Case report of unusual leukoencephalopathy preceding primary CNS lymphoma

Brecher, K.; Hochberg, Fred H.; Louis, D. N.; Monte, Suzanne de la; Riskind, Peter

doi:10.1136/jnnp.65.6.917

Cited by 45 publications

(48 citation statements)

References 20 publications

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“…Epstein-Barr virus involvement in the pathogenesis of CNS lymphoma in immunocompetent patients is less consistent. Although Epstein-Barr virus was not causative in our patients, as shown by our negative PCR studies for the Epstein-Barr virus genome, our 2 patients and those previously reported by Alderson et al [22] and Brecher et al [23] (in which "sentinel lesions" preceded diagnostic biopsies of primary CNS lymphoma by 7-11 months) suggest a common pathogenic mechanism for both the prodrome and primary CNS lymphoma. Based on those cases we have to raise the possibility that lymphocytic infiltrates that accompany leukoencephalopathy may undergo transformation to malignant B cell populations.…”

Section: Discussionsupporting

confidence: 49%

Central Nervous System Lymphoma in Children

Porto

Kieslich

Schwabe

et al. 2005

Pediatric Hematology and Oncology

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This paper describes the rare MR and CT features of central nervous system (CNS) lymphoma in immunocompetent children and in survivors of childhood acute lymphoblastic leukemia (ALL) and discusses the causative role of cranial irradiation and/or leukoencephalopathy preceding central nervous system (CNS) lymphoma in survivors of childhood leukemia. The authors reviewed MR and CT scans of 3 children with biopsy-proved CNS lymphoma. One child had tumor infiltration within the optic nerve sheaths and optic chiasm by previously known non-Hodgkin lymphoma. In 2 patients, CNS lymphoma developed 8 and 10 years after initial ALL treatment. In both cases CNS lymphoma was preceded by cranial irradiation and leukoencephalopathy. A single lesion was present in 3 out of 4 patients. All lesions were isointense or hypointense on the T1-weighted images relative to gray matter and showed homogeneous enhancement. One lesion was centered in the central gray matter, one lesion was centered within a cerebral hemisphere, one lesion was in optic nerve, and there were 2 parasellar lesions. CNS lymphoma has a variable appearance in children. Knowledge of risk factors in children may help in the early recognition of disease, allowing for timely intervention. This may prompt early biopsy or a conservative management in the appropriate clinical setting.

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Section: Discussionsupporting

confidence: 49%

Central Nervous System Lymphoma in Children

Porto

Kieslich

Schwabe

et al. 2005

Pediatric Hematology and Oncology

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“…The typical patient is male, in his seventh decade, and presents with the syndrome of a subacute mass lesion. In rare instances, PCNSL can be preceded by prodromal symptoms and even in ammatory demyelinating lesions (Alderson et al, 1996;Bender and Schapiro, 1989;Brecher et al, 1998;DeAngelis, 1990). The typical MRI features of PCNSL are isointense to hypointense and hyperintense parenchymal masses on T1-weighted, proton density, and T2-weighted scans; homogeneous and intense contrast enhancement; contact with CSF, either at the ependymal or the pial surface; and decreased contrast enhancement in patients given corticosteroids (Jack et al, 1986).…”

Section: P T-pcnslmentioning

confidence: 99%

Posttransplant primary CNS lymphoma

Phan¹

2000

Neuro-Oncology

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The records and neuro-imaging studies of 8 cases of posttransplant primary CNS lymphoma (PT-PCNSL) diagnosed at Mayo Clinic Rochester between 1970 and 1998 were reviewed retrospectively. All patients received organ transplantation. Patients who had hematologic transplantation were not included in the analysis. The median and mean age of the 4 men and 4 women was 45 years (range, 34 to 50 years). The median duration of symptoms before diagnosis was 36 days (range, 5 to 98 days). At diagnosis, the neurologic examination was focally abnormal in 6 of 8 patients. Compared with the initial computed tomographic study, MRI showed 25 additional brain lesions. Only 43.7% of lesions enhanced with contrast agent; of those that did, all but one were heterogeneous. Ependymal contact occurred in 5 patients. MRI lesion burden increased proportionally to the interval between scans. Diagnostic tissue was obtained by stereotactic biopsy from 6 patients and by open biopsy from 2. Hemorrhage occurred in the biopsy area in 4 patients who had stereotactic biopsy and 2 died (all had normal coagulation studies). Slides available for review (7 patients) showed that the tumors were of CD20-positive lineage and were positive for Epstein-Barr virus, using in situ hybridization. Six patients died. Median survival for the cohort was 13 weeks. PT-PCNSL has clinical and imaging features distinct from typical PCNSL. In our series, (1) PT-PCNSL presented nonspeci cally and progressed rapidly, (2) stereotactic brain biopsy had signi cant morbidity, and (3) despite multimodal therapy, survival was poor. NeuroOncology 2, 229-238, 2000 (Posted to Neuro-Oncology [serial online], Doc. 00-034, September 13, 2000

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“…PCNSL presenting as an infiltrative lesion of the brain has sometimes been misdiagnosed initially as a demyelinating disease. 10,11 In doubtful cases a tissue biopsy is essential to guide management. [12][13][14] Thus, some neurological diseases may present with Weber's syndrome, masquerading as a midbrain stroke.…”

Section: Discussionmentioning

confidence: 99%