From the ER to the OR: Initial Evaluation of Primary Central Nervous System Lymphoma

Abstract

Background: Primary central nervous system lymphoma (PCNSL) is a rare, often curable neoplasm, often initially presenting in acute care settings by nonneuroscience specialized physicians. Delays in the recognition of specific imaging findings, lack of appropriate specialist consultation, and urgent incorrect medication administration can delay necessary diagnosis and treatment.

Review summary: In this paper, the reader is moved quickly from the initial presentation to the diagnostic surgical intervention for PCNSL in a manner analogous to the experience of clinicians in the frontline setting. We review the clinical presentation of PCNSL, its radiographic features, the effect of prebiopsy steroids, and the role of a biopsy in the diagnosis. In addition, this paper revisits the role of surgical resection for PCNSL and investigational diagnostic studies for PCNSL.

Conclusion: PCNSL is a rare tumor that is associated with high morbidity and mortality. However, with appropriate identification of clinical signs, symptoms, and key radiographic findings, the early suspicion of PCNSL can lead to steroid avoidance and timely biopsy for rapid administration of the potentially curative chemoimmunotherapy. Surgical resection presents the potential for improving outcomes for patients with PCNSL, however, this remains controversial. Further research into PCNSL presents the opportunity for better outcomes and longer livelihoods for patients.

Figure 1.

Axial Magnetic Resonance Imaging (A) T1 post-contrast demonstrating an area of homogenous enhancement in the left basal ganglia and internal capsule, FLAIR demonstrating (B) an associated area of increased signal which no marked edema surrounding the large enhancing lesion, and diffusion weighted imaging (C) demonstrating restricted diffusion correlating with the area of contrast enhancement.

Figure 2.

(A) Axial Magnetic Resonance Imaging T1 post-contrast image demonstrating a homogenously enhancing primary central nervous system lymphoma. This lesion (B) demonstrates increased fluorodeoxyglucose uptake on Positron Emission Tomography.