Re: Primary CNS Lymphoma or Vasculitis?

Posted By CCF Neurology M.D./DFG on August 01, 1998 at 22:54:28:

In Reply to: Re: Primary CNS Lymphoma or Vasculitis? posted by CCF Neurology MD:NT on July 30, 1998 at 22:31:40:






Writing about current diagnostic difficulties for my daughter (in her 30's).
I have researched numerous articles (using IGM and other sources) and I
volunteer in Medical Library so I have basic information.
All care has been from major Eastern hospital and appear to be excellent.
My question is whether CCF or others have experienced anything like
this? She had a seizure in Dec 96 after a series of headaches going back to
summer of 96.  CSF and blood tests were basically normal and were followed
by an inconclusive MRI-guided steereotactic biopsy. Nonspecific inflammation
was the result and vasculitis was suspected.  No evidence of any other organ
involvement.  MRIs throughout 1997 showed multiple lesions (7 - 10), all in
right cerebral hemisphere.  Most are in right temporal lobe with at one
in right occipital lobe and possibly one or two in frontal lobe. Lesions waxed
and waned somewhat but the dilantin she is taking kept everything in check during
1997.  She's basically otherwise healthy and only missed time from work for
appointments and procedures (MRIs, spinal taps, angiogram, EEG, Ophth, consult
with MS specialist (neg), etc.).  In Jan and Apr she had episodes (aura, short
term confusion) but not definite partial seizures.  MRI at end of May 98 showed
significant growth in two or three of the lesions over Feb 98.  She had partial
seizure in June and the MRI at the end of June MRI showed additional growth.  She
underwent a second biopsy (craniotomy) on July 17 from which she bounced back
with no complications and is already back to partial work days.  Biopsy objective
was to try and rule out lymphoma once and for all.  Results thus far:  specialists
see cuffing of blood vessels with inflammation but no apparent direct infiltration
of vessel walls.  The Neuro-Pathologists are leaning toward vasculitis.  However,
a Hemato-Pathologist believes it is not inconsistent with lymphoma.  Inflammation
is predominately B cells.  Specialized staining now being done to check for genetic
mutation of DNA to check for lymphoma.  Hopefully know the results in 7-10 days.
Other:  ANCA (both types) neg; recent ANA weakly positive (1:40), it was 1:160 once
before; most recent CSF had 8 lymphocyte cells, none malignant; Sjogrens bloods
tests both neg; etc.  Pre- and post-op MRIs still show lesions only in right
hemisphere - none in left.  No treatments (other than dilantin) until we have a
diagnosis.
   As I said at the beginning, anyone had a similar case?  Thanks for listening.      





Dear Tom:
I must compliment you for your most excellent description of your daughter's case. Yes, we see cases like your daughter's relatively frequently (at least a few a year), and it is almost surprising to discover how similar the stories and diagnostic difficulties are.
The predominance of B cells on pathology is indeed quite worrisome for a lymphoma. The proof lies in establishing monoclonality (with kappa/lambda light chain markers, which test, I guess, must have been unrevealing in your daughter's case), or else demonstrating gene rearrangements in the immunoglobulin gene (which is probably the test is pending).
Be reassured that your neuro-oncologists are doing just the right thing. I would like to wish your daughter good luck.



  ____
Dear Tom:
As Dr. NT suggested, this clinical scenario has been witnessed here at
CCF often enough that the suspicion of Primary or Secondary CNS Lymphoma
by your primary physicians is legitimate. By the types of tests you described, the other leading candidates in the differential diagnosis have been thoughtfully considered (i.e. Primary Angiitis of the CNS, Acute Disseminated Encephalomyelitis, MS and atypical varients thereof).
I commend your daughter for having the courage to aggressively pursue
brain biopsy, as this is the only way Lymphoma can be definitively diagnosed or excluded. I am curious about any response to treatment (positive or negative), if any has been attempted at this stage. Also, should the anticipated genetic testing prove to be non-diagnostic, I would be interested in knowing more details about your daughters' neuroimaging, as the localization of the lesions in only one hemisphere
is unusual.
Best wishes to your daughter and the rest of your family.