Diagnosis of primary central nervous system lymphoma: a systematic review of the utility of CSF screening and the role of early brain biopsy

Abstract

Background Because less-invasive techniques can obviate the need for brain biopsy in the diagnosis of primary central nervous system lymphoma (PCNSL), it is common practice to wait for a thorough initial work-up, which may delay treatment. We conducted a systematic review and reviewed our own series of patients to define the role of LP and early brain biopsy in the diagnosis of PCNSL. Methods Our study was divided into 2 main sections: 1) systematic review assessing the sensitivity of cerebrospinal fluid (CSF) analysis on the diagnosis of PCNSL, and 2) a retrospective, single-center patient series assessing the diagnostic accuracy and safety of early biopsy in immunocompetent PCNSL patients treated at our institution from 2012 to 2018. Results Our systematic review identified 1481 patients with PCNSL. A preoperative LP obviated surgery in 7.4% of cases. Brain biopsy was the preferred method of diagnosis in 95% of patients followed by CSF (3.1%). In our institutional series, brain biopsy was diagnostic in 92.3% of cases (24/26) with 2 cases that required a second procedure for diagnosis. Perioperative morbidity was noted in 7.6% of cases (n = 2) due to hemorrhages after stereotactic brain biopsy that improved at follow-up. Conclusions The diagnostic yield of CSF analyses for PCNSL in immunocompetent patients remains exceedingly low. Our institutional series demonstrates that early biopsy for PCNSL is safe and accurate, and may avert protracted work-ups. We conclude that performing an early brain biopsy in a suspected case of PCNSL is a valid, safe option to minimize diagnostic delay.

CXCL13 and CXCL9 CSF Levels in Central Nervous System Lymphoma—Diagnostic, Therapeutic, and Prognostic Relevance

“…MRI alone does not allow a reliable distinction from other CNS lesions of neoplastic, inflammatory, or infectious origin ( 6 , 7 ). In <15%, positive cerebrospinal fluid (CSF) or vitreous biopsy for lymphoma can eliminate the need for histopathologic confirmation by brain biopsy ( 8 ). However, brain biopsy is required to diagnose the vast majority of CNSL patients.…”

Diagnosis, prognosis and treatment of primary central nervous system lymphoma in the elderly population (Review)

“…The most common presentation is focal neurological symptoms, which have been observed in 70% of patients (21). In addition, a total of 43% of patients presented with neuropsychiatric symptoms, followed by signs of increased intracranial pressure, such as headache and vomiting in 33%, seizures in 14% and ocular symptoms in 4% of cases (21). In addition, 'B symptoms', such as weight loss, fever and night sweats have been reported to be rare in PCNSL (21) (Fig.…”

“…PCNSL nearly always exhibits significant contrast uniform enhancement, with or without necrosis, on computed tomography (CT) and magnetic resonance imaging (MRI) scans (21). Furthermore, linear enhancement along perivascular spaces is highly characteristic of PCNSL (22).…”

Effectiveness of digital PCR for MYD88L265P detection in vitreous fluid for primary central nervous system lymphoma diagnosis

“…Consequently, a high rate of false negative results is usually observed when the number of tumor cells is low in the CSF/VFR. In addition, treatment with chemotherapy and steroids may negatively impact the number of intact tumor cells in the CSF/VRF (47). These shortcomings can be overcome by the analysis of circulating tumor DNA in CSF/VRF samples.…”