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brain neoplasm (intracranial neoplasm)

Etiology: 1) primary brain tumor a) neuroepithelial neoplasm - glioma - astrocytoma (18%) - oligodendroglioma (3%) - glioblastoma (22%) - ganglioglioma - ependymoma (2%) - choroid plexus papilloma - medulloblastoma - pineal tumor - pineocytoma - germinoma - dysembryoplastic neuroepithelial tumor [4] b) lymphoma primary to CNS (4%) c) pituitary adenoma (10%) d) meningioma (27%) e) schwannoma (7%) f) craniopharyngioma g) cysts - Rathke cleft cyst - epidermoid cyst - dermoid cyst - colloid cyst of 3rd ventricle h) germ cell neoplasms i) chordoma j) hemangioblastoma k) malignant mesenchymal tumors of the meninges - hemangiopericytoma - chondrosarcoma 2) metastatic tumor - lung cancer (most common) - breast cancer - melanoma - renal cell carcinoma - lymphoma - adenocarcinoma of the colon - adenocarcinoma of the esophagus - cervical cancer 3) genetic disorders associated with intracranial tumors a) gliomas - neurofibromatosis 1 & 2 - tuberous sclerosis - LiFraumeni syndrome - ataxia telangiectasia - Turcot syndrome b) other tumors - von Hippel-Lindau syndrome - multiple endocrine neoplasia-1 (Wermer's syndrome) - Werner's syndrome - retinoblastoma Epidemiology: 1) 2% of solid tumors in men 2) 2nd most common tumor after leukemia in individuals < 15 years of age 3) 3rd most common tumor in men, 4th most common in women age 15-34 years 4) 4th most common tumor in men age 35-54 years Pathology: - most commonly involve cerebral hemispheres [4] Genetics: - see specific types - other implicated genes TMEFF1, ARHGAP23, C14orf166, ADAMTS20 Clinical manifestations: 1) headache 2) nausea/vomiting 3) visual changes a) diplopia b) blurred vision c) visual field defect 4) papilledema 5) lateralized weakness, focal neurologic deficits, tremor 6) ataxia 7) seizures (most common presentation) [4] 8) altered mental status, personality change 9) aphasia 10) nystagmus 12) cranial nerve dysfunction 13) upper motor neuron signs 14) signs of brainstem compression a) decreased level of consciousness b) nonreactive or asymmetric pupils c) hypertension & bradycardia d) abnormal breathing pattern 15) endocrine dysfunction a) pituitary tumor b) hypothalamic tumor 16) vasomotor & autonomic changes 17) signs & symptoms of primary metastatic cancer Laboratory: 1) complete blood count (CBC) - polycythemia may be associated with hemangioblastoma 2) serum chemistries 3) CSF examination a) AFTER neuroimaging - presumptive diagnosis of intracranial tumor increases risk of fatal cerebellar-foramen magnum or temporal- tentorial herniation b) cell count & cytology - medulloblastoma, ependymoma, choroid plexus papilloma & some embryonal pineal & suprasellar tumors may spread into the CSF c) glucose d) protein - high protein & xanthochromia in the absence of RBC suggests obstruction of the subarachnoid space producing stasis of CSF in the lumbar sac - seen with 1} tumors at the base of the skull (acoustic neuroma) 2} spinal cord tumors e) beta-hCG associated with trophoblastic tumor f) alpha-fetoprotein is associated with yolk sac tumor 4) biopsy may have prognostic value a) intravenous bromodeoxyuridine (BUdR) administration prior to surgery facilitates obtaining a proliferation index in biopsied tumor tissue b) immunohistochemical staining for proliferating cell nuclear antigen PCNA c) monoclonal antibody staining for nuclear protein Ki67 5) see ARUP consult [5] Special laboratory: 1) formal visual field testing 2) visual evoked potentials may help distinguish multiple sclerosis from tumor [3] Radiology: 1) magnetic resonance imaging (MRI) with gadolinium contrast a) multifocal ring-enhancing lesions at the gray-white junction suggest brain metastases [4] b) infiltrating white matter lesions suggest glioma - contrast enhancement suggest higher grade neoplasm - vague contast enhancement with honerycomb pattern suggests oligodentroglioma - calcification seen best with CT c) periventricular homogeneous diffusely enhancing white matter lesions sugges primary CNS lymphoma d) posterior fossa suggests ependymoma vs medulloblastoma - ependymoma: - may also involve spinal cord - contrast enhancement - calcification - +/- hydrocephalus - medulloblastoma (cerebellum) - contrast enhancement - hydrocephalus e) cerebellar-pontine angle suggest schwannoma f) dural with diffusely-enhancing lightbulb sign suggests meningioma - calcified 2) computed tomography (CT) of head a) emergency neuroimaging b) calcification may be best seen with CT 3) chest X ray if metastatic lung cancer is possibility Complications: 1) increased intracranial pressure a) cerebellar-foramen magnum herniation b) temporal lobe - tentorium cerebelli herniation 2) obstructive hydrocephalus 3) hemorrhage into a tumor 4) fluid & electrolyte imbalance (especially hyponatremia) 5) opportunistic infections a) bacterial: listeria b) viral: progressive multifocal leukoencephalopathy (PmL) c) fungal: cryptococcus 6) complication of therapy a) radiation-induced complications 1] early reactive peritumor edema or demyelination 2] early-delayed corticosteroid-responsive encephalopathy 3] radiation necrosis (3-13 months after radiation) b) vincristine neuropathy 7) paraneoplastic syndromes 8) meningeal carcinomatosis 9) neoplastic angioendotheliosis 10) venous thromboembolism: pulmonary embolism [4] Differential diagnosis: 1) neoplasm a) primary tumor b) metastatic tumor 2) infection a) brain abscess b) subdural empyema c) epidural abscess d) toxoplasmosis e) tuberculous granuloma f) parasitic infection 3) hemorrhage a) intracerebral hemorrhage b) subdural hematoma c) epidural hematoma (acute) 4) miscellaneous lesions a) multiple sclerosis plaque b) acute infarction with swelling c) arachnoid cyst d) vascular malformation e) large aneurysm Management: 1) metastatic workup, if indicated (10% of unknown primary) 2) neurosurgery consultation a) biopsy b) resection c) ventricular drainage for hydrocephalus d) nothing by mouth (NPO) if neurosurgery is anticipated 3) measures to attenuate cerebral edema a) dexamethasone: 1] generally begun prior to surgical decompression 2] generally tapered 1 week after surgery 3] use lowest dose that maintains comfort & function 4] do not use if primary CNS lymphoma suspected [4] b) mannitol c) intubation & hyperventilation seldom indicated 4) anticonvulsants for seizure prophylaxis not routinely recommended [4,7] - anticonvulsant may be used for 1 week immediately after surgical resection [4] - if seizure occurs, anticonvulsive therapy is recommended [4] 5) radiation therapy 6) chemotherapy a) agents which cross the blood brain barrier have anti- tumor activity against parenchymal tumors - BCNU, CCNU, PCNU, ACNU, procarbazine, temozolomide b) hydroxyurea often used during radiation c) lomustine, procarbazine & vincristine (PCV) is a combination chemotherapy often used after radiation 7) metastasis to the brain a) a single brain metastasis may be amenable to surgical resection b) multiple brain metastases are treated palliatively - whole brain irradiation - stereotactive radiosurgery 8) prophylaxis for venous thromboembolism in hospitalized patients - anticoagulation in combination with pneumatic compression after surgical resection [4] 9) intravenous heparin for pulmonary embolism [4,10] 10) patient education a) no driving b) risks & benefits of procedures c) prognosis 1] astrocytoma: median survival a] 93 months for grade 1 or 2 b] 12 months for grade 3 (anaplastic astrocytoma) c] 5 months for grade 4 (glioblastoma multiforme) 1} 1 year with radiation plus chemotherapy 2} 18 months with radiation plus brachytherapy 2] oligodendroglioma a] 5 year survival is > 50% b] 10 year survival is 25-34% 3] ependymoma: 5 year survival is > 80% 4] primary CNS lymphoma: a] median survival 18 months; may be longer with systemic chemotherapy b] median survival 3 months in patients with AIDS 5] meningiomas a] surgical excision often curative b] radiation therapy reduces recurrence to < 10% 6] schwannomas are clinically benign

Related

brain metastases intracranial mass lesion

Specific

astrocytic neoplasm, WHO grade 1 brainstem neoplasm cerebellar neoplasm craniopharyngioma ganglioglioma; dysembryonic neuroepithelial neoplasm hypothalamic neoplasm malignant brain tumor pineal neoplasm pituitary neoplasm

General

central nervous system (CNS) neoplasm intracranial neoplasm

Figures/Diagrams

Primary CNS tumors CNS neoplasms

References

  1. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 1068-70
  2. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 623, 2398
  3. Levin et al, Neoplasms of the central nervous system, in: Cancer: Principles & Practice of Oncology, DeVita et al, eds, Lippincott, Williams & Wilkins, Philadelphia, 2001, pg 2100
  4. Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 2009, 2012, 2015, 2018, 2021.
  5. ARUP Consult: Central Nervous System Tumors - Brain Tumors The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/central-nervous-system-tumors
  6. Hoffman S, Propp JM, McCarthy BJ. Temporal trends in incidence of primary brain tumors in the United States, 1985-1999. Neuro Oncol. 2006 Jan;8(1):27-37. PMID: 16443945
  7. Glantz MJ, Cole BF, Forsyth PA et al Practice parameter: anticonvulsant prophylaxis in patients with newly diagnosed brain tumors. Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2000 May 23;54(10):1886-93. PMID: 10822423
  8. Ricard D, Idbaih A, Ducray F et al Primary brain tumours in adults. Lancet. 2012 May 26;379(9830):1984-96. PMID: 22510398
  9. Maschio M, Dinapoli L. Patients with brain tumor-related epilepsy. J Neurooncol. 2012 Aug;109(1):1-6 PMID: 22528794
  10. Jo JT, Schiff D, Perry JR. Thrombosis in brain tumors. Semin Thromb Hemost. 2014 Apr;40(3):325-31. Review. PMID: 24599439
  11. Schaff LR, Mellinghoff IK Glioblastoma and Other Primary Brain Malignancies in Adults. A Review. JAMA. 2023;329(7):574-587 PMID: 36809318 https://jamanetwork.com/journals/jama/fullarticle/2801673
  12. Perkins A, Liu G. Primary Brain Tumors in Adults: Diagnosis and Treatment. Am Fam Physician. 2016 Feb 1;93(3):211-7. PMID: 26926614 Free article. Review.
  13. Adult Brain Tumors (PDQ): Treatment http://www.nci.nih.gov/cancerinfo/pdq/treatment/adultbrain/HealthProfessional/ - Childhood Brain Tumors (PDQ): Treatment http://www.nci.nih.gov/cancerinfo/pdq/treatment/childbrain/HealthProfessional/
  14. What you need to know about Brain Tumors http://www.cancer.gov/templates/doc.aspx?viewid=b5500bd0-3da6-496a-8080-3052a630ba57
  15. National Cancer Institute Brain Tumors - Health Professional version https://www.cancer.gov/types/brain/hp - Adult Central Nervous System Tumors Treatment (PDQ) - Health Professional version https://www.cancer.gov/types/brain/hp/adult-brain-treatment-pdq