HemeOnc

Brain Tumor

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Brain Tumor, Brain Tumor in Adults, CNS Lesion, Intracranial Mass

  • Epidemiology
  1. Primary malignant Brain Tumors account for only 1.4% of all cancers (and 2.4% of all cancer deaths)
  2. Primary Brain Tumor diagnosed in 23,380 new cases and 14,320 deaths in 2014 in U.S.
  3. Incidence: 6.4 cases per 100,000 person-years
  4. Age at peak Prevalence for adults: 55 to 64 years old
  5. Lifetime Prevalence in U.S.
    1. Men: 0.65%
    2. Women: 0.5%
  • Risk Factors
  • Environmental (most uproven)
  1. High Dose Ionizing Radiation (Only proven risk factor)
  2. Electromagnetic field exposure (e.g. cellular phones, unproven)
  3. Head Injury (unproven)
  4. Infections (e.g. Toxoplasmosis gondii, unproven)
  5. Chemical exposures (unproven)
    1. Air Pollution from car exhaust
    2. Alcohol Abuse
    3. Hair dyes
    4. Nitrate, nitrite and nitrosamine intake
    5. Pesticides
    6. Petroleum products
    7. Solvents
    8. Tobacco Abuse
    9. Vinyl Chloride
  • Risk Factors
  • Hereditary Condition Associations
  1. Li-Fraumeni Syndrome
  2. Multiple Endocrine Neoplasia Type I
  3. Neurofibromatosis (types I and II)
  4. Nevoid Basal Cell Carcinoma Syndrome
  5. Tuberous Sclerosis
  6. Turcot Syndrome
  7. Von Hippel-Lindau Disease
  8. Cowden Disease
  9. Gorlin Syndrome
  • Symptoms
  • Presenting
  1. Headache (70%, initial symptom in 23.5%)
    1. See Headache Red Flag
    2. Often occurs in the morning
    3. Bifrontal tension-type Headache is most common
  2. Seizures (54%, initial symptom in 21.3%)
    1. Most often Focal Seizures (e.g. Partial Motor Seizures)
    2. Seizures may progress to Generalized Tonic-Clonic Seizures (e.g. Jacksonian Seizure)
  3. Cognitive or Personality Changes (52%)
    1. Memory loss and decreased alertness and attention
  4. Focal Weakness (43%, initial symptom in 7.1%)
  5. Unsteadiness (initial symptom in 6.1%)
  6. Nausea or Vomiting (31%)
  7. Speech altered, especially expresive language (27%, initial symptom in 5.8%)
  8. Altered Level of Consiousness or confusion (25%, initial symptom in 4.5%)
  9. Visual Changes (22%, initial symptom in 3.2%)
    1. Diplopia (initial symptom in 0.3%)
  • Signs
  • Focal findings suggestive of lesion
  1. Eye Exam (observe for signs of Increased Intracranial Pressure)
    1. Papilledema (present in only 5% of cases at primary tumor presentation)
    2. Cranial Nerve 6 Palsy
  2. Motor Exam
    1. Focal motor weakness
  3. Coordination
    1. Gait disturbance
  4. Specific location related signs and symptoms
    1. See Frontal Lobe
    2. See Parietal Lobe
    3. See Temporal Lobe
    4. See Occipital Lobe
    5. See Thalamus
    6. See Cerebellum
    7. See Brainstem
  • Imaging
  1. First-line
    1. MRI with gadolinium contrast (preferred)
    2. CT Head (alternative)
      1. Lower Test Sensitivity for lesions, especially posterior fossa, Brain Stem and cord
  2. Second-line
    1. MR perfusion
    2. MR spectroscopy
    3. PET Scan (fluorodeoxy-Glucose)
  3. Other imaging when metastatic disease is considered
    1. CT chest, Abdomen and Pelvis
    2. Eyes to thighs PET Scan
      1. Not typically recommended as screen for metastatic source of Brain Lesions
      2. May be indicated in specific cases based on history or exam
  • Types
  • Primary Brain Tumors in Adults
  1. See Pediatric Brain Tumor for Types in Children
  2. Benign (account for 50% of all primary Brain Tumors)
    1. Meningioma (17%, Incidence 7.61 per 100,000)
      1. Grade 1: Meningioma
      2. Grade 2: Atypical Meningioma
      3. Grade 3: Anaplastic Meningioma (malignant)
    2. Pituitary Adenoma (5%)
    3. Schwannoma (3-5%)
    4. Craniopharyngioma (<2%)
  3. Malignant
    1. Gliomas
      1. Astrocytoma (18-20% of primary malignant Brain Tumors)
        1. Grade 1: Pilocytic Astrocytoma (Incidence 0.34 per 100,000)
        2. Grade 2: Diffuse Astrocytoma (Incidence 0.55 per 100,000)
        3. Grade 3: Anaplastic Astrocytoma (Incidence 0.37 per 100,000)
      2. Glioblastoma (35-40% of primary malignant Brain Tumors, 15% of all primary Brain Tumors)
        1. Grade 4: Glioblastoma (Incidence 3.19 per 100,000)
    2. Oligodendroglial Tumor (Incidence 0.26 per 100,000)
    3. Pineal Tumor (2%, Incidence 0.04 per 100,000)
    4. Medulloblastoma (<1% of adults, most common brain malignancy in children)
  4. References
    1. Mahaley (1989) J Neurosurg 71: 826-36 [PubMed]
  • Types
  • Metastatic Cancer to Brain
  1. Common
    1. Lung Cancer
    2. Breast Cancer
  2. Less common
    1. Hypernephroma
    2. Melanoma
    3. Prostate Cancer
  • Differential Diagnosis
  1. Infection (immunocompromised state, travel)
    1. Toxoplasmosis (ring enhancing mass, most common CNS Lesion in AIDS)
    2. Amebiasis
    3. Hydatidosis
    4. Cysticercosis
    5. Fungal infection
    6. Sarcoidosis
    7. Syphilis
    8. Tuberculosis
      1. Known exposure or Tuberculosis Risk Factors
    9. Brain Abscess
      1. Septic emboli with persistent fever, recent dental work or recent Upper Respiratory Infection
  2. Multiple Sclerosis
    1. Transient neurologic deficits (esp. visual)
  3. Miscellaneous
    1. Behcet Syndrome
  4. References
    1. Omuro (2006) Lancet Neurol 5(11): 937-48 [PubMed]
  • Evaluation
  1. Perform head imaging if Brain Lesion suspected
    1. See Headache Red Flag
  2. Determine source (primary Brain Lesion versus metastatic disease)
    1. Further evaluate with CT chest, Abdomen, Pelvis if metastatic source is suspected
  3. Consult
    1. Neurosurgery
    2. Oncology
  • Management
  1. Acute management
    1. Significant edema or mass effect resulting in neurologic symptoms, signs or Altered Level of Consciousness
      1. Decadron 0.25 to 0.5 mg/kg up to 10 to 20 mg/kg IV
  2. Lesion evaluation and management
    1. Management varies by tumor type, location, growth potential and patient comorbidities
    2. Maximal safe surgical removal with Radiotherapy and Chemotherapy is preferred
    3. Observe for complications (see below)
    4. Glucocorticoids for first week post-operative status, are tapered over time
    5. Standard DVT Prophylaxis starting before surgery and continuing for 7-10 days after surgery
    6. Post-operative Seizure Prophylaxis in post-operative period and then discontinued
    7. Consider Hospice and Palliative Care where indicated
  3. Anticipate cognitive deficits and Mood Disorder following surgery
    1. Consider cognitive rehabilitation program
    2. Screen and manage Mood Disorder
  1. Venous Thromboembolism
  2. Intracranial bleeding
  3. Wound Infection or systemic infection
  4. Seizure (30% of cases)
  5. Major Depression
  • Prognosis
  • Five year survival
  1. Overall for primary Brain Tumors: 33.4%
  2. Pilocytic astrocytoma: 100%
  3. Low grade astrocytoma: 58%
  4. Anaplastic astrocytoma: 11%
  5. Glioblastoma: 1.2%
  • Prognosis
  • Better prognostic factors
  1. Age under 60 years (esp <40 years old)
  2. Seizure at presentation
  3. Tumor <6 cm in largest diameter
  4. No neurologic deficits before surgery
  5. Cancer characteristics
    1. Low grade Brain Tumors
    2. Frontal Lobe tumors
    3. Tumor cells with low proliferative activity and tumor necrosis is absent
    4. MGMT gene promotor hypermethylation present
  6. Treatment response
    1. Minimal residual tumor post-resection
  • Resources