Ann Clin Neurophysiol.  2022;24(2):63-67. 10.14253/acn.2022.24.2.63.

A case of primary central nervous system lymphoma diagnosed with cerebrospinal fluid analysis: replacement brain biopsy with cerebrospinal fluid immunohistochemistry and immunoglobulin gene rearrangement

• Lee JH
• Yu S
• Lee JY
• Kim YM
• Lee DA
• Kim SE

• KMID: 2535720
• DOI: http://doi.org/10.14253/acn.2022.24.2.63

Abstract

Primary central nervous system lymphoma (PCNSL) is a type of non-Hodgkin lymphoma confined to the central nervous system. Its diagnosis requires a stereotactic biopsy, which is an invasive procedure. Cerebrospinal fluid (CSF) analysis is less invasive and easier to perform than a stereotactic biopsy. We hereby report a PCNSL case diagnosed using CSF analysis and treated with systemic chemotherapy.

Keyword

Lymphoma; Central nervous system; Immunoglobulin gene

Figure

• 

  Fig. 1. (A) Gadolinium-enhanced T1-weighted images showing nodular contrast enhancement in the ependymal cells of the fourth and lateral ventricles (white arrowheads). (B) Gadolinium-enhanced fluid-attenuated inversion recovery images revealing diffuse leptomeningeal enhancement along with brainstem and nodular contrast enhancement in the ependymal cells of the fourth ventricle (white arrows).

• 

  Fig. 2. Monoclonal rearrangement of immunoglobulin heavy chain (IGH) and immunoglobulin κ light chain (IGK) framework regions in the patient: (A) 260 bp of the IGH-B region, (B) 116 bp of the IGH-C region, and (C) 290 bp of the IGK-A region.

• 

  Fig. 3. (A) Gadolinium-enhanced T1-weighted images indicating improvement of previous nodular enhancement in the periventricular lesion (yellow arrowheads). (B) Gadolinium-enhanced fluid-attenuated inversion recovery images indicating improvement in diffuse leptomeningeal enhancement along with brainstem and nodular contrast enhancement (yellow arrows).

Reference

1. Grommes C, DeAngelis LM. Primary CNS lymphoma. J Clin Oncol. 2017; 35:2410–2418.

2. Kleinschmidt-DeMasters BK, Damek DM, Lillehei KO, Dogan A, Giannini C. Epstein Barr virus-associated primary CNS lymphomas in elderly patients on immunosuppressive medications. J Neuropathol Exp Neurol. 2008; 67:1103–1111.

3. Scott BJ, Douglas VC, Tihan T, Rubenstein JL, Josephson SA. A systematic approach to the diagnosis of suspected central nervous system lymphoma. JAMA Neurol. 2013; 70:311–319.

4. Weller M. Glucocorticoid treatment of primary CNS lymphoma. J Neurooncol. 1999; 43:237–239.

5. Langerak AW, Groenen PJ, Brüggemann M, Beldjord K, Bellan C, Bonello L, et al. EuroClonality/BIOMED-2 guidelines for interpretation and reporting of Ig/TCR clonality testing in suspected lymphoproliferations. Leukemia. 2012; 26:2159–2171.

6. Ekstein D, Ben-Yehuda D, Slyusarevsky E, Lossos A, Linetsky E, Siegal T. CSF analysis of IgH gene rearrangement in CNS lymphoma: relationship to the disease course. J Neurol Sci. 2006; 247:39–46.

7. Kros JM, Bagdi EK, Zheng P, Hop WC, Driesse MJ, Krenacs L, et al. Analysis of immunoglobulin H gene rearrangement by polymerase chain reaction in primary central nervous system lymphoma. J Neurosurg. 2002; 97:1390–1396.

8. Baehring JM, Hochberg FH, Betensky RA, Longtine J, Sklar J. Immunoglobulin gene rearrangement analysis in cerebrospinal fluid of patients with lymphoproliferative processes. J Neurol Sci. 2006; 247:208–216.