Importance of Central Nervous System Involvement by Neoplastic Cells in a Patient with Waldenström’s Macroglobulinemia Developing Neurologic Abnormalities

Abstract

The clinicopathology of central nervous system (CNS) involvement with tumor formation in the brain of a patient with Waldenström’s macroglobulinemia (WM) who developed neurologic abnormalities in the absence of blood hyperviscosity is reported. Extravascular infiltration by monoclonal lymphoplasmacytoid cells with considerable perivascular disruption of the brain and reactive gliosis was a conspicuous histopathologic picture. The tumor as well as the patient’s neurologic symptoms were successfully controlled by local radiation to the brain. This case report illustrates the importance of CNS involvement by neoplastic cells in the differential diagnosis of a patient with WM developing neurologic abnormalities.

Nineteen‐year follow‐up of Waldenström's‐associated neuropathy and Bing–Neel syndrome

“…The tumoral form represents a central nervous system lymphoma consisting of lymphoplasmocytic cells, and it usually presents with focal deficits or seizures. 11,21,24 BNS has been treated with a variety of therapies, including surgery, radiotherapy, and chemotherapy, with variable success. 10 …”

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

“…The lesion may have developed due to vascular endothelial cell injury caused by malignant cell perivascular accumulation. Previous histopathologic studies have revealed considerable lymphoid cell infiltration in the meninges and along vessels with perivascular plasma cell accumulation in VirchowRobin spaces 3,6,13) .…”