II. Epidemiology
- Primary malignant Brain Tumors account for only 1.4% of all cancers (and 2.4% of all cancer deaths)
- Primary Brain Tumor diagnosed in 23,380 new cases and 14,320 deaths in 2014 in U.S.
- Incidence: 6.4 cases per 100,000 person-years
- Age at peak Prevalence for adults: 55 to 64 years old
- Although most gliomas are sporadic, up to 5% are familial with varied associated genes
- Lifetime Prevalence in U.S.
- Men: 0.65%
- Women: 0.5%
III. Risk Factors: Environmental (most uproven)
- High Dose Ionizing Radiation (Only proven risk factor)
- Electromagnetic field exposure (e.g. cellular phones, unproven)
- Head Injury (unproven)
- Infections (e.g. Toxoplasmosis gondii, unproven)
- Chemical exposures (unproven)
- Air Pollution from car exhaust
- Alcohol Abuse
- Hair dyes
- Nitrate, nitrite and nitrosamine intake
- Pesticides
- Petroleum products
- Solvents
- Tobacco Abuse
- Vinyl Chloride
IV. Risk Factors: Hereditary Condition Associations
- Li-Fraumeni Syndrome
- Multiple Endocrine Neoplasia Type I
- Neurofibromatosis (types I and II)
- Nevoid Basal Cell Carcinoma Syndrome
- Tuberous Sclerosis
- Turcot Syndrome
- Von Hippel-Lindau Disease
- Cowden Disease
- Gorlin Syndrome
V. Symptoms: Presenting
-
Headache (70%, initial symptom in 23.5%)
- See Headache Red Flag
- Often occurs in the morning
- Bifrontal tension-type Headache is most common
-
Seizures (54%, initial symptom in 21.3%)
- Most often Focal Seizures (e.g. Partial Motor Seizures)
- Seizures may progress to Generalized Tonic-Clonic Seizures (e.g. Jacksonian Seizure)
- Cognitive or Personality Changes (52%)
- Memory Loss and decreased alertness and attention
- Focal Weakness (43%, initial symptom in 7.1%)
- Unsteadiness (initial symptom in 6.1%)
- Nausea or Vomiting (31%)
- Speech altered, especially expresive language (27%, initial symptom in 5.8%)
- Altered Level of Consiousness or confusion (25%, initial symptom in 4.5%)
- Visual Changes (22%, initial symptom in 3.2%)
- Diplopia (initial symptom in 0.3%)
VI. Signs: Focal findings suggestive of lesion
-
Eye Exam (observe for signs of Increased Intracranial Pressure)
- Papilledema (present in only 5% of cases at primary tumor presentation)
- Cranial Nerve 6 Palsy
-
Motor Exam
- Focal motor weakness
- Coordination
- Gait disturbance
- Specific location related signs and symptoms
- See Frontal Lobe
- See Parietal Lobe
- See Temporal Lobe
- See Occipital Lobe
- See Thalamus
- See Cerebellum
- See Brainstem
VII. Imaging
- First-line
- MRI with gadolinium contrast (preferred)
- CT Head (alternative)
- Lower Test Sensitivity for lesions, especially posterior fossa, Brain Stem and cord
- Second-line
- MR perfusion
- MR spectroscopy
- PET Scan (fluorodeoxy-Glucose)
- Other imaging when metastatic disease is considered
- CT chest, Abdomen and Pelvis
- Eyes to thighs PET Scan
- Not typically recommended as screen for metastatic source of Brain Lesions
- May be indicated in specific cases based on history or exam
VIII. Types: Primary Brain Tumors in Adults
- See Pediatric Brain Tumor for Types in Children
- Benign (account for 50% of all primary Brain Tumors)
- Meningioma (17%, Incidence 7.61 per 100,000)
- Grade 1: Meningioma
- Grade 2: Atypical Meningioma
- Grade 3: Anaplastic Meningioma (malignant)
- Pituitary Adenoma (5%)
- Schwannoma (3-5%)
- Craniopharyngioma (<2%)
- Meningioma (17%, Incidence 7.61 per 100,000)
- Malignant
- Gliomas
- Astrocytoma (18-20% of primary malignant Brain Tumors)
- Glioblastoma (35-40% of primary malignant Brain Tumors, 15% of all primary Brain Tumors)
- Grade 4: Glioblastoma (Incidence 3.19 per 100,000)
- Oligodendroglial Tumor (Incidence 0.26 per 100,000)
- Pineal Tumor (2%, Incidence 0.04 per 100,000)
- Medulloblastoma (<1% of adults, most common brain malignancy in children)
- Gliomas
- References
IX. Types: Metastatic Cancer to Brain
- Common
- Less common
- Hypernephroma
- Melanoma
- Prostate Cancer
X. Differential Diagnosis
- Infection (Immunocompromised state, travel)
- Toxoplasmosis (ring enhancing mass, most common CNS Lesion in AIDS)
- Amebiasis
- Hydatidosis
- Cysticercosis
- Fungal infection
- Sarcoidosis
- Syphilis
- Tuberculosis
- Known exposure or Tuberculosis Risk Factors
- Brain Abscess
- Septic emboli with persistent fever, recent dental work or recent Upper Respiratory Infection
-
Multiple Sclerosis
- Transient neurologic deficits (esp. visual)
- Miscellaneous
- References
XI. Evaluation
- Perform head imaging if Brain Lesion suspected
- Determine source (primary Brain Lesion versus metastatic disease)
- Consult
- Neurosurgery
- Oncology
XII. Management
- Acute management
- Significant edema or mass effect resulting in neurologic symptoms, signs or Altered Level of Consciousness
- Decadron 0.25 to 0.5 mg/kg up to 10 to 20 mg/kg IV
- Significant edema or mass effect resulting in neurologic symptoms, signs or Altered Level of Consciousness
- Lesion evaluation and management
- Management varies by tumor type, location, growth potential and patient comorbidities
- Maximal safe surgical removal with Radiotherapy and Chemotherapy is preferred
- Observe for complications (see below)
- Glucocorticoids for first week post-operative status, are tapered over time
- Standard DVT Prophylaxis starting before surgery and continuing for 7-10 days after surgery
- Post-operative Seizure Prophylaxis in post-operative period and then discontinued
- Consider Hospice and Palliative Care where indicated
- Anticipate cognitive deficits and Mood Disorder following surgery
- Consider cognitive rehabilitation program
- Screen and manage Mood Disorder
XIII. Complications: Brain Lesion management
- Venous Thromboembolism
- Intracranial Bleeding
- Wound Infection or systemic infection
- Seizure (30% of cases)
- Major Depression
XIV. Prognosis: Five year survival
- Overall for primary Brain Tumors: 33.4%
- Pilocytic astrocytoma: 100%
- Low grade astrocytoma: 58%
- Anaplastic astrocytoma: 11%
- Glioblastoma: 1.2%
XV. Prognosis: Better prognostic factors
- Age under 60 years (esp <40 years old)
- Seizure at presentation
- Tumor <6 cm in largest diameter
- No neurologic deficits before surgery
- Cancer characteristics
- Low grade Brain Tumors
- Frontal Lobe tumors
- Tumor cells with low proliferative activity and tumor necrosis is absent
- MGMT gene promotor hypermethylation present
- Treatment response
- Minimal residual tumor post-resection
XVI. Resources
- NIH Brain Cancer