TN 16 (03-18)
DI 23022.640 Primary Central Nervous System Lymphoma
COMPASSIONATE ALLOWANCE INFORMATION
PRIMARY CENTRAL NERVOUS SYSTEM LYMPHOMA
PCNSL; Primary CNS Lymphoma; Reticulum Cell Sarcoma; Diffuse Histiocytic Lymphoma; Brain Lymphoma; Cerebral Lymphoma; Primary Lymphoma of the Central Nervous System; Lymphoma-Brain
Primary Central Nervous System Lymphoma (PCNSL) is a rare cancer that involves the central nervous system (brain or spinal cord), and/or the coverings of the brain (meninges). It is a non-Hodgkin B-cell lymphoma that typically stays within the central nervous system (CNS) and rarely metastasizes (spreads outside) of the CNS. PCNSL and HIV are strongly associated with co-infection of Epstein-Barr virus (EBV). Symptoms and signs of PCNSL vary, depending on the area of the brain that is involved and include severe headaches, changes in speech, personality changes, confusion, memory problems, drowsiness, muscle weakness, and numbness in the extremities. Some people have seizures. People with eye involvement report seeing floating spots (floaters), and gradual vision loss.
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING
Diagnosis of PCNSL involves clinical examination, brain imaging, cerebrospinal fluid (CSF) cytology, and infrequently brain tissue biopsy. Clinical evaluation may include a neurological examination or ophthalmologic examination. Imaging includes brain MRI or CT scan. The tumor often presents as a solitary ring- enhancing lesion located in the cerebral hemisphere; however, it could present as multiple lesions located in other areas of the brain. If the eye is involved, a biopsy is taken from the eye (vitrectomy or choroid/retinal biopsy). The definitive diagnosis of PCNSL is documented by CSF cytology or by brain biopsy.
ONSET AND PROGRESSION
PCNSL affects all age groups, but is most commonly diagnosed in people who are over age 50 and people with very low CD4 counts (generally <50 cells/µL) . The survival of untreated PCNSL is under 2 months from the time of presentation. A combination of chemotherapy and radiation therapy may increase survival to about 44 months.
Treatment options are dependent on factors such as the person’s age and general health; stage of cancer; location of the tumor in the central nervous system; and the activity of the tumor (i.e. recurrent or metastatic). Corticosteroids are prescribed to reduce brain swelling caused by the tumor. Multiple therapies such as chemotherapy, high dose chemotherapy with stem cell transplant and radiation are used in the treatment of PCNSL. Surgery is not used to treat primary CNS lymphoma because of the infiltrative nature of the tumor and the fact that the tumor usually involves several structures within the brain.
SUGGESTED PROGRAMMATIC ASSESSMENT*
Suggested MER for Evaluation:
Documentation of HIV infection, CSF cytology report, or tissue biopsy report
Clinical description of findings; neurological or ophthalmological examination report(s); CT scan or MRI report
Suggested Listings for Evaluation:
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* 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.