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Table of Contents
CASE REPORTS
Year : 2021  |  Volume : 4  |  Issue : 1  |  Page : 25-27

Primary intraventricular central nervous system lymphoma of lateral ventricle in an immunocompetent patient


Department of Pathology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India

Date of Submission10-Apr-2021
Date of Acceptance16-Feb-2022
Date of Web Publication12-Apr-2022

Correspondence Address:
Dr. Kavita Mardi
Department of Pathology, Indira Gandhi Medical College, Set No 14, Type VI Quarters, IAS Colony, Meheli, Shimla, Himachal Pradesh.
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJNO.IJNO_6_21

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  Abstract 

Primary CNS lymphoma (PCNSL) is a rare variant of extranodal non-Hodgkin’s lymphoma and accounts for 3%–5% of all primary brain tumors. Their intraventricular location is extremely rare with only a few cases on record so far. All the reported cases showed bilateral ventricular involvement. We report the first case of unilateral lateral ventricle PCNL in a 22-year-old female who presented with intractable vomiting and headaches. Computed tomography (CT) followed by magnetic resonance imaging (MRI) revealed lobulated altered signal intensity lesion in the left temporal horn of lateral ventricle with surrounding peritumoral edema causing midline shift. With these imaging features, the diagnosis of intraventricular meningioma was suggested. Microscopic examination of the excised mass revealed round-to-ovoid tumor cells with scant eosinophilic cytoplasm with large and hyperchromatic nuclei, prominent nucleoli, irregular nuclear membrane, and frequent mitotic figures. The tumor cells are positive for leukocyte common antigen, CD20, CD10, CD43; and were negative for CD3, synaptophysin, chromagranin, pan-cytokeratin (pan-CK), Epstein-Barr virus (EBV), and glial fibrillary acidic protein. The Ki-67 was very high (80%). With these histopathological and immunohistochemistry (IHC) findings, diagnosis of NHL of diffuse large B cell type was rendered.

Keywords: CNS, intraventricular, lateral ventricle, lymphoma, NHL


How to cite this article:
Mardi K, Negi L, Lanzhe T, Murgai P. Primary intraventricular central nervous system lymphoma of lateral ventricle in an immunocompetent patient. Int J Neurooncol 2021;4:25-7

How to cite this URL:
Mardi K, Negi L, Lanzhe T, Murgai P. Primary intraventricular central nervous system lymphoma of lateral ventricle in an immunocompetent patient. Int J Neurooncol [serial online] 2021 [cited 2022 Jun 27];4:25-7. Available from: https://www.Internationaljneurooncology.com/text.asp?2021/4/1/25/342822




  Introduction Top


Primary CNS lymphoma (PCNSL) is a rare variant of extranodal non-Hodgkin’s lymphoma and accounts for 3%–5% of all primary brain tumors. These tumors are almost always found within the brain parenchyma and may be superficial (subpial) or deep-seated (subependymal). Intraventricular PCNSLs are extremely rare with less than 20 cases reported in literature to date.[1] All the four reported cases in the lateral ventricle show bilateral involvement.[2],[3],[4] We describe one such rare occurrence of PCNSL in the lateral ventricle of a 22-year-old woman. This is the first reported case involving a unilateral lateral ventricle.


  Case report Top


A 22-year-old woman presented with intractable vomiting and headaches. She had no previous medical history. The findings of the neurological examination were normal. There was no motor or sensory deficit and cranial nerves were normal. The rest of the clinical examination revealed normal findings. Her initial blood work was negative. Computed tomography (CT) followed by magnetic resonance imaging (MRI) revealed lobulated altered signal intensity lesion in the left temporal horn of lateral ventricle with surrounding peritumoral edema causing midline shift [Figure 1]. With these imaging features, the diagnosis of intraventricular meningioma was suggested. Left pterional craniotomy with excision of large fleshy extra-axial of size 5 cm × 4 cm from left lateral ventricle was performed. Microscopic examination revealed round-to-ovoid tumor cells with scant eosinophilic cytoplasm with large and hyperchromatic nuclei with prominent nucleoli and irregular nuclear membrane [Figure 2]. Frequent mitotic figures were present, and tumor cells are positive for leukocyte common antigen, CD20 [Figure 3], CD10, CD43; and were negative for CD3, synaptophysin, chromagranin, pan-cytokeratin (pan-CK), Epstein-Barr virus (EBV), and glial fibrillary acidic protein. The Ki-67 was very high (80%). These histological and immunohistochemistry (IHC) findings were suggestive of non-Hodgkin’s lymphoma (diffuse large B-cell type). Postoperatively, the patient recovered without any deficit. She was commenced on chemotherapy with intrathecal/intravenous methotrexate and remained well 2 months after the diagnosis.
Figure 1: MRI revealing lobulated lesion in the left temporal horn of lateral ventricle with surrounding peritumoral edema

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Figure 2: Round to ovoid tumor cells with scant eosinophilic cytoplasm, large, and hyperchromatic nuclei with prominent nucleoli and irregular nuclear membrane (H&E, 40×)

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Figure 3: Tumor cells showing positivity for CD20 (IHC, 40×)

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  Discussion Top


Primary central nervous system lymphoma (PCNSL) is a rare type of extra-nodal non-Hodgkin lymphoma (NHL) that can be found in the brain, leptomeninges, and spinal cord without evidence of systemic lymphoma. PCNSL constitutes 1% of all NHLs and 3%–5% of all primary brain tumors. Intraventricular PCNSLs are extremely rare with only few case reports on record.[5],[6],[7],[8],[9],[10],[11] Most of these case reports have described PCNSL located in a single cerebral ventricle except the involvement of the right lateral ventricle and fourth ventricle in one of the reports.[12]

As opposed to secondary CNS lymphoma, the lesions of PCNSL are nearly always found within the brain parenchyma. Pure intraventricular involvement by PCNSL is very rare. In the majority of immunocompetent patients, the lesions are solitary and most are supratentorial, with the posterior fossa being a somewhat rare location. Multiple lesions have been reported in 20%–40% of nonautoimmune deficiency syndrome (AIDS) PCNSL.[3] The association between AIDS-related PCNSL and EBV expression in the tumor is approximately 100%. By contrast, EBV is rarely detected in PCNSL of immunocompetent patients, suggesting a different pathogenesis in each group. Our patient is relatively younger, immunocompetent, had a large mass in left lateral ventricle, and did not harbor EBV in the tumor. Approximately 90% of cases of intraventricular lymphomas are aggressive, diffuse large B-cell lymphoma (DLBCL) with a poor outcome.[1] This is followed by T-cell lymphomas and mucosa-associated lymphoid tissue (MALT) lymphoma. Other types of lymphoma, such as low-grade subtypes, are extremely rare.

The presence of homogeneously enhanced intraventricular MRI lesions should raise the suspicion of PCNSLs. Although advanced imaging techniques can increase the diagnostic accuracy and help in differentiating PCNSL from other tumorous or nontumorous lesions, brain biopsy is required to confirm the diagnosis. Differential diagnosis for intraventricular CNS lesions includes central neurocytomas, meningiomas, ependymomas, choroid plexus papilloma, metastasis, neurocysticercosis, and rarely PCNSL.

The most important role of imaging in PCNSL is directing clinicians to perform a stereotactic biopsy or obtain cerebrospinal fluid (CSF) in order to obtain a histologic diagnosis and avoid futile attempts at resection.

Resection does not play a therapeutic role in PCNSL and can worsen neurologic deficits.[13] Overall, prognosis has improved over the past several decades and PCNSL is a very chemosensitive and radiosensitive tumor. Most oncologists agree on a methotrexate chemotherapy regimen in addition to whole-brain radiation, although there is an increased risk of neurotoxicity with radiation.[13]

In conclusion, the role of imaging in aiding to distinguish from other tumors seen in intraventricular location and the diagnosis of PCNSL is essentially based on biopsy and histopathological examination. It is important that primary B-cell lymphoma should be included in the list of differential diagnosis of intraventricular tumors.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Suri V, Mittapalli V, Kulshrestha M, Premlani K, Sogani SK, Suri K Primary intraventricular central nervous system lymphoma in an immunocompetent patient. J Pediatr Neurosci 2015;10:393-5.  Back to cited text no. 1
    
2.
Brara R, Prasada A, Sharmab T, Vermani N Multifocal lateral and fourth ventricular B-cell primary CNS lymphoma. Clin Neurol Neurosurg 2012;114:281-3.  Back to cited text no. 2
    
3.
Funaro K, Bailey KC, Aguila S, Agosti SJ, Vaillancourt C A case of intraventricular primary central nervous system lymphoma. J Radiol Case Rep 2014;8:1-7.  Back to cited text no. 3
    
4.
Guo R, Zhang X, Niu C, Xi Y, Yin H, Lin H, et al. Primary central nervous system small lymphocytic lymphoma in the bilateral ventricles: Two case reports. BMC Neurol 2019;19:200.  Back to cited text no. 4
    
5.
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. 5
    
6.
Cheatle JT, Aizenberg MR, Weinberg JS, Surdell DL Atypical presentation of primary central nervous system non-Hodgkin lymphoma in immunocompetent young adults. World Neurosurg 2013;79:593.e9-13.  Back to cited text no. 6
    
7.
Haegelen C, Riffaud L, Bernard M, Morandi X Primary isolated lymphoma of the fourth ventricle: Case report. J Neurooncol 2001;51:129-31.  Back to cited text no. 7
    
8.
Hassan HA, Ramli NM, Rahmat K Primary intraventricular lymphoma with diffuse leptomeningeal spread at presentation. Ann Acad Med Singap 2012;41:268-70.  Back to cited text no. 8
    
9.
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. 9
    
10.
Lettau M, Laible M [Primary intraventricular non-Hodgkin’s lymphoma of the CNS]. Rofo 2012;184:261-3.  Back to cited text no. 10
    
11.
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. 11
    
12.
Zhu Y, Ye K, Zhan R, Tong Y Multifocal lateral and fourth ventricular primary central nervous system lymphoma: Case report and literature review. Turk Neurosurg 2015;25:493-5.  Back to cited text no. 12
    
13.
Brastianos PK, Batchelor TT Primary central nervous system lymphoma: Overview of current treatment strategies. Hematol Oncol Clin North Am 2012;26:897-916.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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