Case Report
Prolonged interval between sentinel pseudotumoral demyelination and development of primary CNS lymphoma

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Abstract

Primary central nervous system lymphoma (PCNSL) can be associated with preceding demyelinating pseudotumoral brain lesions. The ‘sentinel’ demyelinating lesions recede spontaneously or with corticosteroid, and are followed by development of PCNSL typically within 12 months. This report describes a 29 year-old post-partum woman who developed PCNSL 4 years after a biopsy-proven pseudotumoral demyelinating episode. She presented with focal seizures in February 2005. She subsequently developed hemiparesis and raised intracranial pressure. MRI showed two contrast enhancing lesions in the right frontal lobe, which were hypermetabolic on 18F-FDG PET. A provisional diagnosis of tumefactive multiple sclerosis was made. Symptoms recurred despite multiple courses of high dose corticosteroid. Brain biopsy confirmed large B-cell non-Hodgkin’s lymphoma. This patient illustrates the importance of considering PCNSL in patients presenting with a space-occupying lesion, even with previously confirmed demyelination, and that the interval between the two events may be several years.

Section snippets

Case report

A 29-year-old woman initially presented in 2001 with 6 weeks of headache and lethargy, and 4 days of left-sided facial droop and limb weakness. MRI revealed a left heterogenous 4 × 3 cm contrast-enhancing lesion near the basal ganglia and the posterior limb of the internal capsule (Fig. 1). Clinically, she responded rapidly to a course of oral dexamethasone. A frameless stereotactic-guided brain biopsy of the lesion a week later showed evidence of inflammatory demyelination (Fig. 2). For the

Discussion

Primary CNS lymphoma is a rare high-grade non-Hodgkin’s lymphoma. In immunocompetent patients, they are almost exclusively of B-cell type. The mean age at diagnosis is 60 years, with slight predominance in men. Mortality is high, and recurrence is common despite high-dose chemoradiotherapy.

Primary CNS lymphoma can be associated with preceding demyelinating pseudotumoral lesions, as briefly described in several case reports.[1], [2], [3] However, little is known about this rare occurrence. The

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