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Review
. 2022 Apr 20:12:884724.
doi: 10.3389/fonc.2022.884724. eCollection 2022.

An Update on Neurosurgical Management of Primary CNS Lymphoma in Immunocompetent Patients

Florian Scheichel  1   2 , Daniel Pinggera  3 , Branko Popadic  1   2 , Camillo Sherif  1   2 , Franz Marhold  1   2 , Christian Franz Freyschlag  3
Affiliations
Review

An Update on Neurosurgical Management of Primary CNS Lymphoma in Immunocompetent Patients

Florian Scheichel et al. Front Oncol. .

Abstract

Primary central nervous system lymphomas (PCNSL) are rare CNS tumors that harbor a conspicuously longer diagnostic delay compared to other malignant brain tumors. The gold standard for diagnosis is stereotactic biopsy to acquire tissue for histopathological analysis and therefore neurosurgery plays a central role when reducing the diagnostic period is mandated. However, histopathological diagnosis could be complicated if the patient was preoperatively exposed to corticosteroids. Besides the histopathological result, diagnosis of a PCNSL also requires full diagnostic workup to exclude cerebral metastatic disease of a systemic lymphoma. Most reviews of PCNSL discuss recent advancements in systemic treatment options from an (neuro-)oncologic viewpoint, whereas our intention was to discuss the optimization of the diagnostic period and therefore describe current standards of imaging, summarizing the diagnostic workup, discussing the surgical workup and future diagnostic prospects as well as the influence of preoperative corticosteroid therapy to reduce the diagnostic delay of PCNSL patients.

Keywords: Primary central nervous system lymphoma (PCNSL); corticosteroid therapy; diagnostic delay; diagnostic workup; diagnostic yield.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Imaging of a patient with a histopathological proven PCNSL. Unenhanced CT scan showed a hyperdense cerebellar lesion (A). Contrast-enhanced T1-weighted axial MRI showed a strong and homogenous contrast enhancement of the lesion (B). The lesion homogeneously appeared hyperintense in T2-weighted MRI (C). Diffusion restriction was detected as well, resulting in a bright DWI (b = 1,000) (D) and dark ADC map signal (E). 1H-MR spectroscopy showed an increased choline peak and decreased creatinine and N-acetylaspartat (F).
Figure 2
Figure 2
Images of open biopsy of a PCNSL with the aid of 5-ALA fluorescence. Axial and coronal navigational MRI showing a heterogeneous contrast enhancing lesion in the right temporal lobe and the exact location of the biopsy (A, B). Intraoperative images at the biopsy location with strong 5-ALA fluorescence (C, D). A tissue specimen later diagnosed as PCNSL showing positive fluorescence under 405 nm wavelength blue light in another patient (E, F).
Figure 3
Figure 3
A systematic workflow for diagnosis of PCNSL. Ideally, lumbar puncture and CSF analysis should be performed early without delaying biopsy. Non-invasive staging should be performed while waiting for biopsy or histopathological results to reduce diagnostic delay.
See this image and copyright information in PMC

References

    1. Ferreri AJM, Marturano E. Primary CNS Lymphoma. Best Pract Res Clin Haematol (2012) 25:119–30. doi: 10.1016/j.beha.2011.12.001 - DOI - PubMed
    1. Wöhrer A, Waldhör T, Heinzl H, Hackl M, Feichtinger J, Gruber-Mösenbacher U, et al. . The Austrian Brain Tumour Registry: A Cooperative Way to Establish a Population-Based Brain Tumour Registry. J Neurooncol (2009) 95:401–11. doi: 10.1007/s11060-009-9938-9 - DOI - PubMed
    1. Eloranta S, Brånvall E, Celsing F, Papworth K, Ljungqvist M, Enblad G, et al. . Increasing Incidence of Primary Central Nervous System Lymphoma But No Improvement in Survival in Sweden 2000-2013. Eur J Haematol (2018) 100:61–8. doi: 10.1111/ejh.12980 - DOI - PubMed
    1. Villano JL, Koshy M, Shaikh H, Dolecek TA, McCarthy BJ. Age, Gender, and Racial Differences in Incidence and Survival in Primary CNS Lymphoma. Br J Cancer (2011) 105:1414–8. doi: 10.1038/bjc.2011.357 - DOI - PMC - PubMed
    1. Kadan-Lottick NS, Skluzacek MC, Gurney JG. Decreasing Incidence Rates of Primary Central Nervous System Lymphoma. Cancer (2002) 95:193–202. doi: 10.1002/cncr.10643 - DOI - PubMed

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