COMPASSIONATE ALLOWANCE INFORMATION

LEPTOMENINGEAL CARCINOMATOSIS

ALTERNATE NAMES

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

DESCRIPTION

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. Clinical features of this disease include: headaches, nausea, vomiting, light-headedness, gait difficulties, ataxia, memory problems, incontinence, and sensory abnormalities.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING

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: (See above)

ICD-9: 349.2

ONSET AND PROGRESSION

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.

TREATMENT

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:

• Clinical history and examination that describes the diagnostic features of the impairment.

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

• Biopsy reports.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets Listing

13.00

13.13 A

13.27

113.00

113.13 A

Listing level severity must be documented; recommend allowance under primary malignant tumor listing requiring distant metastasis. LC meets 13.27 when the primary site is unknown. LC with medulloblastomas or other primitive neuroectodermal tumors (PNETs) meets 13.13A1 and 113.13. LC in grade II brain cancers meets 13.13A2 if progressive or recurrent following initial antineoplastic therapy.

Equals Listing

13.13A

LC in child grade II brain cancers medically equal 13.13A2 if progressive or recurrent following initial antineoplastic therapy.

* Adjudicators may, at their discretion, use the Medical Evidence of Record or Listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.