:
CT:
Histo: …quack…secondary structures=
Oligodendroglioma: rare MALIGNANT tumor (glioma) of OLIGODENDROCYTES with BETTER PROGNOSIS than astrocytoma of comparable grade
Loc: DENSELEY CELLULAR tumor in FRONTAL LOBE
associated sx: SEIZURES
CT: intratumoral calcification
Histo: perinuclear halos “FRIED EGG” appearance and CHICKEN WIRE CAPILLARY PATTERN; secondary structures PERINEURONAL SATELLITOSIS = tumor cells aggreagate around structurels like capillaries, neurons, pia, etc
Ependymoma:
Arises from _______ lining cells
Common site:
Common age:
Imaging:
Histology:
Ependymoma:
Arises from EPENDYMAL LINING CELLS
Common site: 4th ventricle, adults usually spinal
Common age: freq in children but may present in any age
Imaging: discrete, exophytic, contrast enhancing
Histology: PERIVASCULAR PSEUDOROSETTE, TRUE EPENDYMAL ROSETTE
What does the histology represent?
EPENDYMOMA PERIVASCULAR PSEUDOROSETTES
= tumor cells surround a blood vessel
well delineated, moderately cellular glioma with monomorphic nuclear morphology and perivascular pseudorosettes and ependymal rosettes
What does the histology represent?
TRUE EPENDYMAL ROSETTES: TUMOR CELLS SURROUND EMPTY CENTRAL LUMEN
Embryonal Tumor: Medulloblastoma
Arises from undifferentiated _________ cells
______ [benign/malignant] tumor
LOC:
MRI:
Gross:
Histology: …..rosettes….
Embryonal Tumor: Medulloblastoma
Arises from undifferentiated NEUROECTODERMAL cells
MALIGNANT tumor
LOC: CEREBELLUM IN CHILDREN, may be hemispheric in adults, spreading along the CSF pathway
MRI: DISCRETE CONTRAST ENHANCING MASS IN THE 4TH VENTRICLE
Gross: nodular fleshy tumor in 4th ventricle
Histology: sheets of undifferentiated cells with scanty cytoplasm and dark staining nuclei (small blue cell tumor)
Rosettes have a central core composed of delicate neuropil = HOMER-WRIGHT ROSETTES
What does the histology represent?
Undifferentiated cells (small blue cells) with scanty cytoplams and dark staining nuclei
HOMER-WRIGHT Rosettes (tumor cells surrounding neuropil)
- densely packed calls with round to oval or carrot shaped, highly hyperchromatic nuclei surrounded by scanty cytoplasm. Neurblastic rosettes are typical but not a constant feature.*
- **Rosettes in retinoblastoma have cells arranged around empty lume = FLEXNER-WINTERSTEINER ROSETTES*
Meningioma:
Arises from ______ cells
Location:
Sx:
RX and prognosis
MRI:
Gross:
Histo:
Meningioma: tumor arising from the coverings
Arises from ARACHNOID cells
Location: EXTRA-AXIAL (ouside of parenchyma), COMMON IN CONVEXITIES AND PARASAGITTAL AREAYS
Sx: often asymptomatic byt can present with seizures or focal neurological deficits
RX and prognosis: rsecetion usually currative +/- radiation
MRI: well-circumscribed superficial mass w/bright, uniform contrast enhancement and dural tail
Gross: discrete globular solid mass ATTACHED TO DURA - compression of brain
Histo: psammoma bodies (laminated calcifications), whorls, fibroblastic variant, syncytial appearance
Craniopharyngioma:
______ [benign/malignant] tumor of ______ [children/adult]
Derived from:
CT:
Gross:
Histo:
Can be confused with: _____ (associated sxs-3)
Craniopharyngioma:
BENIGN tumor of CHILDREN
Derived from: Rathke’s pouch remants/Rathke’s Clef (which also gives rise to a benign cyst = Rathke’s cleft cyst)
CT: suprasellar mass with tumor calcifications
Gross: sagittal brain section, cystic mass with grossly visible calcifications/cyst has oily appearance
Histo: complex epithelium with compact and loose areas and wet keratin almost look like SQC
Can be confused with: PITUITARY ADENOMA (endocrine dysfunction, visual symptoms, hydrocephalus, calcifications common)
Schwannoma:
[benign/malignant] tumor of _______ cell origin
Involved: ____ or _____ nerves
Common at (2):
–> SX
Bilateral acoustic neuromas found in:
Gross:
Histo:
Schwannoma
BENIGN tumor of SCHWANN cell origin
Involved: CRANIAL or SPINAL nerves
Common at (2): CEREBELLOPONTINE ANGLE and CN VIII (acoustic neuroma)
–> SX: LOSS OF HEARING, TINNITUS
Bilateral acoustic neuromas found in: NEUROFIBROMATOSIS TYPE 2 (NF2)
Gross: globular well circumscribed mass in cerebellopontine angle; yellow coloration due to lipid accumulation, focal hemorrahge
Histo: elongated spindle cells, pallisades and areas that are anuclear, denase area called ANTONI A and the loose cystic area called ANTONI B. Stains with S100 protein
Metastatic Carcinoma
Top 5 organs to which cancer originates:
Which spread via meningeal carcinomatosis:
Which spread hemorrhagically:
Gross:
Metastatic Carcinoma
Lung > Breast > Melanoma > Kidney > GI tract
Which spread via meningeal carcinomatosis: Lung and breast
Which spread hemorrhagically: lung, melanoma, kindey
Gross: frequently multiple, sharply demarcated lesions (INVADE BUT DO NOT INFILATRATE), in typical locations (cortx-white matter junction)
Neurofibromatosis Type I (NF1)
Gene:
Neurofibromin-_______ activating protein
Common findings (5):
_____ neurofibroma is pathognomonic of NF1
Gross: Bag of ______, invovled multiple _________
Micro:
Neurofibromatosis Type I (NF1)
Gene: 17q11.2
Neurofibromin-GTPase activating protein
Common findings: neurofibromas, acoustic schwannomas, optic nerve gliomas, Lisch nodules, cafe-au-lait spots
PLEXIFORM NEUROFIBROMA pathognomonic of NF1 (PIC)
Gross: BAGS OF WORMS, invovles multiple nerve fasicles
Micro: expanded nerve fascicles of variable cellularity and abundant mucin
HIGH FREQUENCY OF MALIGNANT TRANSFORMATION
NEUROFIBROMATOSIS TYPE 2 (NF2)
Gene:
NF2 invovled what protein?
associations (2):
NEUROFIBROMATOSIS TYPE 2 (NF2)
Gene: 22q12, tumor suppressor gene
MERLIN- cytoskeletal protein
associations: BILATERAL acoustic schwannomas (bilateral CP angle tumors- pic), multiple meningiomas
TUBEROUS SCLEROSIS:
Genes and associated proteins:
Classic triad:
Associated tumors:
Key findings:
TUBEROUS SCLEROSIS:
SECOND MOST COMMONE HEREDITARY SYNDROME OF THE NS
Genes/proteins: TSC1 (gene 9q) –> hamartin, TSC2 (16q)-tuberin
Classic triad: adenoma sebaceum, seizures, mental retardation
Associated tumors: CORTICAL TUBERS and SUBEPENDYMAL hamartomas (including subependymal giant cell astrocytoma-SEGA)
[Other tissues: facial angiofibromas, subungual fibromas, hypomelanotic macules (ash-leaf spots), shagreen patches on the forehead, cardiac rhabdomyomas, renal angiomylipomas, pulmonary lymphangiomymatosis]
Von Hippel-Landau Disease:
VHL is a ________ gene on chromosome _____
Protein in _____-ligase complex that targets transcription factor ______ for degradation
Tumors show ______ and expression of _____ –> drives expression of _____ and other growth factors
Lesions:
Von Hippel-Landau Disease:
VHL is a TUMOR SUPPRESSOR gene on chromosome 3P
Protein in UBIQUITIN-ligase complex that targets transcription factor hypoxia-inducible factor (HIF) for degradation
Tumors show LOSS OF VHL and expression of HIF–> drives expression of VEGF and other growth factors
Lesions:
CNS hemangioblastomas (cerebellum)
RENAL CYSTS and RENAL CELL CARCINOMA
retinal angiomatosis, pheochromocytoma, pancreatic cysts, endolymphatic sac tumor of middle ear
