<I>Intracerebral Infiltration by Monoclonal Plasmacytoid Cells in Waldenstrom''s Macroglobulinemia —Case Report—</I>

Abstract

A 68-year-old female presented with Waldenstrom's macroglobulinemia with infiltration into the cerebral parenchyma manifesting as increased confusion, memory loss, and disorientation. She had a past history of Waldenstrom's macroglobulinemia treated 3 years before. Magnetic resonance imaging showed a high intensity area on T2-weighted images in the left frontal lobe extending to the corpus callosum which was well enhanced by gadolinium-diethylenetriaminepenta-acetic acid. Direct infiltration of neoplastic cells was confirmed by biopsy. Immunohistochemical examination showed that mature plasmacytoid cells in the cerebral parenchyma were immunoglobulin M and lambda light chain antigen positive, but immature lymphocytes in Virchow-Robin space were negative. Monoclonal proliferation was confirmed by southern blot analysis. She became symptom free and the size of the lesion was dramatically reduced after 40 Gy irradiation. She showed no evidence of recurrence 3 years after irradiation. As no effective chemotherapy regimen for Bing-Neel syndrome has been established, irradiation is worth considering when neuroimaging suggests intracranial infiltration of neoplastic cells.

Guideline for the diagnosis, treatment and response criteria for Bing-Neel syndrome

“…The effective use of RT has been described in many case reports, both as first-line and as rescue therapy. 12,59,60 Localized RT to affected lesions at a dose of 30 to 40 Gy may be preferable to whole brain radiation to limit toxicity; this may fail to address widespread deposits that are not apparent on imaging. In general, cerebral RT, even when localized (stereotactic techniques), is associated with enhanced neurotoxicity, especially the occurrence of late neurocognitive effects in elderly patients which can affect up to 80% of patients.…”

Brain Magnetic Resolution Imaging to Diagnose Bing-Neel Syndrome

“…Only extremely rare cases of Bing-Neel syndrome, involving direct CNS infiltration by malignant cells, have been reported 1,3,4,[9][10][11] .…”

Therapeutic Experience of Bing-Neel Syndrome Associated with Waldenstrom's Macroglobulinemia

“…In WM, the CNS may be involved by a variety of mechanisms, including hyperviscosity and direct infiltration by neoplastic cells (7, 8). Patients with Bing-Neel syndrome have sometimes presented with a mass containing neoplastic cells, but the masses have been intraparenchymal rather than meningeal (8, 9). This syndrome can be subdivided into diffuse and tumoral forms.…”

“…However, a review of the literature reveals that the outcome for most patients who underwent chemotherapy was poor, and the patients died within several months (10, 13, 14). Therefore, patients with Bing-Neel syndrome may benefit from cranial radiation therapy prior to chemotherapy (9, 12, 16). Recently, a great deal of interest has been noted by treatments with purine nucleoside analogs (fludarabine, cladribine, and pentostatin) because of their remarkable activity in lymphoproliferative disorders.…”