PRIMARY
CENTRAL NERVOUS SYSTEM LYMPHOMA |
ALTERNATE NAMES
|
PCNSL; Primary CNS Lymphoma; Reticulum Cell Sarcoma; Diffuse Histiocytic Lymphoma;
Brain Lymphoma; Cerebral Lymphoma; Primary Lymphoma of the Central Nervous System;
Lymphoma-Brain
|
DESCRIPTION
|
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/ICD-10-CM
CODING
|
Diagnostic testing: 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.
Physical
findings:
-
-
-
-
-
-
-
•
Muscle weakness and numbness in the extremities;
-
-
•
Seeing floating spots; and
-
ICD-9: 200.5
ICD-10: C72.9
|
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
|
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); and
-
|
Suggested Listings for
Evaluation: |
DETERMINATION
|
LISTING
|
REMARKS
|
Meets |
14.11
|
|
114.11
|
|
Equals |
|
|
* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
|