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Authors

  1. Schweisguth, Diane BSN, RN

Article Content

Mr. M, 34, walks into the ED accompanied by his wife, with an unsteady gait, holding onto the wall. He says he has a "really bad headache" and feels like he's drunk although he hasn't been drinking.

  
Figure. No caption a... - Click to enlarge in new windowFigure. No caption available.

Mr. M tells the ED nurse that he was recently diagnosed with stage IV colon cancer. He's currently undergoing outpatient chemotherapy, which he says makes him feel tired. When asked about his current symptoms, Mr. M says he started having difficulty with his balance a couple of days ago and it's gotten worse; his oncologist told him to go to the ED immediately.

 

Mr. M is hypertensive (BP 158/95 mm Hg), but he's afebrile and denies neck pain or stiffness or changes in vision. A Romberg test performed to assess Mr. M's balance is positive. (See Understanding the Romberg test.)

 

The ED physician suspects that Mr. M has leptomeningeal metastases (LM) and following a complete history and physical, orders a diagnostic lumbar puncture to test the cerebrospinal fluid for cancer cells.

 

Defining LM

LM is an inflammation of the meninges (the thin layers of tissue that line the brain and spinal cord) caused by cancer cells.1-41-4 The meninges consist of three layers: the dura mater, the arachnoid mater, and the pia mater. The two innermost layers, the arachnoid mater and pia mater, together are known as the leptomeninges. The space between the arachnoid mater and pia mater (the subarachnoid space) is filled with cerebrospinal fluid (CSF).1-41-4 (See Sorting out cranial meninges.)

 

LM is also known as carcinomatous meningitis, leptomeningeal carcinoma, leptomeningeal carcinomatosis, meningeal carcinomatosis, meningeal metastasis, and neoplastic meningitis when it's related to a solid tumor. It may be known as lymphomatous meningitis if it occurs with systemic lymphoma, or leukemic meningitis if it occurs with leukemia.3

 

LM usually develops when cancer cells metastasize from the original (primary) tumor site to the meninges; however, it can also occur from direct extension of a primary brain tumor.1,51,5 Cancer cells metastasize to the meninges via the arterial or venous systems (hematogenous dissemination), or the lymphatic system (lymphatic spread). Once the cancer cells reach the meninges and CSF, they may attach themselves to the leptomeninges or flow unattached in the CSF. In either case, the cancer cells thrive in the subarachnoid space, where they grow and eventually obstruct CSF flow or prevent CSF reabsorption.1

 

LM occurs in approximately 5% of patients with solid tumors.1,3,51,3,51,3,5 However, the incidence may be higher due to undiagnosed or asymptomatic LM. Although LM may occur in patients with any type of cancer, the solid tumor cancers most commonly associated with LM include breast cancer (12% to 35%), lung cancer (usually small-cell lung cancer, 10% to 26%), melanoma (5% to 25%), gastrointestinal cancers (4% to 14%), and cancers of unknown primary origin (1% to 7%).1 LM may also be a complication of hematologic cancers, such as leukemia and lymphoma, and primary brain tumors, which can infiltrate the leptomeninges or disseminate along CSF pathways.3,5,63,5,63,5,6 This article focuses on LM related to solid tumor cancers.

 

Aggressive cancer complication

The number of people diagnosed with LM has risen in recent years, most likely because people with cancer are living longer due to treatment advances.1-31-3

 

LM is an aggressive complication of cancer with a very poor prognosis. For patients who don't receive treatment, the median overall survival time is 4 to 6 weeks; for those who receive aggressive treatment, the usual survival time is less than 8 months. The median overall survival time is 2 to 3 months.1,31,3 Some patients survive longer, depending on the type of cancer, the patient's neurologic status, and the patient's response to treatment.

 

Signs and symptoms

Patients with LM may present with subtle neurologic signs and symptoms that can be difficult to differentiate from adverse reactions to chemotherapy or radiation, or from signs and symptoms of brain metastasis. The patient's signs and symptoms typically relate to the areas of the central nervous system (CNS) affected by the cancer cells: the cerebral hemisphere, cranial nerves, and spinal cord. (See Recognizing LM.) Many patients present with a combination of cerebral, cranial nerve, and spinal cord signs and symptoms.1,2,41,2,41,2,4 In the case study above, Mr. M presented to the ED with two of the most common signs and symptoms of cerebral dysfunction due to LM: headache and difficulty walking.1

 

Besides signs and symptoms related to the CNS, the patient may have signs and symptoms of increased intracranial pressure (ICP) such as headache, lethargy, nausea, vomiting, papilledema, decreased level of consciousness, and a widened pulse pressure. These result from increased ICP due to the cancer cells obstructing CSF outflow or impairing CSF reabsorption.1

 

Signs and symptoms of LM differ from those typical of bacterial or hemorrhagic meningitis in that fever, photophobia, and nuchal rigidity are extremely rare. Seizures are also rare with LM.1

 

Diagnosing LM

For patients suspected of having LM, a health history and physical assessment with a comprehensive neurologic evaluation should be performed. The neurologic evaluation should include assessment of the patient's mental status; cranial nerves; and motor, sensory, and cerebellar functioning.3

 

Diagnostic studies used to diagnose LM include magnetic resonance imaging (MRI) and CSF analysis. A lumbar puncture is performed and the diagnosis confirmed by a positive CSF cytology (or flow cytometry for patients with leukemia or lymphoma) in which cancer cells are found in the CSF. An MRI study of the brain and spinal cord may also be performed when LM is suspected or to support the diagnosis.1-41-4 Depending on the patient's history and clinical status, an MRI study may be performed before a lumbar puncture to rule out contraindications to performing a lumbar puncture.6

 

Current National Comprehensive Cancer Network guidelines include the following for the diagnosis of LM:

 

* CSF positive for tumor cells OR

 

* positive radiologic findings supportive by clinical findings OR

 

* signs and symptoms with suggestive CSF findings (high white blood cell count, low glucose, and high protein) in a patient known to have a malignancy.3

 

 

Diagnosis and initial treatment for Mr. M

Mr. M's lumbar puncture in the ED revealed cancer cells in the CSF, confirming a diagnosis of LM. Mr. M was discharged from the ED with an oral opioid for his headache and referred to his oncologist for next-day evaluation and follow up.

 

Managing LM

Treatment for a patient with LM is difficult and focuses on lessening symptoms to improve or maintain the patient's quality of life, improving or stabilizing the patient's neurologic status, and prolonging the patient's life.1,31,3 The specific care plan depends on the primary cancer and the patient's neurologic status. While no standardized treatment approaches have been developed, management usually includes symptomatic care, LM-directed therapies, and palliative care.

 

Due to the aggressive nature of LM, determining which patients to consider for LM-directed therapy and which patients to consider for palliative care can be difficult. Patients with LM are typically classified into one of two groups, good risk or poor risk, based on their performance status using the Karnofsky Performance Scale (KPS) index (http://bit.ly/1GyfDli) or the Eastern Cooperative Oncology Group (ECOG) grade (http://bit.ly/1FUOBtz); neurologic deficits; and the extent of their disease.1,3,71,3,71,3,7 (See Risk classifications and treatment considerations.)

 

Symptomatic care

All patients with LM should receive individualized symptomatic care based on their signs and symptoms and needs. This includes treatment for the patient's head, back, or radicular pain using appropriate therapies such as opioid analgesics, tricyclic antidepressants, and/or corticosteroids. Nausea and vomiting can be managed with antiemetics; confusion can be managed with haloperidol; and depression or fatigue can be managed with serotonin reuptake inhibitors or stimulant medications, as appropriate.1

 

Repeat lumbar punctures may be performed to temporarily relieve headaches. If the patient has symptomatic hydrocephalus or increased ICP, a ventriculoperitoneal (VP) shunt, which drains excess CSF into the peritoneal cavity, may be placed.1

 

LM-directed therapies

LM-directed therapies may include radiation therapy to the brain and/or the use of intrathecal (intralumbar or IT) or intraventricular (IVent) chemotherapy or drugs administered directly into the spinal fluid (Intra-CSF).1,31,3 If radiation therapy is indicated, involved-field or whole-brain irradiation may be performed.1

 

IT or IVent chemotherapy is typically administered. Because of the blood-brain barrier, chemotherapeutic agents delivered directly into the CSF have greater access to the cancer cells than systemic chemotherapy. IT chemotherapy can be administered by repeated lumbar punctures or via a ventricular access device, such as a ventricular catheter.1,31,3

 

The choice of chemotherapeutic drugs used to manage LM depends on the patient's primary cancer, route of administration, and neurologic status. Methotrexate, liposomal cytarabine, and thiotepa are commonly prescribed.1

 

Palliative care and patient education

End-of-life care should be discussed with all patients with LM and their family members to realistically plan the patient's course of palliative care. Comfort and quality of life care measures appropriate for the patient should be discussed, along with the need for spiritual and hospice care.1

 

Patient teaching for a patient with LM is challenging because of the patient's poor prognosis and changing neurologic and cognitive function. While some patients may experience a rapid clinical deterioration, others may not. Individualize patient education to the patient's specific situation. Plan brief teaching sessions focusing on need-to-know information because the patient and family members have much to learn and understand during a stressful time.

 

Patients and their families have emotional and psychosocial challenges as they're dealing with unexpected changes and losses (such as alterations in hearing or vision, or loss of the patient's independence) that may happen quickly. Collaborate with the patient's interdisciplinary team to coordinate care.

 

Besides providing patient education, emotional support, and coordinated care, continue to provide nursing interventions based on the patient's clinical status and treatment approaches, all of which are highly individualized and may change rapidly as the disease progresses. Assess for signs and symptoms of relief or worsening of the patient's clinical status and for indicators of efficacy of or adverse reactions to medications and therapies. Encourage the use of complementary therapies such as relaxation techniques and provide care as appropriate for the patient's activities of daily living. For example, if the patient has difficulty walking, a hiking pole, cane, walker, or wheelchair may be indicated. Use of computer tablets or paper notes may be indicated if the patient has hearing loss; voice-activated computer or cell phone aids may help if the patient has vision loss.

 

As the patient's disease progresses, palliative care should focus on providing comfort and quality of life measures whether in the hospital or at home. Help achieve the patient's wishes, such as being able to visit with a pet or enjoy a favorite food. Balance the patient's need for rest and personal care with the family's need to be with the patient.

  
Table Risk classific... - Click to enlarge in new windowTable Risk classifications and treatment considerations

Mr. M's journey

The day after Mr. M's ED visit, he was evaluated by his oncologist and an MRI study was performed to help guide treatment options. Plans were made for Mr. M to temporarily stop chemotherapy and begin outpatient radiation therapy. He continued on an oral opioid for his headache and began using a hiking pole to help with his balance while walking.

 

A few days after consulting with a radiation oncologist, Mr. M started outpatient radiation therapy, but his clinical status gradually declined and his neurologic deficits increased. Mr. M's balance and coordination worsened and he began having severe back pain along with his headache. He also began experiencing alterations in vision and hearing.

 

Mr. M was admitted to the hospital to continue radiation therapy as an inpatient and to receive I.V. opioids and corticosteroid therapy. After 1 week in the hospital, his neurologic condition worsened; radiation therapy was stopped and palliative care begun.

 

Mr. M expressed a wish to go home, so a VP shunt was placed to help relieve his headaches and drain excessive CSF. The day after the VP shunt was placed, Mr. M was discharged home with hospice care. He died peacefully in his sleep the day after he went home, less than 1 month after his diagnosis of LM.

 

Early diagnosis guides care

LM is a serious complication of cancer with a very poor prognosis, so it's important for nurses caring for patients with cancer to know when to suspect this aggressive and deadly complication. An early diagnosis is needed to help provide patients with timely and appropriate treatment, support, and care, and improve patient outcomes.

 

Understanding the Romberg test

This is a test of position sense. Stand close enough to the patient to prevent a fall, and ask the patient to stand with feet together and both eyes open. Then ask the patient to close both eyes for 30 to 60 seconds without support. Note the patient's ability to maintain an upright posture; only minimal swaying should occur. Patients with a positive Romberg test stand fairly well with eyes open but lose balance when their eyes are closed.10

 

Recognizing LM1,2,41,2,41,2,4

These signs and symptoms are commonly seen in LM according to the area of CNS involvement.

 

Cerebrum

 

* behavioral changes

 

* cognitive deficits

 

* coordination difficulties

 

* gait difficulties

 

* headache

 

* lethargy

 

* mental status changes

 

* nausea and vomiting

 

* sensory disturbances

 

 

Cranial nerves

 

* sensory disturbances

 

* cranial nerve palsies

 

* diplopia

 

* facial numbness/paresis

 

* hearing impairment or loss

 

* sensory deficits

 

* vertigo

 

* vision impairment or loss

 

 

Spinal cord

 

* back pain

 

* bladder and bowel dysfunction

 

* lower motor weakness

 

* neck pain

 

* paresthesias

 

* radiculopathy

 

* reflex asymmetry

 

* sensory loss

 

* upper motor weakness

 

 

REFERENCES

 

1. Le Rhun E, Taillibert S, Chamberlain MC. Carcinomatous meningitis: leptomeningeal metastases in solid tumors. Surg Neurol Int. 2013;4(suppl 4):S265-S288. [Context Link]

 

2. Jimenez Mateos A, Cabrera Naranjo F, Gonzalez Hernandez A, Fabre Pi O, Diaz Nicolas S, Lopez Fernandez JC. Neoplastic meningitis. Review of a clinical series. Neurologia. 2011;26(4):227-232. [Context Link]

 

3. National Comprehensive Cancer Network. NCC Guidelines Version 2.2014 Central Nervous System Cancers. Version 2.1024, 08/29/14; LEPT-1; MS-29-MS-31. [Context Link]

 

4. Walkty A, Abbott B, Swirsky N, Safneck J, Embil JM. Keeping an open mind about meningitis: a case report of carcinomatous meningitis. CJEM. 2011;13(5):352-356. [Context Link]

 

5. Walker JG. Diagnosis and management of leptomeningeal disease. Clin J Oncol Nurs. 2009;13(4):384-387. [Context Link]

 

6. Demopoulos A. Clinical features and diagnosis of leptomeningeal metastases from solid tumors. UpToDate. 2015. http://www.uptodate.com. [Context Link]

 

7. MD Anderson Cancer Center. Leptomeningeal metastases. The University of Texas, MD: Anderson Cancer Center; 2014. http://www.mdanderson.org/education-and-research/resources-for-professionals/cli. [Context Link]

 

8. Karnofsky Performance Status Scale. http://www.hospicepatients.org/karnofsky.html. [Context Link]

 

9. Oken MM, Creech RH, Tormey DC, et al. Toxicity and response criteria of the Eastern Cooperative Oncology Group. Am J Clin Oncol. 1982;5(6):649-655. [Context Link]

 

10. Bickley LS. Bates' Guide to Physical Examination and History Taking. 11th ed. Philadelphia, PA: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2013. [Context Link]