spontaneous temporary remission in primary CNS lymphoma

Abstract

We report a case of primary CNS lymphoma in which complete, though temporary, spontaneous clinical and radiologic remission occurred. This is the first such case report to our knowledge.

Case Details

Disease Location

Primary cns (left fronto-parietal region, right centro-temporal regions)

Personal Characteristics

56 -year-old female

Clinical Characteristics

Admitted due to hemiparesis and an abnormal CT she was last well 21 month earlier when she awoke one morning with right-sided weakness which progressed over two days and then stabilized neurological exam only disclosed a mild, spastic right hemiparesis eeg revealed minimal asymmetry over the centro-temporal regions. An infused CT showed a round 1cm enhancing nodule in the left fronto-parietal region that was not seen on the plain CT. There was considerable surrounding white matter edema but no midline shift. The patient was sent home with no biopsy due to the critical location. 6 weeks later she had a follow-up, CT showed a new enhancing dense nodule in the left hemisphere, several centimeters below and slightly lateral to the original nodule. She was seen as an outpatient and followed up 4 months later 1 year later (13 months), she returned with a 3 week history of increasing left sided weakness accompanied by mild occipital headache general physical revealed early papilledema and mild spastic left hemiparesis. Eeg showed mild to moderate irregularity over the right centro-temporal region. CT showed multiple enhancing lesions in the right cerebral and cerebellar hemispheres, with edema and mass effect lumbar puncture yielded clear csf with 67 rbcs and 6 lymphocytes per cc. Lymphocytes were small and mature with rare cells showing atypical features. Csf protein was 124 mg/dl. IGG was 13.3% with negative oligoclonal banding. Sugar was 70mg/dl. Tentative diagnosis was ms and started tapering doses of prednisone. She was seen 4 weeks later after discontinuing prednisone, 3 days later left hemiparesis recurred. She was restarted on prednisone but weakness progressed and she started experiencing gait ataxia and headache. Exam again revealed bilateral papilledema and left spastic hemiparesis. CT with and witho infusion showed the high rght fronto-parietal lesion to be larger, plus a new small left frontal enhancing lesion. Decadron was started and a decompressive right frontal biopsy and excision of mass was performed pathology of the biopsy showed diffuse infiltration of large cell type with high mitotic activity. Accumulation of cells in perivascular spaces was prominent in some fragments pyearsoninophilia was demonstrated by mgp stain. Immunofluorescence on snap frozen tissue showed monoclonal positivity for IGM-lambda consistent with a b cell lymphoma this was interpreted as diffuse lymphoma immunoblastic b cell type the patient did well post-op and was discharged on steroids, radiotherapy was arranged on an outpatient basis

Remission Characteristics

At the 6 week follow-up, she felt perfectly well and her exam was normal. Despite a new nodule on CT, the original hadreturned to normal 4 months later, she was well and CT completely normalized 4 weeks after starting prednisone, she was seen feeling well and her neurological exam was unremarkable and her steroids were discontinued

Treatment & Mechanisms

Proposed Remission Mechanisms

Steroids lymphoma cells may be sensitive to glucocorticoid-killing activity

Clinical Treatment

Prednisone decadron excisional biopsy post-op steroids and radiotherapy

Non-Clinical Treatment

None reported