Leptomeningeal Metastases:
A Comprehensive Guide
For Cancer Patients
Leptomeningeal Metastases:
A Comprehensive Guide
For Cancer Patients
Leptomeningeal
Metastases:
A Comprehensive
Guide For
Cancer Patients
What are Leptomeningeal Metastases?
Cancer is a formidable adversary that can affect any part of the body. One of its most insidious forms is Leptomeningeal Metastases (LM), a rare and often fatal complication of advanced cancer. This patient guide explores the intricate world of leptomeningeal metastases, shedding light on its diagnosis, treatment options, and the current state of research in this field.
Leptomeningeal Metastases (LM) are sometimes referred to as leptomeningeal disease, neoplastic meningitis, meningeal cancer, or carcinomatous meningitis. Leptomeningeal Metastases tend to occur most commonly with cancers that spread to the central nervous system, specifically the leptomeninges and cerebrospinal fluid (CSF). The leptomeninges are the delicate, thin membranes that cover and protect your brain and spinal cord while the CSF flows in and around your brain and spinal cord. With Leptomeningeal Metastases, the cancer involves the pia mater and arachnoid of the leptomeninges as well as the CSF.
How do Leptomeningeal Metastases Occur?
Leptomeningeal Metastases occur when cancer cells from the primary cancer, the original site of the cancer, invade nearby normal tissue. Then, they move through the walls of nearby lymph vessels or blood vessels by traveling to the leptomeninges and CSF where they can form a new metastatic tumor. When cancer cells infiltrate the leptomeninges, they can disrupt normal CSF flow and cause various neurological symptoms. The most common primary cancers giving rise to Leptomeningeal Metastases are breast cancer (12-35% of patients), lung cancer (10-26% of patients), melanoma (5-25% of patients), and gastrointestinal cancer (4-14%).
How Common are Leptomeningeal Metastases?
- Leptomeningeal Metastases are considered to be rare and are diagnosed in approximately 5-8% of patients with solid tumors (breast, lung, melanoma, gastrointestinal) and 5-15% of patients with hematologic malignancies (i.e. leukemia, lymphoma, myeloma).
- It is estimated that 110,000 cases of Leptomeningeal Metastases from solid tumors are diagnosed in the U.S. each year.
What is the Prognosis for Leptomeningeal Metastases?
- Leptomeningeal Metastases are a serious and life-threatening condition.
- Survival for patients with Leptomeningeal Metastases is poor, limited to 3-9 months, depending on the type of primary cancer and treatment administered.
What are the Symptoms of Leptomeningeal Metastases?
- The symptoms result from cancer cells blocking the normal exits for CSF, causing a fluid buildup and increased pressure in the brain.
- This increased pressure can cause vague but uncomfortable symptoms, including headaches (often worse in the morning), nausea, vision changes, and difficulty walking.
The Challenge of Misdiagnosis or Late Diagnosis
Unfortunately, Leptomeningeal Metastases are often not diagnosed or misdiagnosed, leading to patients receiving other treatments to manage symptoms or being sent to hospice care. This is primarily due to the complexity of the disease and the subtlety of its symptoms. The symptoms can be similar to other conditions, making it difficult to diagnose accurately.
How are Leptomeningeal Metastases Diagnosed?
Because Leptomeningeal Metastases is a metastatic disease, it often occurs in patients who are already battling cancer, which can further complicate the diagnosis. Diagnosing Leptomeningeal Metastases can be challenging. Suspicion of Leptomeningeal Metastases may arise if these symptoms develop in someone with other cancers such as breast or lung cancer.
In many cases, Leptomeningeal Metastases can be seen on magnetic resonance imaging (MRI). However, the best test for Leptomeningeal Metastases is to obtain a CSF sample by performing a lumbar puncture (spinal tap). This is when a needle is inserted into the back between the bones of the spine, below the level of the spinal cord, and the CSF is examined for cancer cells under a microscope.
A thorough evaluation is essential, typically involving several diagnostic tools:
Clinical Symptoms Assessment: Physicians start by evaluating the patient’s clinical symptoms. Common symptoms include headache, neck pain, nausea, vomiting, altered mental status, and neurological deficits like weakness or numbness.
Cerebrospinal Fluid (CSF) Analysis: The gold standard for diagnosing Leptomeningeal Metastases is analyzing CSF obtained through a lumbar puncture (spinal tap). This procedure can detect the presence of cancer cells, elevated CSF pressure, or abnormal protein levels.
Imaging Studies: Imaging techniques such as magnetic resonance imaging (MRI) and computed tomography (CT) scans can reveal abnormalities in the brain and spinal cord, such as leptomeningeal enhancement or nodular lesions. Contrast-enhanced MRI is particularly useful for detecting these changes.
Biopsy: In some cases, a biopsy of affected tissue may be necessary for a definitive diagnosis, especially when CSF analysis and imaging studies are inconclusive. This invasive procedure involves the removal of a small tissue sample for examination under a microscope.
Treatment Options for Leptomeningeal Metastases
The treatment for Leptomeningeal Metastases is based on two guiding principles focused on reducing the: (1) pressure on the brain caused by any CSF buildup, and (2) number of cancer cells causing the pressure. Note: Treatment options are based on risk status and individual disease state.
If the CSF pressure is elevated, it can be reduced via lumbar puncture or use of some medications. If needed, a more permanent drain, called a shunt, can be placed by a neurosurgeon. A subset of drugs, including some of the newer oral targeted therapies, can cross the blood-brain barrier and may be used to treat Leptomeningeal Metastases. Other drugs may be administered directly into the CSF through a special port, Ommaya reservoir, placed into the brain by a neurosurgeon. Radiation therapy can also be used to kill cancer cells on the leptomeninges.
The patient’s physician may consider one or more treatment options:
Intrathecal Chemotherapy: Intrathecal chemotherapy involves delivering cancer-fighting drugs directly into the CSF via a lumbar puncture or an Ommaya reservoir. Commonly used drugs include methotrexate, cytarabine, and thiotepa. Intrathecal chemotherapy can help reduce cancer cell growth in the leptomeninges while minimizing systemic side effects.
Radiation Therapy: Radiation therapy may be used to target specific areas of leptomeningeal involvement. Techniques like whole-brain radiation therapy (WBRT) or stereotactic radiosurgery (SRS) can help control tumor growth and relieve symptoms.
Systemic Therapy: Depending on the primary cancer type, systemic therapies like targeted therapies and immunotherapies may be considered. These treatments aim to control the systemic cancer and indirectly affect leptomeningeal metastases.
Symptom Management: Supportive care is essential for managing symptoms such as pain, nausea, and neurological deficits. Medications, physical therapy, and counseling can all play a crucial role in improving the patient’s quality of life.
Clinical Trials: In some cases, participation in clinical trials may offer promising experimental treatments that are not yet widely available. This option can be appropriate for any stage of cancer depending on ongoing trials rather than been seen as a last resort. It is important to research and talk to your healthcare provider about all clinical trial options that you may qualify for.
Conclusion
Living with Leptomeningeal Metastases can be challenging, but understanding the disease and the available treatment options can help patients and their families navigate this journey. It’s important to remember that while Leptomeningeal Metastases is a serious condition, there are treatment options available, and ongoing research continues to explore new therapies. It’s never too early to equip yourself with resources and ask questions.
If you or a loved one has been diagnosed with Leptomeningeal Metastases, it’s crucial to discuss all treatment options with your healthcare provider. If you have additional questions, you can contact our team.
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References
: Cancer Research UK. (2023). What are leptomeningeal metastases?
: Cleveland Clinic. (2023). Leptomeningeal Disease.
: Hofer, S. et al. “Leptomeningeal metastases from solid tumors.” European Medical Oncology 2021
: Le Rhun, E. et al. “Carcinomatous meningitis: Leptomeningeal metastases in solid tumors.” SNI 2013