Primary Central Nervous System Lymphoma: A Critical Review of the Role of Surgery for Resection

Abstract

Background: Primary central nervous system lymphomas (PCNSL) are rare CNS tumors that carry a poor prognosis, with most patients suffering recurrence. Progress has been made in the treatment of this pathology, notably with the widespread use of systemic high dose methotrexate. However, unlike most other malignant CNS neoplasms, surgery for cytoreduction is not routinely performed for this disease, mainly as a result of negative experiences decades ago. Since these studies were published, the availability of intraoperative monitoring, MR imaging and neuro-navigation as well as surgical adjuncts such as fluorescence- guided resection have greatly improved the safety of intracranial procedures. More recent data is suggestive of a potential survival benefit for resection of single PCNSL lesions when patients are subsequently treated with modern regimen high-dose methotrexate, yet this evidence is limited, and should be interpreted conservatively.

Methods and findings: A systematic review of the literature was performed to identify trials evaluating surgical options for the treatment of PCNSL.

Conclusion: In this review, we provide a critical overview of the evidence favoring and discouraging resection for PCNSL. This literature suffers from several biases and limitations that must be considered in the context of the extrapolation of this literature into clinical decision-making.

Keywords: Cytoreduction; Primary central nervous system lymphoma; Surgery.

Figure 1A

Post (L) and pre (R) contrast T1 MRI demonstrating a right temporal homogenously enhancing lesion with surrounding edema (A) The patient underwent surgery for symptomatic relief and local mass effect,and had gross total resection.

Figure 1B

Post (L) and pre (R) contrast T1 MRI demonstrating aright temporal homogenously enhancing lesion with surrounding edema (B) Pathology report showed PCNSL.

Figure 2

Progression free survival (PFS) and overall survival (OS) by extent of resection in 526 subjects, analyzed with the Cox regression model. PFS significantly increased in patients with gross or subtotal resection vs. biopsy (p=0.005), no difference seen in gross total vs. subtotal resection (p=0.023). OS improved for both gross total resection alone and gross or subtotal resection vs. biopsy (p=0.024). No difference in OS seen between gross and subtotal resection (p=0.297).