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Table of Contents
LETTERS TO THE EDITOR
Year : 2022  |  Volume : 25  |  Issue : 6  |  Page : 1206-1208
 

Primary CNS lymphoma masquerading as ventriculitis


1 Department of Neurology, Dr. D Y Patil Medical College, Hospital and Research Center, Dr. DY Patil Vidyapeeth Pune, Maharashtra, India
2 Department of Radiology, Star Imaging and Research Center, Pune, Maharashtra, India

Date of Submission19-Jul-2022
Date of Decision12-Sep-2022
Date of Acceptance15-Sep-2022
Date of Web Publication17-Nov-2022

Correspondence Address:
Shalesh Rohatgi
Dr. D Y Patil Medical College, Hospital and Research Center, Pimpri, Pune - 411 060, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aian.aian_625_22

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How to cite this article:
Rohatgi S, Rao P, Nirhale S, Naphade P, Deshmukh Y. Primary CNS lymphoma masquerading as ventriculitis. Ann Indian Acad Neurol 2022;25:1206-8

How to cite this URL:
Rohatgi S, Rao P, Nirhale S, Naphade P, Deshmukh Y. Primary CNS lymphoma masquerading as ventriculitis. Ann Indian Acad Neurol [serial online] 2022 [cited 2023 Feb 6];25:1206-8. Available from: https://www.annalsofian.org/text.asp?2022/25/6/1206/361415




Sir,

We report a case of a 74-year-old female patient who presented with complaints of irrelevant talk, excessive sleepiness, and delayed verbal response for 25 days. No h/o fever, seizures, headache, or vomiting. No history of any rash. The patient was admitted to a hospital and was found to have hyponatremia, which was corrected. However, there was no improvement in her condition and subsequently, the patient was transferred to our hospital. Clinical examination revealed that she was afebrile and had no lymphadenopathy or organomegaly. She was drowsy and disoriented. No signs of meningeal irritation were present and there was no other neurological deficit. Routine lab investigations including hemogram, liver function tests, renal function tests, electrolytes, and thyroid function tests were normal. The erythrocyte sedimentation rate was 65 mm fall.

MRI brain [Figure 1] revealed T2 and Fluid-attenuated inversion recovery (FLAIR) hyperintensity involving the wall of the ventricular system that showed diffusion restriction and post-contrast enhancement, suggestive of ventriculitis. MRI of the spine was unremarkable. Her CSF examination revealed a pressure of 18 Cms, proteins 235 mg%, glucose 7 mg%, (blood sugar 126 mg%), and200 cells; predominantly lymphocytes. There were no malignant cells seen. CSF Gram's stain and Acid Fast Bacilli (AFB) stain were negative. Cryptococcal antigen was negative. The bacterial culture showed no growth.
Figure 1: MRI Brain Images. (a and b) T2 FLAIR images showing hyperintensities along the ventricles and septum (black arrows); (a and d) Contrast enhanced T1 images showing enhancement along the ventricular walls (black arrows); (e and f) DWI images showing diffusion restriction along the ventricular walls (black arrows)

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TB PCR was negative. HIV was negative. EBV, VZV, HSV1, HSV2, CMV PCR was negative. Initial magnetic resonance imaging (MRI) spine was normal. In view of the CSF picture, the possibility of malignancy was considered, and repeat CSF was sent for flow cytometry that showed 50% of large atypical lymphoid cells which were myeloperoxidase positive. Cells were characterized by the expression of CD19, eCD79a, CD10, CD20, CD22, and HLA-DR and negative for cCD3, sCD3, CD8, CD34, CD13, CD117, CD33, Kappa, and Lambda. The findings were suggestive of B cell non-Hodgkin's lymphoma. PET-CT was done that showed hypermetabolic nodular thickening in the interventricular septum and along the ventricles. There was no increased uptake in any other organ, suggesting it to be primary CNS lymphoma. The patient was treated with high-dose methotrexate with leucovorin and rituximab. She was discharged after the first pulse. She reported 2 weeks later with complains of burning pain in both lower limbs along with weakness. Contrast-enhanced, fat-saturated T1W image of the lumbar spine showed diffuse leptomeningeal enhancement surrounding lower dorsal cord and conus, suggestive of leptomeningeal dissemination of lymphoma [Figure 2]. The patient refused any further treatment and was discharged.
Figure 2: Contrast enhanced fat saturated T!W midsagittal image of lumbar spine shows diffuse leptomeningeal enhancement surrounding lower dorsal cord

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Primary CNS lymphoma (PCNL) is a rare form of extra-nodal highly invasive non-Hodgkin's lymphoma. Ninety percent arise from B cells and 10% arise from T cells. It can occur in both immune-competent and immune-compromised patients. In immune-competent patients, it accounts for almost 4% of all intracranial neoplasms and 4-6% of extra-nodal lymphomas.[1] Radiological imaging usually reveals a solitary homogenously enhancing mass in the supratentorial region. PCNL involving the ventricular system is very rare and accounts only for 1% of all primary CNS tumors. Only a few cases of involvement of the ventricles have been reported in the literature and that too all presented as mass in the ventricles.[2],[3],[4],[5]

Isolated case reports of involvement of only lateral ventricles,[6] only fourth ventricle[3],[7],[8] and only third ventricle[3],[9],[10] are available but to the best of our knowledge; this is the first case of primary CNS lymphoma presenting as ventriculitis on imaging involving the entire ventricular system.

Ethical approval and patient's consent

Obtained.

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

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.  Back to cited text no. 1
    
2.
Kumar H, Sharma A, Sharma V, Singhvi S. Primary central nervous system lymphoma involving entire ventricular system. Asian J Neurosurg 2020;15:126-7.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Cecchi PC, Billio A, Colombetti V, Rizzo P, Ricci UM, Schwarz A. Primary high-grade B-cell lymphoma of the choroid plexus. Clin Neurol Neurosurg 2008;110:75-9.  Back to cited text no. 3
    
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Park SW, Yoon SH, Cho KG. An endoscopically proven ventriculitis-type, cyst-like intraventricular primary lymphoma of the central nervous system. Acta Neurochir (Wien) 2006;148:981-4.  Back to cited text no. 4
    
5.
Bühring U, Herrlinger U, Krings T, Thiex R, Weller M, Küker W. MRI features of primary central nervous system lymphomas at presentation. Neurology 2001;57:393-6.  Back to cited text no. 5
    
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Tersaki M, Abe T, Tajima Y, Fukushima S, Hirohata M, Shegemori M. Primary choroid plexus T Cell lymphoma and multiple aneurysms in the CNS. Leuk Lymphoma 2006;47:1680-2.  Back to cited text no. 6
    
7.
Hill CS, Khan AF, Bloom S, McCartney S, Choi D. A rare case of vomiting: Fourth ventricular B-cell lymphoma. J Neurooncol 2009;93:261-2.  Back to cited text no. 7
    
8.
Werneck LC, Hatschbach Z, Mora AH, Novak EM. Meningitis caused by primary lymphoma of the central nervous system. Report of a case. Arq Neuropsiquiatr 1977;35:366-72.  Back to cited text no. 8
    
9.
Kelley TW, Prayson RA, Barnett GH, Stevens GH, Cook JR, Hsi ED. Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue arising in the lateral ventricle. Leuk Lymphoma 2005;46:1423-7.  Back to cited text no. 9
    
10.
Jung TY, Jung S, Lee MC, Lee KH. Extranodal marginal zone B-cell lymphoma mimicking meningioma in lateral ventricle: A case report and possible pathogenesis. J Neurooncol 2006;80:63–7.  Back to cited text no. 10
    


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