Abstract
BackgroundPrimary central nervous system lymphoma is a rare, malignant non-Hodgkin lymphoma that can arise in the brain, spinal cord, eye, leptomeninges, or cranial nerves. Primary central nervous system lymphoma is rare, accounting for 2–6% of all primary brain neoplasms and 1–2% of all non-Hodgkin lymphomas, and it usually presents as a solitary lesion. Cerebellar involvement is present in only 9% of cases. We present an unusual case of primary central nervous system lymphoma presenting as multiple lesions in the cerebellum in an immunocompetent host.Case presentationA 71-year-old Caucasian man presented to our hospital with acute onset of dizziness, nausea, vomiting, and gait imbalance. Contrast-enhanced computed tomography revealed three intensely enhancing masses in the right cerebellar hemisphere. Whole-body positron emission tomography and computed tomography failed to demonstrate a primary tumor of origin outside the central nervous system. The patient underwent right suboccipital craniotomy with partial resection of the visible tumor from the right cerebellum. Histopathology revealed diffuse large B-cell lymphoma, non-germinal center type.ConclusionsPrimary central nervous system lymphoma is rare, even more so in the cerebellum. However, the overall incidence of primary central nervous system lymphoma is rising in both immunocompromised and immunocompetent patients. The highly aggressive nature of primary central nervous system lymphoma necessitates timely diagnosis and intervention. In this report, we review the available literature for a better understanding of the pathophysiology and management of primary central nervous system lymphoma. To the best of our knowledge, this is the first reported case of a patient with primary central nervous system lymphoma presenting with multiple masses in the cerebellum.
Highlights
Primary central nervous system lymphoma is a rare, malignant non-Hodgkin lymphoma that can arise in the brain, spinal cord, eye, leptomeninges, or cranial nerves
We review the available literature for a better understanding of the pathophysiology and management of primary central nervous system lymphoma
To the best of our knowledge, this is the first reported case of a patient with primary central nervous system lymphoma presenting with multiple masses in the cerebellum
Summary
PCNSL is a rare disease with an increasing incidence in both immunocompromised and immunocompetent individuals. Our patient, who had multiple lesions in the cerebellum, highlights two unusual presentations of this rare disease that made the initial diagnosis challenging. Because imaging in this disease is nondiagnostic, biopsy is crucial to differentiating PCNSL from malignant and benign lesions that present in a similar manner and appear similar on imaging. The highly aggressive nature of PCNSL necessitates timely diagnosis and intervention. Physicians must include this disease in their differential diagnosis of mass lesions found in the central nervous system
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