II. 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%

III. 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

IV. 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

V. 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%)

VI. 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

VII. 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

VIII. 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]

IX. Types: Metastatic Cancer to Brain

  1. Common
    1. Lung Cancer
    2. Breast Cancer
  2. Less common
    1. Hypernephroma
    2. Melanoma
    3. Prostate Cancer

X. 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]

XI. 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

XII. 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

XIII. Complications: Brain Lesion management

XIV. 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%

XV. 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

XVI. Resources

Images: Related links to external sites (from Bing)

Related Studies

Ontology: Brain Neoplasms (C0006118)

Definition (NCI) A benign or malignant neoplasm that arises from or metastasizes to the brain.
Definition (NCI_NCI-GLOSS) The growth of abnormal cells in the tissues of the brain. Brain tumors can be benign (not cancer) or malignant (cancer).
Definition (MSH) Neoplasms of the intracranial components of the central nervous system, including the cerebral hemispheres, basal ganglia, hypothalamus, thalamus, brain stem, and cerebellum. Brain neoplasms are subdivided into primary (originating from brain tissue) and secondary (i.e., metastatic) forms. Primary neoplasms are subdivided into benign and malignant forms. In general, brain tumors may also be classified by age of onset, histologic type, or presenting location in the brain.
Definition (CSP) neoplasms of the intracranial components of the central nervous system, including the cerebral hemispheres, basal ganglia, hypothalamus, thalamus, brain stem, and cerebellum; subdivided into primary (originating from brain tissue) and secondary (metastatic) forms; primary neoplasms are subdivided into benign and malignant forms; brain tumors may also be classified by age of onset, histologic type, or presenting location in the brain.
Concepts Neoplastic Process (T191)
MSH D001932
ICD9 239.6
SnomedCT 189537005, 254935002, 126952004
English Brain Neoplasms, Neoplasm, Brain, Neoplasms, Brain, Neoplasm of unspecified nature of brain, brain neoplasm, Brain neopl.unspecified nature, Brain tumors, BRAIN NEOPL, NEOPL BRAIN, neoplasm of brain (diagnosis), neoplasm of brain, Brain tumor NOS [Ref.20909], Brain neoplasm NOS, Brain tumor NOS, Brain tumour NOS, Brain Tumors, Tumor, Brain, Tumors, Brain, Brain Neoplasms [Disease/Finding], brain neoplasms, brain tumour, brain tumours, brain tumors, brain tumor (diagnosis), brain tumor, Neoplasm of unspecified nature of brain (disorder), Brain--Tumors, Brain neoplasm, Brain tumor, Brain tumour, BT - Brain tumor, BT - Brain tumour, Neoplasm of brain (disorder), Neoplasm of brain, Brain Neoplasm, Brain Tumor, Neoplasm of Brain, Neoplasm of the Brain, Tumor of Brain, Tumor of the Brain
Italian Tumore del cervello, Tumore cerebrale, Tumore del cervello NAS, Tumore cerebrale NAS, Tumore cerebrale NAS [Rif.20909], Tumore di natura non specificata dell'encefalo, Tumori cerebrali, Neoplasie cerebrali
Dutch hersentumor, hersenneoplasma NAO, hersentumor NAO, hersentumor NAO [Ref.20909], neoplasma van de hersenen niet-gespecificeerd, hersenneoplasma, Gezwel, hersen-, Gezwellen, hersen-, Hersengezwel, Hersengezwellen, Hersentumor
French Tumeur du cerveau SAI, Tumeur du cerveau de nature non précisée, Tumeur cérébrale SAI, Tumeur cérébrale, Tumeur du cerveau SAI [Réf.20909], Tumeur du cerveau, Tumeurs cérébrales, Tumeurs de l'encéphale, Tumeurs du cerveau, Tumeurs encéphaliques
German Neubildung des Gehirns NNB, Hirntumor NNB [Ref.20909], Neubildung unspezifischer Natur des Gehirns, Hirntumor, Hirntumor NNB, Gehirntumor, Neubildung des Gehirns, Hirntumoren, Hirnneoplasien
Portuguese Tumor cerebral NE, Neoplasia cerebral NE, Tumor cerebral, Neoplasia de natureza NE do cérebro, Neoplasias Encefálicas, Tumores Encefálicos, Neoplasias Cerebrais, Tumores Cerebrais Primários Malignos, Câncer Cerebral, Câncer de Cérebro, Tumores Cerebrais, Neoplasia cerebral
Spanish Neoplasia cerebral NEOM, Tumor cerebral NEOM, Tumor cerebral, Tumor cerebral NEOM [Ref.20909], Neoplasia de tipo no especificado de cerebro, Neoplasias Encefálicas, Tumores del Encéfalo, neoplasia de cerebro de naturaleza no especificada (trastorno), neoplasia de cerebro de naturaleza no especificada, Neoplasias Cerebrales, Tumores Primarios Malignos del Cerebro, Cáncer del Cerebro, Tumores del Cerebro, neoplasia del encéfalo (trastorno), neoplasia del encéfalo, tumor cerebral, Neoplasia cerebral
Japanese 脳腫瘍NOS, 脳の新生物、性質不明, ノウシンセイブツ, 脳新生物NOS, ノウシュヨウNOS, ノウシュヨウ, ノウシンセイブツNOS, ノウノシンセイブツセイシツフメイ, 脳新生物, 脳腫瘍, 頭蓋内腫瘍
Swedish Hjärntumörer
Finnish Aivokasvaimet
Czech Nádor mozku, Mozkový novotvar NOS, Nádor mozku NOS, Novotvar mozku blíže neurčeného původu, Mozkový nádor, Mozkový nádor NOS, Mozkový novotvar, Mozkový nádor NOS [Ref.20909], tumory mozku, nádory mozku, mozek - nádory, mozek - nádory benigní, mozkové nádory
Polish Nowotwory mózgu
Hungarian Agyi tumor, Agydaganat k.m.n., Agytumor, Agyi tumor k.m.n., Agydaganat, Agy nem meghatározott természető daganata, Agytumor k.m.n., Agyi tumor k.m.n. (Ref.20909)
Norwegian Hjernesvulster, Hjerneneoplasmer, Hjernetumorer

Ontology: adult brain tumor (C0220624)

Definition (NCI) A benign or malignant, primary or metastatic neoplasm of the brain occurring in adults.
Concepts Neoplastic Process (T191)
English adult brain tumors, adults brain tumors, brain tumor adult, adults brain tumor, adult brain tumor, brain tumor, adult, Adult Brain Neoplasm, Adult Brain Tumor, Neoplasm of Adult Brain, Neoplasm of the Adult Brain, Tumor of Adult Brain, Tumor of the Adult Brain, Brain Neoplasms, Adult, Brain tumor, adult: General and other

Ontology: Intracranial mass (C0270614)

Concepts Finding (T033)
SnomedCT 85974009
English Mass of intracranial structure (finding), Intracranial mass (disorder), Mass of intracranial structure, Intracranial lump, Intracranial mass
Spanish masa de estructura intracraneal (hallazgo), masa intracraneal (trastorno), masa intracraneal, masa de estructura intracraneal, bulto intracraneal