Intracranial Neoplasms

Author(s): Rina Bloch, MD

Originally published:11/11/2011

Last updated:04/13/2016

1. DISEASE/DISORDER:

Definition

Intracranial neoplasms are abnormal growths within the cranial cavity that may be composed of benign or malignant cells. The tumor may be primary or a metastasis from another. Brain tumors are the second most common type of childhood cancer, with posterior fossa more likely than in adults.

Etiology

  • Neuroepithelial origin: gliomas
    • Astrocytoma: ordinary, grade 1, grade 2 (anaplastic), grade 3 (glioblastoma) (former term glioblastoma multiforme)
      • Special (pilocytic, microcytic)
    • Oligodendroglioma
    • Ependymoma can spread via cerebrospinal fluid leading to drop metastases
  • Meningioma: slow growing, usually benign
  • Primary central nervous system lymphoma
  • Pituitary adenoma: functional type causes endocrine dysfunction, nonfunctional type causes problems through mass effect
  • Schwannoma (benign, most commonly cerebellopontine angle): hearing loss, tinnitus, impaired balance
  • Cerebral metastases (secondary): lung, breast, gastrointestinal (including colon), melanoma, renal cell

Epidemiology including risk factors and primary prevention

Risk factors include genetic, ionizing radiation. For primary tumors that metastasize: lung, breast, melanoma, renal cell. There is a higher risk of lymphoma in people with immune compromise, such as acquired immune deficiency syndrome or organ transplant.

The primary prevention is treating primary tumors early on or preventing primary tumors.

Patho-anatomy/physiology

Tumors cause problems from local invasion and mass effect with cerebral edema. Hemorrhage into the tumor can cause sudden change in clinical presentation.

Metastatic tumors are usually at the gray/white junction where blood vessels narrow and trap cells and at terminal arterial watershed areas. Certain tumor types’ predilection for certain areas of the brain have not been elucidated.  Hematologic, lung, breast, melanoma, and gastric cancers can spread to leptomeninges.

Disease progression including natural history, disease phases or stages, disease trajectory (clinical features and presentation over time)

new onset/acute: Headache, seizures, nausea, vomiting, focal deficits such as hemiparesis, visual field defects and aphasia depending on location, ataxia, dysphagia, impaired cognition and personality changes. Symptoms of fatigue and endocrine abnormalities may occur if pituitary is involve  The types and acuity of symptoms often correlated with location and tumor grade.

subacute: Same.

chronic/stable: Same.

pre-terminal: Cerebral edema, herniation, and progressive weakness. Dysphagia may cause dehydration and malnutr Disease progression depends on tumor type. If solitary metastasis, may be resectable or treatable by focal radiation.

Specific secondary or associated conditions and complications

May include hydrocephalus (potentially requiring ventriculoperitoneal shunt), pituitary abnormalities, seizures muscle weakness, and loss of coordination, urinary incontinence, deep venous thrombosis, and paraneoplastic phenomena. Surgery, chemotherapy,  and radiation therapy can also cause complications and deep venous thrombosis.

2. ESSENTIALS OF ASSESSMENT

History

One needs to ascertain symptoms, including the time course of symptoms. Typically one would enquire about headache, nausea/vomiting, motor changes such as weakness or coordination problems, sensory and visual changes. Headache typically occurs in the morning on awakening.  Are there any documented seizures or episodes of loss of consciousness?  Is there any shortness of breath?

History should include details of how the cancer was treated.  Ideally one wants to know what doses of medications and radiation were used. Is there a past personal or family history of cancer? Has the patient used tobacco? One should enquire as to the results  of screening tests such as mammograms, pap smears, colonoscopies, digital prostate exams, and prostate specific antigen (PSA) tests. For functional history, one needs to get details of the recent past functional skills and enquire as to current abilities to ambulate, transfer, toilet, bathe, do light meal preparation, and find out about driving skills. For social history, one needs details of the work and home duties.

Physical examination

The examination should include testing for strength, balance,  gross and fine motor coordination, sensation, and reflexes. Cranial nerve exam should include visual fields, assessment for papilledema,  assessment of palate symmetry, and tongue position. Cognitive and linguistic assessment should be done. Depending on practice setting, check stool hemoccult.

Functional assessment

One needs to assess the ability to transfer, ambulate, toilet, bathe, homemaking skills. Cognitive screening should be done, and assessment of mood, judgement and decision-making.

Laboratory studies

Electrolytes, liver function tests, blood urea nitrogen, and creatinine, albumin, and complete blood count. Specialized tests for tumor progression/recurrence may be done depending on tumor type. Specialized blood testing for pituitary function may be done.

Imaging

Cranial magnetic resonance imaging, with gadolinium to assess the tumor itself, and depending on tumor type, chest imaging or spine imaging to assess other sites. Possible positron emission scan (PET) scan depending on clinical need to look for metastases, often in conjunction with computerized tomography scan. Imaging may need repeating if there is a change in clinical condition, in order to look for edema, change in tumor size, or hydrocephalus.

Supplemental assessment tools

Electromyography/nerve conduction velocities for possible peripheral neuropathy, paraneoplastic disease problems.   Brain biopsy to ascertain primary tumor type and to determine if recurrence or posttreatment changes. Electroencephalogram if question of seizures.  Modified barium swallow can evaluate for dysphagia.

Early predictions of outcomes

Primary tumor prognosis depends most on histology, with glioblastoma  having a significantly worse prognosis (mean lifespan 18mo). Size and location also predict outcome, with tumors, which cross midline, doing significantly worse, For metastatic tumors, the primary tumor prognosis has a strong effect (e.g., breast cancer patients with brain  metastases often live > 3 years). Isolated metastasis can often be resected and has better  prognosis than multiple metastasis.

Environmental

Identify what adaptations are needed to move safely in the home and the community.

Social role and social support system

Is the patient cognitively and physically able to perform job duties? If unable to work, how does that affect insurance coverage? How does the patient wish to spend his/her days if the end of life is near? Assist family/friends in planning how to cope with day to day needs of patient as well as long term related to diagnosis. Identify support groups for the family as well as the patient. Identify financial resources in the community for care. Make patient/family aware of groups such as the American Cancer Society. Palliative care and hospice referral as appropriate.

Professional Issues

Can the patient make their own decisions about finances, driving, and medical care or is she/he too cognitively impaired? Different cultural and personal norms as to what the patient should be told if prognosis is dire. End of life decisions may need to be made depending on prognosis.

3. REHABILITATION MANAGEMENT AND TREATMENTS

At different disease stages

new onset/acute

  1. includes potential curative interventions: Surgery and chemotherapy. Radiation therapy: whole brain, stereotactic radiosurgery.
  2. includes symptom relief: Treatment of headache, nausea with medications, physical therapy, and pain management. Treat the side effects of drugs, such as constipation, peripheral neuropathy (which may develop later in course of treatment). Monitor surgical site. Watch for radiation therapy early reactions, such as nausea, vomiting, alopecia, tinnitus, and skin changes. Some of these are due to cerebral edema.
  3. includes rehabilitation strategies that intend to stabilize or optimize function or prepare for further interventions at later disease stages: Physical therapy for mobility, occupational therapy for activities of daily living, speech therapy for cognitive problems, speech and language, and dysphagia. Adaptive equipment. For acute inpatient rehabilitation, cognitive deficits and weakness may be more common.1 For fatigue, stimulants may be used. Functional gains for brain tumor patients during inpatient rehabilitation hospital stays were comparable with traumatic brain injury patients matched by age, sex, and admission functional status.2 In a retrospective study, the tumor type did not affect efficiency of functional improvement in inpatient rehabilitation, though patients receiving concurrent radiation therapy made greater gains per day.3

subacute

  1. includes secondary prevention and disease management strategies: Radiation and chemotherapy. Monitor for radiation encephalopathy. Late delayed radiation can cause focal cerebral necrosis, or diffuse injury. Minimize risk of other complications, such as osteoporosis leading to fracture. Promote adequate nutrition for skin integrity and muscle mass maintenance.
  2. includes symptom relief: Treatment of headache, nausea with medications, physical therapy; pain management including peripheral neuropathic pain due to chemotherapy. Treat side effects of drugs. Treat anxiety and/or depression if present.
  3. includes rehabilitation strategies that intend to optimize function: Physical therapy, occupational therapy, and speech therapy. Neuropsychologic testing as needed for cognitive deficits. Orthoses if needed.

chronic/stable

  1. includes secondary prevention and disease management strategies: Late radiation encephalopathy may develop. Late delayed radiation can cause focal cerebral necrosis, or diffuse injury. Imaging to address growth/recurrence of primary tumor. If metastatic, it is necessary to follow what is occurring in the rest of body.
  2. includes palliative strategies: Treatment of headache, nausea with medications, and physical therapy; pain management.
  3. includes symptom relief: Treatment of headache, nausea with medications, and physical therapy; pain management. Fatigue is often multifactorial due to deconditioning, drugs including antiseizure meds, and anemia. Workup if needed for endocrine problems, especially if the pituitary gland is involved (tumor itself, or in treatment field).
  4. includes rehabilitation strategies that intend to optimize function: Physical therapy, occupational therapy, and speech therapy. Neuropsychologic testing as needed for cognitive deficits and the development of a behavioral plan.

pre-terminal or end of life care

  1. includes symptom relief: Pain management, treatment of anxiety by counseling and medications; meet family needs including more help in the home as mobility declines. Decisions needed regarding fluid and nutritional intake and treatment of pain if that conflicts with other goals such as avoiding sedation.

Coordination of care

Treatment of brain tumors need to take into account overall neurologic and cognitive functioning in assessing management options.This requiresmultidisciplinarycoordination amongneurosurgeons, oncologists, radiationoncologists,primary care physicians, as well asphysiatrists. Theinter-disciplinary rehabilitation team, including psychology,social work,pastoral care, must work together to identifybarriers to return to home or staying at home. They mustfocuson thepatients’ goals of improvingqualityof life, or in cases oflimited life expectancy, ofmaintainingquality of life and dignityfor as long as possible.

Patient & family education

Need to work in tandem with oncology, radiation therapy, and neurosurgery to ensure no mixed messages, especially as far as life expectancy. If cognition is impaired, behavioral strategies may need to be elucidated to ensure safety.

Emerging/unique Interventions

IMPAIRMENT-BASED MEASUREMENT

Functional Independence Measure, Get Up and Go test. Berg Balance Scale. Timed 10-meter walk test, 6-minute walk test to measure the distance walked indoor on a level surface in 6 minutes. Karnofsky Performance Status Scale (KPSS): 100 is normal, 0 is dead. Not enough specificity for rehabilitation purposes. On the KPSS, a score of 60 requires occasional assistance but can care for most needs; a score of 50 requires considerable assistance and frequent medical care; a score of 40 and the patient is considered disabled, requiring special care and assistance.

MEASUREMENT OF PATIENT OUTCOMES

KPSS: 100 is normal, 0 is dead. Not enough specificity for rehabilitation purposes. On the KPSS, a score of 60 requires occasional assistance but can care for most needs; a score of 50 requires considerable assistance and frequent medical care; a score of 40 and the patient is considered disabled, requiring special care and assistance.

Translation into practice: practice “pearls”/performance improvement in practice (PIPs)/changes in clinical practice behaviors and skills

Cognitive deficits are common in brain tumor patients; one needs to look for them as patient may not self-report. More work with oncologists is needed to address functional outcome measures, particularly for outpatients and to address practical needs of patients and caregivers if the patient is being cared for at home. Palliative care programs need to include a functional component.

4. CUTTING EDGE/EMERGING AND UNIQUE CONCEPTS AND PRACTICE

Cutting edge concepts and practice

Systemic medications to enhance radio sensitivity for radiation therapy. PET scanning for brain imaging.

5. GAPS IN THE EVIDENCE-BASED KNOWLEDGE

Gaps in the evidence-based knowledge

Lack of data on outpatient rehabilitation progress/efficacy. Optimal duration of seizure prophylaxis is not clearly known.

REFERENCES

  1. Mukand JA, Blackinton DD, Crincoli MG, Lee JJ, Santos BB. Incidence of neurologic deficits and rehabilitation of patients with brain tumors. Am J Phys Med Rehabil. 2001;80:346-350.
  2. O’Dell MW, Barr K, Spanier D, Warnick RE. Functional outcome of inpatient rehabilitation in persons with brain tumors. Arch Phys Med Rehab 1998:79:1530-1534.
  3. Marciniak CM, Sliwa J, Heinemann A, Semik PE. Functional outcomes of persons with brain tumors after inpatient rehabilita Arch Phys Med Rehabil. 2001;82:457-463.
  4. http://brain.mgh.harvard.edu/ChemoGuide.htm, accessed 2/16/2016
  5. Robinson G, BloggsV, and Walker DG. Front Oc Cognitive screening in brain tumors: short but sensitive enough? pub online 2015 Mar 2011:doi: 10.3389/fonc.2015.00060.

Supplemental resources

Asher A. Cognitive dysfunction among cancer survivors. Am J Phys Med Rehabil. 2011;90(5 Suppl 1):S16-26.

Bell KR, O’Dell M, Barr K, Yablon, SA. Rehabilitation of the patient with brain tumor. Arch Phys Med Rehabil. 1998;79(3 Suppl 1):S37-46.

DeAngelis L. Brain Tumors. NEJM. 200;344:114-123.

Kirschblum S, O’Dell M, Ho C, Barr, K. Rehabilitation of persons with central nervous system tumors. CANCER. 2001; 92 (4 Suppl):1029-1038.8.

Omoro, A, DeAngelis, L. Glioblastoma and other malignant gliomas: a clinical review. JAMA. 2013;310 (17): 1842-1850.

Vargo, M. Brain tumor rehabilitation. Am J Phys Med Rehabil 2011;90(5 Suppl 1):S50-62.

Wen PY, Loeffler JS. Overview of the clinical manifestations, diagnosis, and management of patients with brain metastases. Available at: http://www.uptodate.com/contents/overview-of-the-clinical-manifestations-diagnosis- and-management-of-patients-with-brain-metastases. Accessed March 18, 2011.

Original Version of the Topic:

Rina Bloch, MD. Intracranial Neoplasms. Publication Date: 2011/11/11.

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Rina Bloch, MD
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