Documente Academic
Documente Profesional
Documente Cultură
BRAIN TUMORS
Could be characterized/described in several ways:
1. Clinically the neoplasmic mass because of
its size or location could cause symptoms of mass
effect and neurologic deficits
2. Tissue according to the group of cells and
associated extracellular matrix whose growth are
uncoordinated and exceeds that of normal tissue.
Grade I: circumscribed, biphasic, bipolar and
multipolar cells, fibers, microcysts, granular
bodies; no/rare mitotic figures, no/rare vascular
proliferation, no/focal necrosis
Grade II: moderately hypercellular, monotonous
cells, mild nuclear atypia, no/minimal mitotic
activity
Grade III: increased cellularity and diffuse
infiltration, increased nuclear atypia, increased
mitotic activity
Grade IV: vascular proliferation, necrosis,
crowded anaplastic cells, marked nuclear atypia,
brisk mitotic activity
3. Cellular individual cells differ from normal
cells in size, shape, pigmentation, nuclear size.
As tumors become more malignant, features of
cellular anaplasia becomes more pronounced
20.6 / 100,000
Malignant
7.3 / 100,000
Non-malignant
13.3 / 100,000
Females
22.3 / 100,000
Males
18.8 / 100,000
US Population
6.5 / 100,000
Males
7.7 / 100,000
Females
5.4 / 100,000
Primary NON
MALIGNANT Brain
and CNS Tumors
6.5 / 100,000
Males
10.6 / 100,000
Females
16.1 / 100,000
US Population
Age distribution of CNS Tumors
Grade II
Grade III
Grade IV
Cytologically malignant
Mitotically active, necrosis prone neoplasm
Rapid post operative disease evolution
Fatal outcome
Grade I
Grade II
Grade III
Grade IV
Survival ~ 1 year
Pilocytic astrocytoma
Pleomorphic xanthoastrocytoma
Diffuse astrocytoma
Fibrillary
Gemistocytic
Protoplasmic
Anaplastic astrocytoma
Glioblastoma
Oligodendroglial tumors
Oligodendroglioma
Anaplastic Oligodendroglioma
Oligoastrocytic tumors
Oligoastrocytoma
Anaplastic Oligoastrocytoma
Tumors of Neuroepithelial Tissue
Ependymal tumors
Subependymoma
Myxopapillary ependymoma
Ependymoma
Cellular
Papillary
Clear Cell
Tancytic
Anaplastic ependymoma
Choroid plexus tumors
Choroid plexus papilloma
Choroid plexus carcinoma
Neuronal and Mixed neuronal-glial tumors
DNET
Gangliocytoma
Central neurocytoma
Paraganglioma
Tumors of Neuroepithelial Tissue
Tumors of the Pineal region
Pineocytoma
Pineal parenchymal tumor
Pineoblastoma
Embryonal tumors
Medulloblastoma
PNET
Atypical teratoid / rhabdoid tumor
Pilocytic Astrocytoma
WHO grade I
subgroup of astrocytomas with better
prognosis (10 year survival: 94%) than
infiltrating astrocytoma
Age < 20yo in 75%
Common locations:
Cerebellar hemisphere
Optic nerve/chiasm
hypothalamus
Radiologic: discrete appearing, contrast
enhancing lesion, often with cystic with mural
nodule
AKA:
Cystic cerebellar astrocytoma
Juvenile pilocytic astrocytoma
Optic gliomas if it occurs on optic nerve
Hypothalamic gliomas
Treatment
Cerebellar PCA
Maximal surgical excision of the tumor
without producing deficit
Excision of the nodule is sufficient, cyst wall
is non neoplastic
Optic Glioma
Optic nerve sparing chiasms excision
If chiasm is involve biopsy, chemotherapy
or radiotherapy
Diffuse Astrocytoma
AKA Low Grade Diffuse Astrocytoma
WHO grade II
3 cell types
Fibrillary (most common)
Gemistocytic (prone to progress to high
grade)
Protoplasmic
Predilection for temporal, posterior frontal and
anterior parietal lobe
Clinical presentation
Most commonly seizures
Headache
Imaging: Tumor does not significantly enhance
with contrast
Genetic markers
Loss of heterozygosity on chromosome 10
& 17
Alteration of tumor suppressor gene at 9p,
13q, 19q and 22q
Transformation of the p53 gene
Dedifferentiation: Major cause of morbidity
with low grade astrocytoma is dedifferentiation
to a more malignant grade
Once dedifferentiation occurs, median survival
is 2-3yrs
Treatment
Chemotherapy
Observation
Glioblastoma
AKA: Glioblastoma multiforme (GBM)
WHO grade IV
Most common primary brain tumor, it is also
the most malignant
Irregular lesion
Oligodendroglioma
WHO grade II or III
Frequently present with seizures
Predilection for the frontal lobes
Histology: classic fried egg cytoplasm and
chicken wire vascularity
Imaging: Calcifications are common
Treatment
Surgery
Chemotherapy for all
Radiotherapy for anaplastic trasformation
Prognosis
10 yr survival 10-30%
Medulloblastoma
WHO grade IV
A small cell embryonal tumor of the cerebellum
found predominantly in children
Arises in the cerebellar vermis in the region of
the apex of the roof 4th ventricle
Compromises 30-55% of posterior fossa tumors.
Most common malignant pediatric brain tumor
Presentation:
Headache
Nausea/vomiting
Ataxia
Irritability, lethargy and macrocrania in
infants
Imaging:
Cranial CT or MRI
Spinal MRI to check for drop metastasis
Treatment
Cellular
Plexiform
Melanotic
Neurofibroma
Plexiform
Perineurioma
Malignant peripheral nerve sheath tumor
Schwannoma
WHO grade I
Lipoma
Angiolipoma
Leiomyoma
Osteoma
Osteosarcoma
Hemangioma
Hemangiopericytoma
Primary Melanotic lesions
Diffuse melanocytosis
Melanocytoma
Malignant melanoma
Meningioma
WHO grade I, II, or III depending on subtype
Slow growing extra axial tumor arising from the
arachnoid cap cells
2nd most common primary intracranial tumor
Location:
Grade
Degree of Removal
II
III
IV
Biopsy
and
CNS Lymphoma
May be primary or secondary
Ssuspected with homogenously enhancing
lesions in the central grey matter, corpus
callosum, periventricular
Presenting symptoms
Mental status changes
Symptoms of inc ICP
Generalized seizures
Diagnostics
MRI no pathognomonic feature. Difficult to
determine if tumor is subependymal
Ghost tumor disappears when steroids
are given
CSF studies elevated protein and cell
count, positive cytology in 10%
Treatment
Surgery for biopsy purposes only
Radiotherapy Whole brain but lower dose
compared to other primary CNS tumors
Chemotherapy
Methotrexate
Prognosis
No treatment 1.8-3.3 months
RT median survival 10 mos
Chemo median survival of 41 mos
Teratoma
Mixed germ cell tumor
Pineal Region Neoplasm
Tumors in this region are more common in
children
Pineal Tumors
Pineal cell tumors
Pineocytoma
Pineoblastoma
Germ cell tumors
Germinoma
Pituitary Tumors
Most are benign adenomas arising from the
anterior pituitary (adenophypophysis)
Neurohypophyseal tumors are rare
Microadenoma if tumor is < 1cm
Macroadenoma if tumor > 1cm
May be classified by endocrine function
Prolactin secreting
Cortisol secreting
Presentation
Endocrine disturbance
Mass effect
Optic chiasm bitemporal
hemianopsia
3rd ventricle Obstructive
hydrocepahlus
Caverous sinus Cranial nerve
palsy
Pituitary Apoplexy
Due to sudden expansion of
adenoma due to hemorrhage or
necrosis
Surgical emergency
Give
glucocorticoid
immediately
Diagnostics:
Endocrine screening
Cortisol
TSH, T4
Prolactin
FSH, LH
IGF-1
Prolactin
(ng/ml)
Interpretation
Situation
3-30
Normal
Normal physiologic
25-150
Mod elevated
> 150
Markedly
elevated
Prolactinoma
Stalk effect
Primary
Lung
44
Breast
10
Kidney
GI
Melanoma
Undetermined
10
Prolactinoma
Management
Prolactinoma - may opt medical treatment
using bromocriptine
Growth hormone secreting may opt
medical treatment using octreotide
Surgery
Radiotherapy
SPINE TUMORS
Could be classified into 3 groups
Brain Metastasis
Most common brain tumor seen clinically
They compromise more than half of brain
tumors
Group
Description
Extradural (ED)
55
%
Intradural
Extramedullary
(IDEM)
40
%
Arise
leptomeninges
roots
Intradural
Intramedullary (IDIM)
5
%
in
or
EXTRADURAL
A. Metastatic compromises majority of ED
tumors
B. Primary spinal cord tumors very rare
1.
Chordomas
2.
Osteoma
3.
Osteoblastoma
4.
Aneurysmal bone cyst
5.
Vertebral hemangioma
Arterial
Venous via Batsons plexus
perinervous
Presentation
Pain
Mechanical exacerbated by
movement
Motor dysfunction
Sensory dysfunction
Radiotherapy
A.
B.
C.
D.
INTRADURAL EXTRAMEDULLARY
Meningomas
Neurofibromas/Schwanomas
Lipomas
Misc.
Spinal Meningioma
Peak age: 40-70yo
Female:male ratio 4:1
90% completely intradural
Location
82% thoracic
10
15% cervical
2% lumbar
Recurrence rate with complete excision 7%
Spinal Schwannoma
Slow growing benign tumors
Most (75%) arise from the dorsal (sensory)
rootlets
Early symptoms are often radicular
Recurrence is rare after total excision
A.
B.
C.
INTRADURAL INTRAMEDULLARY
Astrocytoma 30%
Ependymoma 30%
Misc 30%
Spinal Ependymoma
Slight male predominance
Peak in 3rd to 6th decade
50% occurs in filum, next is cervical
It is the most common glioma of the lower
cord, conus and filum