Introduction: Primary Central Nervous System Lymphoma (PCNSL) is a rare variant of extranodal non-Hodgkin lymphoma most commonly affecting the eyes, brain, spinal cord and leptomeninges. In the United States, it has a reported incidence of 51 cases per year. Among the subset of PCNSL are tumors arising primarily from the intraventricular regions including the choroid plexus. These cases are extremely rare and on literature review, only four cases have been documented worldwide. This is a case report of a confirmed B-cell lymphoma of the choroid plexus, the first reported case in the Philippines.
Results: This is a case of a 79-year-old-male with a 2 month-history of continuous headache accompanied by dizziness and seizures manifesting as blank stares and visual hallucinations. He was brought to the hospital with a decreased sensorium and dysarthria. A cranial magnetic resonance imaging displayed enhancing frond-like structures with prominent lobulations in the body, frontal, and occipital horns of both lateral ventricles extending to the third and fourth ventricles with corresponding hydrocephalus. The primary diagnostic consideration on admission was CNS infection. He underwent left frontal burr, endoscopic biopsy of choroid plexus, exudates, and ependyma followed by septostomy, intraventricular lavage, and placement of Ommaya reservoir. Histologic diagnosis was confirmed to be diffuse large B-cell lymphoma. Metastatic work up was negative. Systemic chemotherapy with methotrexate and rituximab was initiated.
Conclusions: Adults presenting with chronic headache, progressive decline in sensorium and imaging finding of enlarged choroid plexus should prompt the diagnosis of PCNSL. Specific diagnostic modalities and intervention are necessary. Due to the rarity of these types of tumors, its natural history, prognosis and standard of therapy remain a challenge and are yet to be established.
Patient Care: In adult cases presenting with chronic headache, progressive decline in sensorium and an imaging of enlarged choroid plexus, the consideration or diagnosis of these types of tumors cannot be discounted. Specific diagnostic modalities and intervention are necessary. Due to the rarity of these types of tumors, its natural history, prognosis and standard of therapy remain a challenge and are yet to be established.
Learning Objectives: The objective of this paper is to report an additional case of a confirmed B-cell lymphoma of the choroid plexus and present a review of pertinent literature as well as the approach to diagnosis and management of this case—a primary intraventricular lymphoma of the choroid plexus.
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