diseasea
• Physical exam with careful neurologic evaluation
• Brain and spine MRI if patient is a candidate for active treatmentb
• CSF analysisc,d
CSF positive for tumor cells orPositive radiologic findings with supportive clinical findings
orSigns and symptoms with suggestive CSFe in a patient known to have a malignancy
Poor riskf:
• KPS <60
• Multiple, serious, major neurologic deficits
• Extensive systemic disease with few treatment options
• Bulky CNS disease
• Encephalopathy Good risk:
• KPS ≥60
• No major neurologic deficits
• Minimal systemic disease
• Reasonable systemic treatment options, if needed
See Treatment (LEPT-2)
See Treatment (LEPT-2)
Note: All recommendations are category 2A unless otherwise indicated.
Clinical Trials: NCCN believes that the best management of any patient with cancer is in a clinical trial. Participation in clinical trials is especially encouraged.
Version 5.2020 , 04/15/21 © 2021 National Comprehensive Cancer Network® (NCCN®), All rights reserved. NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN.
Leptomeningeal Metastases
LEPT-2
fPatients with exceptionally chemosensitive tumors (eg, small cell lung cancer, lymphoma) may be treated. Patients with a good risk status who do not desire further therapy may also be treated with palliative and/or best supportive care.
gSee Principles of Brain and Spinal Cord Tumor Systemic Therapy (BRAIN-D).
hStrongly consider Ommaya reservoir/intraventricular catheter.
iSee Principles of Brain and Spinal Cord Tumor Radiation Therapy (BRAIN-C).
jDue to substantial toxicity, craniospinal RT should only be considered in highly select patients (eg, leukemia, lymphoma).
RISK STATUS TREATMENT
Poor risk:f
• KPS <60
• Multiple, serious, major neurologic deficits
• Extensive systemic disease with few treatment options
• Bulky CNS disease
• Encephalopathy Good risk:
• KPS ≥60
• No major neurologic deficits
• Minimal systemic disease
• Reasonable systemic treatment options, if needed
Palliative/best supportive care
andConsider involved-field RTi to neurologically symptomatic or painful sites for palliation (including spine and intracranial disease)
• SRS or RT (involved-field and/or whole brain) to bulky disease and neurologically symptomatic (such as cranial neuropathies) or painful sites.i,j
See Assessment of response (LEPT-3)
• Intra-CSF chemotherapyg,h
If symptoms or imaging suggest CSF flow blockage, perform a CSF flow scan prior to starting intra-CSF chemotherapy.
If flow abnormalities confirmed:
◊Fractionated external beam RTi to metastatic or painful sites of obstruction and repeat CSF flow scan to see if flow abnormalities have resolved.
or
◊High-dose methotrexate if breast cancer or lymphoma
• Systemic chemotherapyg
Note: All recommendations are category 2A unless otherwise indicated.
Clinical Trials: NCCN believes that the best management of any patient with cancer is in a clinical trial. Participation in clinical trials is especially encouraged.
Version 5.2020 , 04/15/21 © 2021 National Comprehensive Cancer Network® (NCCN®), All rights reserved. NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN.
Leptomeningeal Metastases
LEPT-3
gSee Principles of Brain and Spinal Cord Tumor Systemic Therapy (BRAIN-D).
iSee Principles of Brain and Spinal Cord Tumor Radiation Therapy (BRAIN-C).
kIf CSF cytology was initially negative, then assess response with MRI of spine/brain.
lIf cytologic analysis is negative from CSF obtained from an Ommaya reservoir, then assess CSF obtained via a lumbar puncture to confirm CSF cytology is negative.
CSF cytology negativek,l
CSF cytology positive
Continue on current regimen and re-evaluate CSF cytology every 4–8 weeks
Maintenance
chemotherapyg and Monitor CSF cytology every 4–8 weeks
Patient clinically stable or improving and there is no evidence of radiologic progression of leptomeningeal disease
Evidence of clinical or radiologic progression of leptomeningeal disease
Continue
chemotherapygfor 4 wks orConsider switching
chemotherapy and treat for 4 wks before re-testing CSF
Consider switching chemotherapy
RTi to symptom sites orSystemic chemotherapyg orPalliative/best supportive care Negative cytology or
persistent positive cytology, but patient is clinically stable
Cytology continually positive and evidence of clinical or radiologic progression of
leptomeningeal disease TREATMENT
Note: All recommendations are category 2A unless otherwise indicated.
Clinical Trials: NCCN believes that the best management of any patient with cancer is in a clinical trial. Participation in clinical trials is especially encouraged.
Version 5.2020 , 04/15/21 © 2021 National Comprehensive Cancer Network® (NCCN®), All rights reserved. NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN.
Leptomeningeal Metastases
SPINE-1
aBiopsy if remote history of cancer.
bIf the patient is unable to have an MRI, then a CT myelogram is recommended, which may also be useful for SRT planning.
c15%–20% of patients have additional lesions. Highly recommend complete spine imaging.
dSee Principles of Brain and Spine Tumor Imaging (BRAIN-A).
eSee Principles of Brain and Spinal Cord Tumor Radiation Therapy (BRAIN-C).
fSee Principles of Brain and Spinal Cord Tumor Systemic Therapy (BRAIN-D).
gIncludes cauda equina syndrome.
PRESENTATION WORKUP TREATMENT
Patient diagnosed with cancer
or patient with newly discovered abnormality suspicious for spine metastasis
Asymptomatic (Incidental finding)
Symptomatic:
• Severe, new, or progressive pain or neurologic symptoms or myelopathy
• Systemic imaging (ie, contrast-enhanced chest/abdominal/
pelvic CT or whole body PET/
CT, bone scan as indicated for metastatic workup)
• Biopsya if it alters management
• Observation
Spine MRId in 6–8 weeks, then every 2–3 months until the nature of the lesion is established
• Surgery/focal RTe or chemotherapyf are options for patients with
asymptomatic epidural disease
Spinal MRIb,c,d
(urgent in the event of neurologic symptoms)
See SPINE-2 No tumor
Spinal cord compressiong
No spinal cord compressiond
Note: All recommendations are category 2A unless otherwise indicated.
Clinical Trials: NCCN believes that the best management of any patient with cancer is in a clinical trial. Participation in clinical trials is especially encouraged.
Version 5.2020 , 04/15/21 © 2021 National Comprehensive Cancer Network® (NCCN®), All rights reserved. NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN.