Why primary?
Lymphoma is a cancer that is derived from lymphocytes; we are most used to hearing about Hodgkin's and non-Hodgkin's lymphoma.
Lymphoma may start out in the lymph nodes or it may start in the bloodstream, but in PCNSL, the lymphoma starts in the CNS (central nervous system) itself. This means that the cancer cells do not spread (metastasize) from the lymph nodes to the brain, but instead, start out in the brain (or leptomeninges - covering of the CNS, spinal cord or eyes).
Who gets PCNSL?
It was felt to be a, relatively, rare form of cancer, but it appears that its incidence may be on the rise. It is seen in 0.46 per 100,000 (46 people per 10,000,000) and is two times more often in men (in HIV patients, 95% are men). In people with HIV/AIDS, it may be seen a lot more frequently (6-20%). The median age for non-HIV patients is 55 years and for HIV patients, it is 35 years.
What is the treatment and what are the outcomes?
PCNSL is very responsive to steroids, and this is why a biopsy should usually be performed prior to steroids. Steroids reduce swelling (edema), but do not usually cause the tumor to go away.
PCNSL is a fatal cancer, but survival may be slightly increased by radiation and chemotherapy.
What testing is needed?
- MRI with and without Gadolinium
- Eye (ophthalmologic) exam (includes slit-lamp)
- Spinal tap
- HIV test
- CT of the chest, abdomen and pelvis
- Bone marrow biopsy
Usually the lesions are hypointense (dark) on T1-weighted images and enhances after the administration of Gadolinium.
MS (multiple sclerosis) lesions also may enhance, but PML (progressive multifocal leukoencephalopathy) usually does not (one of the recent PML cases in a natalizumab [Tysabri(R) -- Biogen Idec and Elan Pharmaceuticals: BIIB and ELA] treated patient did have Gadolinium enhancement, though.
Chief Complaint (CC): A 59 year-old right-handed white woman complains of 3 months of fatigue, nausea, light-headedness, anorexia (with a 15 lb. weight loss) and left hand weakness.
History of present illness (HPI): She presented to outside hospital 12/07/03 with the above symptoms, 3 weeks after eye surgery.
The brain MRI was interpreted as “embolic strokes.”
A transesophageal echocardiogram (TEE) – small patent foramen ovale (PFO) and she was started on coumadin (warfarin).
She was re-hospitalized 1 week later and the MRI was interpreted as “metastatic neoplasm.”
She was started on: dexamethasone (steroids).
She had a complex partial seizure on 12/25/03 -- which started with left hand shaking.
Dilantin (phenytoin) was started
She was re-hospitalized on 01/15/04 with hyperventilation (blood gas showed a PCO2 = 9.8).
The MRI was interpreted as– “demyelination.”
At this point she was sent to a tertiary care hospital.
She had an additional MRI:
