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Year : 2020  |  Volume : 15  |  Issue : 1  |  Page : 126-127

Primary central nervous system lymphoma involving entire ventricular system

1 Department of Neurosurgery, S.M.S. Medical College, Jaipur, Rajasthan, India
2 Department of Pathology, Patho Care and Research Centre, S.M.S. Medical College, Jaipur, Rajasthan, India

Date of Submission08-Mar-2016
Date of Acceptance29-Mar-2017
Date of Web Publication25-Feb-2020

Correspondence Address:
Dr. Hemant Kumar
Department of Neurosurgery, S.M.S. Medical College, Jaipur, Rajasthan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ajns.AJNS_94_16

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Primary central nervous system lymphoma (PCNSL) is a rare tumor that accounts for <1%–4% of primary CNS tumor.[1] PCNSLs are class of non-Hodgkin's lymphomas which are primarily of diffuse large B-cell origin (90%), with remaining being T-cell lymphoma (10%). Author report a rare case of PCNSL presenting as an intracranial mass involving the entire ventricular system, in an immunocompetent 36-year-old male with severe headache, decreased vision, and unsteady gait. The diagnosis was obtained by histopathological and subsequent immunohistochemistry.

Keywords: Intraventricular lymphoma, primary central nervous system lymphoma

How to cite this article:
Kumar H, Sharma A, Sharma V, Singhvi S. Primary central nervous system lymphoma involving entire ventricular system. Asian J Neurosurg 2020;15:126-7

How to cite this URL:
Kumar H, Sharma A, Sharma V, Singhvi S. Primary central nervous system lymphoma involving entire ventricular system. Asian J Neurosurg [serial online] 2020 [cited 2020 Nov 27];15:126-7. Available from:

  Introduction Top

Before the emergence of HIV/AIDS, primary central nervous system lymphoma (PCNSL) was most often seen either in the setting of known immunocompromised status or with advanced age. After emergence of the AIDS epidemic was mirrored by a rise in the incidence of PCNSL, and it soon became an AIDS-defining illness. The majority of the PCNSL cases present as solitary masses in cerebral hemisphere, thalamus, basal ganglia, and periventricular region. Only a few cases of PCNSL involving ventricular system have been reported in the literature. Our case involved the whole ventricular system (bilateral lateral, third, and fourth ventricle). To the best of our knowledge, only one case in the literature has been reported before this in which PCNSL involved the whole ventricular system.[2]

  Case Report Top

A 36-year-old male patient admitted with chief complaints of headache, decreased vision, and difficulty in walking for 15 days. General physical examination of the patient was unremarkable. On neurological examination, the higher mental function was normal. On cranial nerve examination, the patient had bilateral papilledema, motor, and sensory examination was normal. The patient had the presence of cerebellar sign bilaterally. Lymphoreticular system examination was unremarkable. On further evaluation, routine blood investigation including HIV was done which was nonreactive. Computed tomography (CT) scan showed the presence of multiple lobulated intensely enhancing intraventricular space occupying lesion in lateral, third, and fourth ventricle [Figure 1]a. Magnetic resonance imaging (MRI) brain of the same patient showed that the presence of multiple solid lobulated enhancing masses of variable size is seen involving lateral, third, fourth ventricles, and infiltrating into the periventricular white matter in bilateral frontal and parietal lobe [Figure 1]b. Magnetic resonance spectroscopy showed lactate and choline peaks elevated, and N-acetylaspartate peak was reduced suggestive of mitotic pathology. Screening of the whole spine with MRI was done which did not show any pathology. CT scan abdomen and thorax were unremarkable. The patient was planned for tumor decompression from fourth ventricle keeping in view possibility of early brainstem compression as well as obtaining a tissue diagnosis. Midline suboccipital craniectomy with tumor decompression was done. Intraoperatively, the tumor was grayish white, suckable, and highly vascular. Histopathological examination of the submitted tissue was suggestive of round cell tumor. The biopsy submitted showed cerebellar tissue with diffusely infiltrating large neoplastic lymphocyte-like round cell with brisk mitotic activity. The cells are large; nuclei are round to oval with predominant nucleoli. Mitotic and apoptotic activity are seen frequently [Figure 2]a. On immunomarker, the cells were positive for Pax5, leukocyte common antigen (CD45), CD20, with Ki-67 (MIB index) being approximately 80%. The tumor cells were negative for synaptophysin, EMA, Alk1, NF, p53, TDT, CD3, CD 4, CD8, CD19, CD99, GFAP, and S100 [Figure 2]b. Immunomarkers were for non-Hodgkin's lymphoma type, diffuse large B-cell lymphoma.
Figure 1: (a) Computed tomography scan showing multilobulated homogenous contrast-enhancing mass in lateral ventricles and fourth ventricle. (b) Magnetic resonance imaging showing multilobulated homogenous enhancing mass in lateral, third, and fourth ventricles

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Figure 2: (a) Cerebellar tissue with diffusely infiltrating sheath of large neoplastic cells with necrosis (HPE, ×10). (b) Diffuse membrane staining of CD20 (HPE, ×60)

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  Discussion Top

CNS lymphomas are highly chemo- and radiation-sensitive tumors and management of these tumors is histopathological confirmation by either open or stereotactic biopsy followed by chemotherapy or radiotherapy or both. Primary intraventricular lymphomas are rare entity.[3],[4],[5] Only a few cases of intraventricular PCNSL have been reported in the literature, of which three cases were in the fourth ventricle,[3],[6],[7],[8] four cases were in the third ventricle,[3],[9],[10] and three cases were in the lateral ventricle,[11],[12] while only one case as per literature available was identified with simultaneous involvement of the lateral and fourth ventricles. CNS lymphoma is known to present in many different forms. As described, in our case report, PCNSL should be considered as one of the differentials diagnosis of diffuse intraventricular masses. So that, operative interventions can be avoided and biopsy either stereotactic or open should be obtained followed by chemotherapy and radiotherapy.

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There are no conflicts of interest.

  References Top

Rubenstein J, Ferreri AJ, Pittaluga S. Primary lymphoma of the central nervous system: Epidemiology, pathology and current approaches to diagnosis, prognosis and treatment. Leuk Lymphoma 2008;49 Suppl 1:43-51.  Back to cited text no. 1
Qin JZ, Wu YK, Yang ZJ, Lv J, Dang YY, Zhang HT, et al. Endoscopic biopsy of a B-cell lymphoma involving the entire ventricular system: A case report. Exp Ther Med 2016;11:325-7.  Back to cited text no. 2
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
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
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
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. 6
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
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
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
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
Terasaki M, Abe T, Tajima Y, Fukushima S, Hirohata M, Shigemori M. Primary choroid plexus T-cell lymphoma and multiple aneurysms in the CNS. Leuk Lymphoma 2006;47:1680-2.  Back to cited text no. 11
Bogdahn U, Bogdahn S, Mertens HG, Dommasch D, Wodarz R, Wünsch PH, et al. Primary non-Hodgkin's lymphomas of the CNS. Acta Neurol Scand 1986;73:602-14.  Back to cited text no. 12


  [Figure 1], [Figure 2]


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