Primary central nervous system lymphoma with ependymal involvement: analysis of clinical manifestations and cerebrospinal fluid cytological features of 4 cases

Abstract

Objective To analyse clinical and cerebrospinal fluid cytological features of primary central nervous system lymphoma (PCNSL) with ependymal involvement and investigate the diagnostic value of cerebrospinal fluid cytology. Methods Clinical and radiological features of 4 cases of PCNSL were reviewed retrospectively. The findings in conventional cytology and immunocytochemistry of cerebrospinal fluid were analysed. Results All of the patients suffered from headache. Meningeal irritation signs were positive in all the 4 cases. Other clinical features were as follow: radiculoneuropathy in 2 cases, multiple cranial nerves paralysis in one case, hemiplegia in one case, and intermittent low-grade fever in 3 cases. Two cases died of cerebral hernia 2-4 months later after the onset. Lumber puncture was performed in 3 cases and intracranial hypertension was found in 2 cases. One case received ventricular drainage. Cerebrospinal fluid examination showed lymphocyte pleocytosis (4 cases) with elevated protein (4 cases) and lower glucose level (3 cases). The examination of cerebrospinal fluid found lymphocyte pleocytosis with remarkable atypical lymphoma cells in all of the cases. Among 3 patients received cerebrospinal fluid immunocytochemical studies, the lymphocytes were positive for CD19, CD20, CD79a and implied a B-lymphocyte source. Brain magnetic resonance imaging (MRI) showed irregular enhancement of the ventricular wall with nodular periventricular and septal enhancement in part of the 4 cases and pituitary enhancement in one case. Two patients were diagnosed via biopsy of the brain, which showed non-Hodgkin's lymphoma. The other two were diagnosed through cytology and immunocytochemical studies of cerebrospinal fluid. One case received positron emission tomography (PET) examination, which showed no evidence of extracranial involvement. Bone marrow biopsy in other patients were normal too. Conclusion Cytological and immunocytochemical studies of cerebrospinal fluid specimen should be stressed in the diagnosis of PCNSL. This report also emphasizes the importance of clinical and radiological findings in the diagnosis of this disease.

DOI：10.3969/j.issn.1672-6731.2011.05.018