Brain Tumors

A. Lieberman
11.07.06

I. important concepts
A. most neoplasms have counterparts among normal cells (ex. astrocytoma and astrocyte)
B. gliomas kill (astrocytomas, glioblastomas, ependymomas, oligodendrogliomas) because they infiltrate brain
tissue (exeptions include pilocytic astrocytomas and some ependymomas
C. cranial vault has little room for tumors or edema
1. tend to cause herniation
2. herniated tissue may cut off its own blood supply
3. herniation may compress other vital structures
D. gliomas are graded for prognosis and therapy
1. qualitative criteria are available to distinguish low and high grade gliomas
2. based on necrosis, cellularity, mitoses, and vascular endothelial proliferation
3. WHO grading scale
a. grade 1: essentially benign, cured by resection
b. grade 2: low grade
c. grade 3: middle grade
d. grade 4: high grade
E. most schwannomas and meningiomas are benign
F. unfavorable anatomy can make a killer out of a benign tumor or cyst
II. specific tumors
A. gliomas
1. astrocytic tumors
a. astrocytoma
i. tumors are derived from astrocytes
I. abundant cell processes
II. express glial fibrillary acidic protein (defines cell as being of astrocytic lineage)
ii. infiltrative growth pattern - grow diffusely
iii. even after surgical resection tumor cells remain
iv. diffuse fibrillary astrocytoma
I. grade 2 lesion (lower grade)
II. 3-10 year post-operative survival
III. characterized by increased nuclear pleomorphism, increased cellularity, no mitosis
b. anaplastic astrocytoma
i. grade 3 lesion (higher grade)
ii. 2-3 year post-operative survival
iii. characterized by mitosis, increased nuclear pleomorphism, increased cellularity
c. glioblastoma multiforme
i. common adult brain tumor derived from astrocytes
ii. grade 4 lesion (highest grade)
iii. less than 1 year post-operative survival
iv. diffusely infiltrates brain and fills cranial vault
v. characteristically ring enhancing on imaging
vi. microscopic features
I. cellular features:
A. cellular pleomorphism - crazier cells of astrocytic lineage
B. mitoses
C. vascular endothelial proliferation
D. necrosis
II. pseudopallisading necrosis
III. vascular endothelial proliferation (lumen is compressed, may be occluded by fibrin
thrombi)
2. oligodendroglioma
a. glioma derived from oligodendrocytes
b. most are grade 2 lesions, but a few are anaplastic (Grade 3)
c. better prognosis than same grade astrocytoma
d. diffusely infiltrates brain
e. microscopic features - fried egg appearance
i. round nuclei
ii. perinuclear halos
 iii. fine branching blood vessels - chicken wire vasculature
f. characteristic chromosomal abnormality
i. deletions of chromosomes 1 and 19
ii. predict longer survival
3. ependymoma
a. tumor of ependymal cells
b. most are grade 2 or 3
i. grading is not a good predictor of outcome
ii. age, extent of resection, and location are also factors
c. most commonly occur in posterior fossa or spinal cord
d. tumors may invade less than astrocytomas or oligos (premium on gross total removal at surgery)
e. microscopic features
i. cells express GFAP - same protein expressed by astrocytic cells
ii. characteristic arrangements of cells
I. perivascular rosettes
II. ependymal rosettes
iii. round to oval nuclei
iv. EM shows cilia and basal bodies (characteristic of ependymal cells)
4. pilocytic astrocytoma
a. most occur in children and are grade 1 lesions
b. surgically resectable tumors with little brain infiltration
c. often located in posterior fossa or near 3rd ventricle
d. often form a cyst with a mural nodule
e. microscopic features
i. long, hair-like astrocytic cells (lots of pink cytoplasm and long wavy processes)
ii. Rosenthal fibers - strings of GFAP
B. medulloblastoma
1. poorly differentiated and multipotential tumor
2. high grade (grade 4)
3. common brain tumor of children
4. often located in cerebellum or roof of 4th ventricle
5. commonly spread along CSF pathway (cells are not very adherent so they spread easily)
6. more radiosensitive than glioblastoma
7. microscopic features
a. small cells wit high nuclear to cytoplasmic ratio (gives tumor blue appearance on H & E stain)
b. brisk mitotic activity
c. may form Homer Wright rosettes - rosettes with fibrillary cores and no lumen (contrast with other
rosettes with lumen or blood vessel at center)
C. primary brain lymphoma
1. occurs sporadically and in immunocompromised patients
2. forms a large intraparenchymal mass - may be multicentric
3. microscopic features
a. often shows angiocentric growth
b. tumor cells have high nuclear to cytoplasmic ratio
c. cells are discohesive - shows infiltration of adjacent brain tissue by individual cells
d. most often stain with B-cell markers
4. lymphomas which arise outside CNS may infiltrate meninges, but usually do not form masses
D. meningioma
1. tumor of meningotheilal cells (of the arachnoid)
2. most do not infiltrate brain (grade 1 tumors)
3. most often attached to the dura
4. may contain psammoma bodies (laminated calcifications)
5. cellular characteristics
a. round, oval nuclei
b. not much mitotic activity
c. synsytial growth pattern and whorls
E. metastatic tumors
1. carcinoma and melanoma are common hematogenous spread
2. often multiple
3. form distinct margin with adjacent brain
F. nerve sheath tumors - most are grade 1 tumors
1. schwannoma
 a. tumor of schwann cells
b. also called neurilemmoma and acoustic neuroma
c. do not invade brain - can be peeled off of axon resulting in functional outcomes after resection
d. attached to but do not invade 8th cranial nerve or spinal nerve routes
e. bilateral nerve schwannomas: NF2
f. microscopic features
i. Antoni A and B: biphasic growth patter with less dense and more dense
I. A: more dense patterns (pallisades: barricade bodies)
II. B: less dense (foamy cells)
ii. Verocay bodies
iii. basement membrane around each tumor cell
2. neurofibroma
a. tumor of schwann cells and fibroblasts
b. most occur outside the craniospinal vault
c. have axons within the tumor (axon is trapped and cannot be extracted from tumor)
d. NF1
i. multiple neurofibromas
ii. plexiform neurofibromas with involvement of multiple nerve fascicles
iii. malignant peripheral nerve sheath tumor occurs in NF1 patients with plexiform neurofibromas
I. grade 3 and 4 lesion
II. arises from neurofibroma usually in patients with NF1