LEPTOMENINGEAL CARCINOMATOSIS

Leptomeningeal Cancer; Neoplastic Meningitis; Carcinomatous Meningitis; Leptomeningeal Metastasis; Leptomeningeal Carcinoma; Meningeal Metastasis

Leptomeningeal Carcinomatosis (LC) is a rare complication of cancer in which cancerous cells spread to the membranes (meninges) that covers the brain and spinal cord. It occurs when cancer cells from other parts of the body, such as the breast, colon, kidney, lungs, and skin metastasize and implant into the covering of the brain and spinal cord.

Diagnostic testing: The diagnosis of LC is made with lumbar puncture with positive CSF (cerebral spinal fluid) cytology; and gadolinium enhanced multiplanar MRI of the brain, spinal cord, cauda equina, or subependymal areas.

Physical findings: Clinical features of this disease include:

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  Headaches;

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  Nausea;

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  Vomiting;

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  Light-headedness;

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  Gait difficulties;

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  Ataxia;

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  Memory problems;

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  Incontinence; and

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  Sensory abnormalities.

ICD-9: 349.2

LC occurs in approximately 5% of people with cancer and is usually terminal. If left untreated, the median survival is 4-6 weeks; if treated, the median survival is 7 months for people with LCs from the breast, and approximately 4 months for people with LCs from small-cell lung carcinomas and melanomas.

The treatment of LC is symptom-specific and palliative. Most people with LC are treated with surgery, radiation, and chemotherapy. Individuals with this disorder are often prescribed analgesics with opioids, anticonvulsants for seizures, antidepressants, and anxiolytics.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

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  Clinical history and examination that describes the diagnostic features of the impairment;

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  Imaging reports of the brain, spinal cord, cauda equina or subepedymal areas showing leptomeningeal enhancement or CSF flow obstruction; and

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  Biopsy reports.

Suggested Listings for Evaluation:

REMARKS

Listing level severity must be documented; recommend allowance under primary malignant tumor listing requiring distant metastasis.

LC with medulloblastomas or other primitive neuroectodermal tumors (PNETs) meets the criteria in listings 13.13 A 1. LC in grade II brain cancers meets the criteria in listing 13.13 A 2 if progressive or recurrent following initial antineoplastic therapy. PNETs are also known as embryonal tumors with multilayer rosettes (ETMRs) due to recent changes in the World Health Organization classification of tumors of the central nervous system.

LC meets the criteria in listing 13.27 when the primary site is unknown.

Listing level severity must be documented; recommend allowance under primary malignant tumor listing requiring distant metastasis.

LC with medulloblastomas or other primitive neuroectodermal tumors (PNETs) meets the criteria in listings 113.13. PNETs are also known as embryonal tumors with multilayer rosettes (ETMRs) due to recent changes in the World Health Organization classification of tumors of the central nervous system.

LC in child grade II brain cancers medically equals the criteria in listing 13.13 A 2 if progressive or recurrent following initial antineoplastic therapy.