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SYSTEM
PART 2: CNS
TUMORS
VOLTAIRE C. YABUT,M.D. DPSP
CNS TUMORS
intracranial 10-17 : 100,000 population
Intraspinal 1-2: 100,000 population
Primary ½-¾
Metastatic – remainder
Gemistocytic
Neoplastic astrocytes
Brightly eosinophilic cell body, stout processes
ASTROCYTOMA
GEMISTOCYTIC ASTROCYTOMA
GLIOMAS
Astrocytomas
Categories:
Glioblastoma (Glioblastoma Multiforme)
variable gross appearance
Firm white to soft and yellow
Well demarcated with infiltration beyond outer margin
Microscopic:
Similar to Anaplastic
Necrosis – “pseudopalisading”
Vascular or endothelial proliferation – “glomeruloid body”
GLIOBLASTOMA
GLIOMAS
Astrocytomas
WHO Grading
Grade I/IV Pilocytic
Grade II/IV Well differentiated astrocytomas
Grade III/IV Anaplastic
Grade IV/IV Glioblastoma
GLIOMAS
Astrocytomas
Molecular Genetics
Inactivation of p53
Overexpression of PDGF-A and its receptors
Transition to higher grade associated with additional
disruption of tumor suppressor genes, the RB genes,
p16/CDKNZA gene, putative tumor suppressor on
chromosome 19q
GLIOMAS
Astrocytomas
Clinical Features:
Presenting symptoms depend in part on location of
tumor and growth rate
Well-differentiated remain static, progress slowly
Mean survival 5 years
Anaplastic present with rapid deterioration, very poor
prognosis
Current treatment (resection, radiotx, chemotx)
8 – 10 mos surivival
< 10% alive after 2 yrs
GLIOMAS
Astrocytomas
Categories:
Pilocytic
Children and young adults
Cerebellum, floor and wall of 3rd ventricles, optic nerves,
cerebral hemispheres
GLIOMAS
Astrocytomas
Categories:
Pilocytic
Morphology
often cystic, with mural nodule in the cyst wall
If solid, well circumscribed, less frequently infiltrative
GLIOMAS
Astrocytomas
Categories:
Pilocytic
Microscopic:
Bipolar cells with long thin “hairlike” processes that are GFAP (+)
Rosenthal fibers
Eosinophilic granular bodies
Microcysts
Increase no. of blood vessels
Necrosis and mitosis uncommon
Narrow infiltrative border
PILOCYTIC ASTROCYTOMA
GLIOMAS
Astrocytomas
Categories:
Pilocytic
Clinical
Grow very slowly
Cerebellar tumors treated with resection
Rarely have p53 mutations or other changes found in
diffuse fibrillary
GLIOMAS
Astrocytomas
Categories:
Pleomorphic Xanthoastrocytoma
Relatively superficial
Temporal lobe
Children and young adults
Long history of seizures
GLIOMAS
Astrocytomas
Categories:
Pleomorphic Xanthoastrocytoma
Microscopic:
neoplastic occ bizarre astrocytes
nuclear atypia can be extreme and may suggest high grade
astrocytoma
abundant reticulin deposits
relative circumscription
chronic inflammatory cell infiltrates
absence of necrosis and mitotic activity
GLIOMAS
Astrocytomas
Categories:
Pleomorphic Xanthoastrocytoma
Clinical Features:
Better prognosis than astrocytomas
Ave 5 – 10 years survival
GLIOMAS
Ependymomas
Arise next to ependyma-lined ventricular system
First 2 decades – 4th ventricle
5 – 10% of primary brain tumors
Adults – most common in spinal cord
GLIOMAS
Ependymomas
Morphology:
In 4th ventricle, solid or papillary masses
Intraspinal, sharply demarcated
Microscopic:
Cells with regular, round to oval nuclei with abundant granular
chromatin
Dense fibrillary background
Rosettes, canals
Perivascular pseudorosettes
GFAP (+)
EPENDYMOMA
GLIOMAS
Ependymomas
Morphology:
Well-differentiated – WHO Grade II/IV
Anaplastic ependymomas – WHO Grade III/IV
Slow growing
Glial components occ become anaplastic and progress rapidly
Present with seizure disorder
WHO grade I-II/IV
NEURONAL TUMORS
Slow growth
Good prognosis
NEURONAL TUMORS
Medulloblastoma
Atypical Teratoid/Rhabdoid Tumor (AT/RT)
POORLY DIFFERENTIATED
NEOPLASMS
Medulloblastoma
Predominantly in children
Exclusively in the cerebellum
Largely undifferentiated
Morphology
Midline of cerebellum
Lateral in adults
Medulloblastoma
Microscopic:
Extremely cellular, sheets of anaplastic cells
Little cytoplasm
Hyperchromatic nuclei, elongated or crescent
shaped
Mitosis abundant
(+) Ki-67 markers
Express neuronal (neurosecretory granules, Homer
Wright rosettes) and glial (GFAP) phenotypes
MEDULLOBLASTOMA
POORLY DIFFERENTIATED
NEOPLASMS
Medulloblastoma
Molecular Genetics:
Loss of short arm of chromosome 17
Clinical features:
Highly malignant
Prognosis dismal in untreated patients
radiosensitive
Better survival following complete resection
75% 5-year survival rate for total excision and radiation
POORLY DIFFERENTIATED
NEOPLASMS
Schwannoma
Neurofibroma
Malignant
Peripheral Nerve Sheath Tumor
(MPNST, Malignant Schwannoma)
PERIPHERAL NERVE SHEATH
TUMORS
Schwannoma
Neural crest-derived Schwann cell
Assoc with Neurofibromatosis type 2
Schwannoma
Microscopic:
Mixture of two growth patterns
Antoni A – pattern of growth of elongated cells with
cytoplasmic processes arranged in fascicles in areas
of moderate to high cellularity with little stromal
matrix
Antoni B – pattern of growth,less densely cellular
with loose meshwork of cells along with microcysts
and myxoid changes
SCHWANNOMA
PERIPHERAL NERVE SHEATH
TUMORS
Schwannoma
Clinical Features:
Most common in cerebellopontine angle, attached to
vestibular branch of 8th cranial nerve
Tinnitus and hearing loss
Lisch nodules
Tuberous sclerosis
Autosomal-dominant
Hamartomas
Cortical tubers
Subependymal hamartomas
Benign neoplasms involving the brain and other tissues
Renal angiomyolipomas
Retinal glial hamartomas
Pulmonary lesions
Cardiac rhabdomyomas
Cysts in liver, kidneys, pancreas
Cutaneous lesions e.g. Angiofibromas,Shagreen patches, ash-leaf
patches, subungal fibromas
FAMILIAL TUMOR SYNDROMES