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LETTER TO EDITOR
Year : 2013  |  Volume : 61  |  Issue : 4  |  Page : 450-453

Primary B-cell central nervous system lymphoma involving fourth ventricle: A rare case report with review of literature


1 Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
2 Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Date of Submission22-Jul-2013
Date of Decision26-Jul-2013
Date of Acceptance14-Aug-2013
Date of Web Publication4-Sep-2013

Correspondence Address:
Ram Nawal Rao
Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.117608

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How to cite this article:
Rao RN, Mishra D, Agrawal P, Kumar R. Primary B-cell central nervous system lymphoma involving fourth ventricle: A rare case report with review of literature. Neurol India 2013;61:450-3

How to cite this URL:
Rao RN, Mishra D, Agrawal P, Kumar R. Primary B-cell central nervous system lymphoma involving fourth ventricle: A rare case report with review of literature. Neurol India [serial online] 2013 [cited 2021 Jan 26];61:450-3. Available from: https://www.neurologyindia.com/text.asp?2013/61/4/450/117608


Sir,

Primary B-cell central nervous system lymphoma (PCNSL) is an uncommon aggressive lymphoma accounting for 1% of all intracranial tumors [1],[2] and is usually associated with human immunodeficiency virus (HIV) patients. However, a central nervous system (CNS) lymphoma involving fourth ventricle is extremely rare.

A 59-year-old male patient presented with a vomiting, vertigo, tremors of both upper limbs and head and difficulty in walking with the tendency to fall toward the left side since 8 months. There was worsening of the symptoms for 3 weeks before admission. Neurological examination showed swaying to the left on tandem walking. Serology for HIV and other infections was negative. Magnetic resonance imaging (MRI) revealed a well-defined extra axial mass lesion with the well-defined plane and intervening cerebrospinal fluid cleft at fourth ventricle, isointense on T1-weighted [Figure 1], hypointense on T2-weighted [Figure 2] sequences with contrast enhancement and perilesional edema. The pre-operative diagnosis was either a hemangioblastoma or low grade glioma. Mid line suboccipital craniectomy and decompression was done. Lesion was soft suckable and vascular and near total excision of the lesion was done. Histopathological examination revealed a tumor composed of medium sized atypical lymphoid cells have round nucleus, coarse chromatin, inconspicuous nucleoli and scant amount of cytoplasm along with adjacent normal brain parenchyma [Figure 3] and [Figure 4]. Immunohistochemistry showed tumor cells were positive for leukocyte common antigen [Figure 5], CD20 [Figure 6] and negative for CD3, synaptophysin, neuron-specific enolase, desmin, CD99 and cytokeratin. Ki67 index was high (60-70%) [Figure 7]. Post-operative MRI showed an operative cavity filled with fluid with near total excision of the tumor. At the time of discharge patient was conscious, oriented, afebrile and self-ambulation with healthy surgical site.
Figure 1: Magnetic resonance imaging showed a well‑defined extraaxial mass lesion with the well‑defined plane and intervening cerebrospinal fluid cleft at fourth ventricle

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Figure 2: Magnetic resonance imaging showed contrast enhancing mass with isointense on T1, hypointense on T2 with perilesional edema

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Figure 3: Histopathogical section showed sheet of atypical lymphoid cells adjacent to brain parenchyma (H and E, ×200)

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Figure 4: Section showed sheet of atypical lymphoid cells have small to medium size, condensed chromatin, inconspicuous nucleoli and scant cytoplasm (H and E, ×400)

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Figure 5: Atypical lymphoid cells are strongly positive for leukocyte common antigen (LCA, ×400)

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Figure 6: Atypical lymphoid cells are strongly positive for CD20 (CD20, ×400)

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Figure 7: Tumor cells showed high Ki67 (60‑70%) proliferating cells (Ki67, ×400)

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Literature review revealed only 12 published cases of intraventricular PCNSL, five in the fourth ventricle, [3],[4],[5],[6],[7] four in the third ventricle and three in the lateral ventricles including one in both lateral and fourth ventricle [7] [Table 1]. This patient is the sixth case with fourth ventricle involvement. Pre-operative diagnostic possibility of this entity may help the surgeon for planning the extent of resection.
Table 1: Description of previous reported primary fourth ventricular B‑cell CNS lymphomas (n=6)

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The pathogenesis of this tumor is uncertain and two possible pathogenetic mechanisms have been suggested. [8] According to one hypothesis an infectious or inflammatory process evokes reactive lymphocyte population in the CNS which then transforms into neoplastic cells. The second hypothesis suggests B lymphocytes in an undetected lymph node or extranodal site get activated, proliferate and transform to become neoplastic. These cells may involve the CNS by hematogenous spread. Since lymphoma is a diffuse infiltrative tumor, [4] surgical resection is not an effective treatment and the disease may be controlled by chemotherapy and radiotherapy. All the previous reported cases and our patient received chemotherapy [Table 1]. Chemotherapy with methotrexate (MTX) based regimens has improved survival in PCNSL patients and other approaches included a variety of drugs and varying doses and timing of whole brain radiotherapy (WBRT). [1] Current treatment protocols focus on maximizing survival while minimizing WBRT and MTX-related toxicity. Newer approaches such as immunotherapy with monoclonal antibodies and autologous stem cell rescue after myeloablative chemotherapy are being evaluated. The treatment response rate is 72.7% in immunocompetent patients treated with MTX, procarbazine and vincristine (MPV), with or without WBRT. [9]

 
 » References Top

1.Shah GD, DeAngelis LM. Treatment of primary central nervous system lymphoma. Hematol Oncol Clin North Am 2005;19:611-27.  Back to cited text no. 1
[PUBMED]    
2.Batchelor T, Loeffler JS. Primary CNS lymphoma. J Clin Oncol 2006;24:1281-8.  Back to cited text no. 2
[PUBMED]    
3.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. 3
[PUBMED]    
4.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. 4
[PUBMED]    
5.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. 5
[PUBMED]    
6.Brar R, Prasad A, Sharma 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. 6
[PUBMED]    
7.Bokhari R, Ghanem A, Alahwal M, Baeesa S. Primary isolated lymphoma of the fourth ventricle in an immunocompetent patient. Case Rep Oncol Med 2013;2013:614658.  Back to cited text no. 7
[PUBMED]    
8.Hochberg FH, Miller DC. Primary central nervous system lymphoma. J Neurosurg 1988;68:835-53.  Back to cited text no. 8
[PUBMED]    
9.Agarwal PA, Menon S, Smruti BK, Singhal BS. Primary central nervous system lymphoma: A profile of 26 cases from Western India. Neurol India 2009;57:756-63.  Back to cited text no. 9
[PUBMED]  Medknow Journal  


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]
 
 
    Tables

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