Prof WBP MATUJA

DEPARTMENT OF INTERNAL MEDICINE,

MUHAS/MNH
 They are collection of neoplasms, each with its own
biological, prognosis and treatment
However the clinical presentation, diagnostic approach and
initial treatment are similar
Facts and figures
Figures vary from time to time and place:
primary brain tumours account for 20% of all
deaths from all cancer deaths and 10 times
more from metastases to the brain
Age-and sex-adjusted incidence of
primary CNS tumours at Mayo clinic(1950-
1989) was 19.1 per 100,000 persons per year
Risk factors for brain
neoplasm
Ionizing radiation has been identified unequivocally as
risk factor for Glial and Meningeal neoplasms with risk
x10 for meningiomas and x3 to7 incidence for glial
tumours with latency period of 10 to >20 years after
exposure VS non exposed group

No other environmental exposure or behavior has been

clearly identified as a risk factor eg cellular phones, exposure to
high tension wires, dye hair, head injury, dietary N nitro-soureas,
Clinical presentation of
brain tumours
Focal symptoms and signs- such as aphasia,
hemiparesis, focal seizures etc.. are generally progressive
but can have a rapid evolution in high grade gliomas
Generalized features- reflect raised ICP:- headache,
nausea, vomiting and abducen nerve palsy: note
headache may be unilateral but typically diffuse more
noticeable on wakening and intermittent
Seizures 15-95% of pts may be focal or generalized and
postictal focal signs indicate location of tumour
Visual field deficits progressive in sellar, suprasellar
Symptoms of brain
tumours
Diagnosis in brain
tumours
Cranial magnetic resonance imaging
(MRI) is the only noninvasive tests
Brain biopsy for histology and classification
Histological classification on CNS
tumours
A.Tumours of nueroepithelial tissue:
(i) Astrocytic tumours: astrocytoma, anaplastic astrocytoma, glioblastoma multiforme
(ii) Oligodendroglial tumours: oligodendroglioma, anaplastic oligo
(iii) Mixed gliomas: oligoastrocytoma, anaplastic oligoastrocytoma
(iv) Ependymal tumours: ependymoma, anaplastic ependymoma, etc myxopapillar
(v) Choroid plexus tumours: c-p-papilloma, c-p carcinoma
(vi) Neuronal, neuronal-glial: Gangliocytoma, ganglioglioma, anaplastic
(vii) Pineal parenchymal : pineocytoma, pineoblastoma
(viii) Embryonal tumours: Medulloblastoma,
B. Meningeal tumours
C. Primary CNS Lymhomas
D. Tumours of the sellar region: adenomas, carcinomas. craniopharyngioma
E. Metastatic tumours
Types of brain tumours
Gliomas particularly astrocytic are histologically, genetically and therapeutically heterogeneous
Gliomas are graded pathological on WHO system or St. Anne-Mayo system based on the presence or
absence of anaplastic-nuclear atypia and mitoses,microvascular proliferation and necrosis e.g
glioblastoma multiforme- cells arranged around necrotic tissue:

ASTROCYTOMAS
low-grade fibrillary astrocytoma(WHO grade11)- increased cellularity with
monomorphic population cells infiltrirating the neuropil

They are found in the young peak age third to fourth decade,
Typically manifest with seizure other CNS features.
MRI shows nonennhancning mass that is hypodencse on T2-
weighted and bright mass on FLAIR , PET hypometabolism;
hpermetabolic? High grade
MRI Low garde
astrocytonma
Management of low-grade
astrocytomas
Surgical removal if the lesion is amenable
Radiation therapy (Majority of low-grade tumours are
not amenable to resection) focal low dose 50.4 or 45 Gy
Vs high dose 64.8 or 59.4Gy-- no deference in survival or
time to disease progression; timing of Xtherapy soon
after surgery/biopsy Vs delay until sings of progression,
no dereference in overall survival
Nevertheless pts with hemiparesis or impaired cognition
require immediate surgical debulking followed by
Xtherapy
All pts must have clinical radiological follow up
Malignant astrocytoma
The anaplastic astrocytoma and glioblastoma
multiforme are the most common glial tumours
Annual incidence of 3 t0 4 per 100,000 pop. At
least 80% of these are glioblastomas
Occur anywhere in the brain but usually the
cerebral hemispheres
Males predominant M:F ratio of 3:2
Peak age of onset 4th and 5th decade with
glioblastomas usually in 6th decade
Types of malignant astrocytomas-
PRIMARY GLIOBLASTOMAS
Primary glioblastomas tend to occur in the
older pts (mean age 55 yrs); which have
molecular pathway associated with high rate
of overexpression or mutation of epidermal
growth factor receptor,p16 deletions and
mutations in the gene for phosphatese and
tensin homologues (PTEN)
Diagnosis of malignant astrocytoma
Cranial MRI;- typical lesion is an irregular
contrast enhancing often ring-like lesion;
surrounded with edema which could be
massive to cause herniation
Typically involves the white matter and can
spread across the corpus callosum and involve
both hemispheres
Treatment of malignnant astrocytomas
Resection is the initial intervention- gross total excision is
associated with longer survival and improved neurological
function
Followed by field radiotherapy up to total dose 60 Gy-
other intensive X therapy, radiosensitizers, radioactive
seeds none have improved survival
stereotactic radiosurgery is yet to be studied on
RTCs
Chemotherapy in addition to cranial radiation is still
controversial but improves long term survival to 15-20%-
Carmustine best agent or procarbazine,lomustine, and
vincristine
SECONDARY
GLIOBLASTOMAS
Secondary glioblastoma tend to occur in younger
adults (45 years or less)
Radiolographically can occasionally be recognized
of nonenhancing lesions on MRI
Secondary glioblastoma have genetic alterations
involving the p53 gene and overpression of
platelet-derived growth factor
Treatment is resection and Radiotherapy
Treatment of recurrence
gliomas
1. Resection- or stereotactic radiosurgery for
discrete lesions
2. Chemotherapy- Temozolomide orally
3. Gene therapy
Oligodendroglial tumours
Tumours of the oligdendrocytes or their
precursors or composite histologically (mixed
cells oligodroctes and astrocytes)
Oligodendrogliomas and oligoasrocytomas
Account for 5- 20% of glial tumours
They uniquely sensitive to chemotherapy
May be low-grade and high grade
Treatment of anaplastic
oligodendroglioma
anaplastic oligod require immediate
treatment
Extensive resection and chemotherapy
Followed by radiotherapy
Reccurent oligodendroglial tumors- surgery
and chemotherapy: melphalan, thiotepa,
temozolomide, carboplatin cisplatin and
etoposide
MENINGIOMA
They arise from mengingothelial cells
They constitute 20% of all intracranial tumours
Annual incidence 7.8 per 100,000, the majority are
asymptomatic and the incidence of symptomatic is 2 per
100,000
Female-to-male ratio of 3:2 or even 2:1
Multiple meningiomas may be found in neurofibromatosis
type 2 or sporadic by contiguous spread from a clonal tumor
-<10%
Pts with breast cancer have increased frequency of
meningiomas must be distinguished from metastases
Location and diagnosis of
meningioma
Sites: base of skull, the parasellar and over he
cerebral convexities
They are slow growing not associated with
underlying brain edema
Pts with hemispheric convexities often present
with seizures or progressive hemiparesis
Basal skull present with cranial neuropathy
Headache may be a feature
MRI are adjacent to bone with or without a dural
tail diffuse pattern of enhancement
Meningioma
histology/treatment
Majority are benign, 5% atypical, 2% malignant
Characterized by loss of chromo 22q
Small tumour detected incidentally need follow
up
Surgery is definitive therapy
20% recur within 10 yrs
X-Therapy in pts with aplastic or malignant
Middle fossa mengioma after contrast
with gadolium
Primary CNS Lymphoma
Once rare tumour <1% but now >3%
Common in immunosuppression
Incidence is greater in men with peak age 6th and
7th decade
Usually subcortical or May be multifocal 40%
Two thirds present with behavioral and cognitive
changes; 50% hemiparesis aphasia, and visual-
field
Seizures are very rare
Treatment of primary
CNS lymphomas
Diagnosis is established by stereotactic
biopsy
Cranial radiation
Chemotherapy is first line treatment: high
dose of Methotrexate or combined with X-
therapy
Lymphoma in splenial-corpus callosum
CT and MRI
Lymphoma after chemotherapy-Axial
MRI
THE END
Any questions?