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Page 1: 30 Nursing2015 l Volume 45, Number 12nursing.ceconnection.com/ovidfiles/00152193-201512000-00010.pdf34 l Nursing2015 l Volume 45, Number 12 Symptomatic care All patients with LM should

30 l Nursing2015 l Volume 45, Number 12 www.Nursing2015.com

Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.

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www.Nursing2015.com December l Nursing2015 l 31

By Diane Schweisguth, BSN, RN

2.0ANCC CONTACT HOURS

Mr. M, 34, walks into the ED accompa-nied 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.

Mr. M tells the ED nurse that he was recently diagnosed with stage IV colon cancer. He’s currently undergoing out-patient chemotherapy, which he says makes him feel tired. When asked about his current symptoms, Mr. M says he started having difficulty with his bal-ance 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 lum-bar puncture to test the cerebrospinal fluid for cancer cells.

Defining LMLM is an inflammation of the meninges (the thin layers of tissue that line the brain and spinal cord) caused by cancer cells.1–4 The me-ninges consist of three layers: the dura mater, the arachnoid mater, and the pia mater. The two innermost SC

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Leptomeningeal metastases: What nurses need to know

Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.

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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).1–4 (See Sorting out cranial meninges.)

LM is also known as carcinoma-tous meningitis, leptomeningeal carcinoma, leptomeningeal carcino-matosis, meningeal carcinomatosis, meningeal metastasis, and neoplas-tic meningitis when it’s related to a solid tumor. It may be known as lymphomatous meningitis if it oc-curs 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.1,5 Cancer cells metastasize to the meninges via the arterial or venous systems (hema-togenous dissemination), or the lymphatic system (lymphatic spread). Once the cancer cells reach the meninges and CSF, they may attach themselves to the leptomen-inges 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.1,3,5

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 (usu-ally 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 he-matologic cancers, such as leuke-

mia and lymphoma, and primary brain tumors, which can infiltrate the leptomeninges or disseminate along CSF pathways.3,5,6 This article focuses on LM related to solid tumor cancers.

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

LM is an aggressive complica-tion 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.1,3 Some patients sur-vive longer, depending on the type of cancer, the patient’s neurologic status, and the pa-tient’s response to treatment.

Signs and symptomsPatients 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 re-late 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 com-bination of cerebral, cranial nerve, and spinal cord signs and symp-toms.1,2,4 In the case study above, Mr. M presented to the ED with two of the most common signs and symptoms of cerebral dysfunc-tion due to LM: headache and difficulty walking.1

Understanding the Romberg testThis 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

Patients with LM may present with subtle

neurologic signs and symptoms that can be difficult to differentiate from adverse reactions

to chemotherapy or radiation.

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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 obstruct-ing 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 LMFor patients suspected of hav-ing LM, a health history and physical assessment with a com-prehensive neurologic evalua-tion should be performed. The neurologic evaluation should in-clude assessment of the patient’s mental status; cranial nerves; and motor, sensory, and cerebel-lar 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 cytol-ogy (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 per-formed when LM is suspected or to support the diagnosis.1–4 De-pending on the patient’s history and clinical status, an MRI study may be performed before a lumbar puncture to rule out contraindica-tions to performing a lumbar puncture.6

Current National Comprehen-sive Cancer Network guidelines include the following for the diag-nosis of LM:

• CSF positive for tumor cells OR• positive radiologic findings sup-portive by clinical findings OR• signs and symptoms with sugges-tive 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. MMr. M’s lumbar puncture in the ED revealed cancer cells in the CSF, con-firming 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 LMTreatment for a patient with LM is difficult and focuses on lessen-ing symptoms to improve or main-tain the patient’s quality of life, improving or stabilizing the pa-tient’s neurologic status, and

prolonging the patient’s life.1,3 The specific care plan depends on the primary cancer and the patient’s neurologic status. While no stan-dardized treatment approaches have been developed, management usually includes symptomatic care, LM-directed therapies, and pallia-tive care.

Due to the aggressive nature of LM, determining which patients to consider for LM-directed thera-py 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 Oncol-ogy Group (ECOG) grade (http://bit.ly/1FUOBtz); neurologic defi-cits; and the extent of their dis-ease.1,3,7 (See Risk classifications and treatment considerations.)

Sorting out the cranial meninges

Source: Porth CM. Essentials of Pathophysiology: Concepts of Altered States. 4th ed. Philadelphia, PA: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2015.

Superior sagittal sinus

Subarachnoidspace

Subduralspace

Skin

Periosteum

Bone

Dura mater

Arachnoid

Pia mater

Falxcerebri

Arachnoid villi

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Symptomatic careAll patients with LM should re-ceive individualized symptomatic care based on their signs and symptoms and needs. This in-cludes treatment for the patient’s head, back, or radicular pain using appropriate therapies such as opi-oid analgesics, tricyclic antidepres-sants, and/or corticosteroids. Nau-sea and vomiting can be managed with antiemetics; confusion can be managed with haloperidol; and depression or fatigue can be man-aged with serotonin reuptake in-hibitors or stimulant medications, as appropriate.1

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

LM-directed therapiesLM-directed therapies may in-clude radiation therapy to the brain and/or the use of intrathecal (intra-

lumbar or IT) or intraventricular (IVent) chemotherapy or drugs ad-ministered directly into the spinal fluid (Intra-CSF).1,3 If radiation therapy is indicated, involved-field or whole-brain irradiation may be performed.1

IT or IVent chemotherapy is typ-ically administered. Because of the blood-brain barrier, chemothera-peutic 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 ventricu-lar access device, such as a ven-tricular catheter.1,3

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

Palliative care and patient educationEnd-of-life care should be dis-cussed with all patients with LM

and their family members to realis-tically 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 cogni-tive 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 focus-ing 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 inde-pendence) that may happen quick-ly. Collaborate with the patient’s interdisciplinary team to coordinate care.

Besides providing patient educa-tion, emotional support, and coor-dinated care, continue to provide nursing interventions based on the patient’s clinical status and treat-ment 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 ad-verse 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 ex-ample, if the patient has difficulty walking, a hiking pole, cane, walker, or wheelchair may be

Recognizing LM1,2,4

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

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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 pro-gresses, palliative care should fo-cus 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 person-al care with the family’s need to be with the patient.

Mr. M’s journeyThe 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 temporar-ily 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 wors-ened and he began having severe back pain along with his headache. He also began experiencing altera-tions 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 thera-py 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 dis-charged 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 careLM is a serious complication of cancer with a very poor prognosis, so it’s important for nurses caring for patients with cancer to know

Risk classifications and treatment considerations1,3,8,9

Good risk Poor risk

Performance status high performance status (KPS 60 or greater; ECOG grade 0-2)*

low performance status (KPS less than 60; ECOG grade 3 or higher)*

Neurologic deficits no major neurologic deficits multiple serious, major neurologic deficits

Extent of disease • minimal systemic disease with reasonable treatment options, if needed

• no CSF block

• extensive systemic disease with few treatment options

• bulky CNS disease

• encephalopathy

Treatment considerations

• symptomatic care• LM-directed therapy: radiation therapy

(fractionated external beam radiotherapy/involved field radiotherapy to bulky disease and/or symptomatic sites)

• LM-directed therapy: chemotherapy (insertion of intraventricular catheter with CSF flow scan)

• Other: insertion of VP shunt if signs and symptoms and/or radiologic findings are suggestive of hydrocephalus

• symptomatic care• LM-directed therapy: radiation

therapy (fractionated external beam radiotherapy to symptomatic sites)

• palliative care

*KPS grades on a scale of 0% to 100%, with 0% indicating that the patient has died and 100% indicating that the patient is normal with no complaints or evidence of disease; no special care is needed. A score of 60% or higher indicates that, at minimum, the patient can care for most of his or her personal needs with little or no assistance. ECOG grades on a scale of 0 to 5, with 0 indicating that the patient is fully active and able to carry on all predisease performance without restriction, and 5 indicating that the patient has died. A grade of 2 or less indicates that, at minimum, the patient is ambulatory and capable of all self-care, but is unable to carry out any work activities.

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when to suspect this aggressive and deadly complication. An ear-ly diagnosis is needed to help provide patients with timely and appropriate treatment, support, and care, and improve patient outcomes. ■

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.

2. Jiménez Mateos A, Cabrera Naranjo F, González Hernández A, Fabre Pi O, Díaz Nicolás S, López Fernández JC. Neoplastic meningitis. Review of a clinical series. Neurologia. 2011;26(4):227-232.

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.

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.

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

6. Demopoulos A. Clinical features and diagnosis of leptomeningeal metastases from solid tumors. UpToDate. 2015. www.uptodate.com.

7. MD Anderson Cancer Center. Leptomeningeal metastases. The University of Texas, MD: Anderson Cancer Center; 2014. www.mdanderson.org/education-and-research/resources-for-professionals/clinical-tools-and-resources/practice-algorithms/ca-treatment-leptomeningeal-web-algorithm.pdf.

8. Karnofsky Performance Status Scale. www.hospicepatients.org/karnofsky.html.

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.

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

Diane Schweisguth is a clinical nursing consultant in Lake Oswego, Ore.

The author would like to dedicate this article to her nephew, Ian, whose spirit and smile brighten the lives of many.

The author and planners have disclosed no potential conflicts of interest, financial or otherwise.

DOI-10.1097/01.NURSE.0000473391.13435.97

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