<%server.execute "isdev.asp"%> Primary intraventricular central nervous system lymphoma in an immunocompetent patient Suri V, Mittapalli V, Kulshrestha M, Premlani K, Sogani S K, Suri K - J Pediatr Neurosci
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CASE REPORT
Year : 2015  |  Volume : 10  |  Issue : 4  |  Page : 393-395
 

Primary intraventricular central nervous system lymphoma in an immunocompetent patient


Department of Neurology Sciences, Indraprastha Apollo Hospital, New Delhi, India

Date of Web Publication20-Jan-2016

Correspondence Address:
Vinit Suri
Department of Neurology Sciences, Indraprastha Apollo Hospital, Sarita Vihar, New Delhi - 110 076
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1817-1745.174433

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   Abstract 

We report a young 15-year-old boy with 6 months history of headache, vomiting, and seizure. He underwent septostomy followed by right ventriculoperitoneal shunt for obstructive hydrocephalus and was managed with empirical antituberculosis treatment. Magnetic resonance imaging (MRI) revealed solid, nodular, enhancing masses in bilateral lateral ventricles and 4th ventricle. Surgical biopsy from 4th ventricular lesion confirmed a B-cell lymphoma. Staging evaluation with MRI positron emission tomography and bone marrow biopsy were normal suggesting an intraventricular primary central nervous system lymphoma.


Keywords: Intraventricular tumor, immunocompetent, primary central nervous system lymphoma


How to cite this article:
Suri V, Mittapalli V, Kulshrestha M, Premlani K, Sogani S K, Suri K. Primary intraventricular central nervous system lymphoma in an immunocompetent patient. J Pediatr Neurosci 2015;10:393-5

How to cite this URL:
Suri V, Mittapalli V, Kulshrestha M, Premlani K, Sogani S K, Suri K. Primary intraventricular central nervous system lymphoma in an immunocompetent patient. J Pediatr Neurosci [serial online] 2015 [cited 2019 Jul 20];10:393-5. Available from: http://www.pediatricneurosciences.com/text.asp?2015/10/4/393/174433



   Introduction Top


Lymphoma of the central nervous system (CNS) can present with a wide variety of clinical symptoms and imaging findings, which can be challenging for both the clinician as well as the radiologist. Primary CNS lymphoma (PCNSL) is a rare variant of extranodal non-Hodgkin's lymphoma and accounts for 1% of all primary brain tumors. Lesions of PCNSL are almost always found within the brain parenchyma and may be superficial (subpial) or deep-seated (sub ependymal). Intraventricular PCNSL is an extremely rare presentation of PCNSL with = 15 patients described in literature.

We present a patient with PCNSL with lesions in bilateral lateral ventricle and in the 4th ventricle.


   Case Report Top


A young 15-year-old boy had a 6 months history of continuous headache, recurrent vomiting, and a single episode of generalized tonic-clonic seizure. Magnetic resonance imaging (MRI) brain initially within 1 month of onset revealed asymmetrical dilation of bilateral lateral ventricles (L > R) for which endoscopic septostomy was performed at another hospital. The patient showed initial improvement for 2–3 weeks followed by recurrence of symptoms of headache and vomiting. Cerebrospinal fluid examination revealed mild pleocytosis with mildly elevated protein, normal sugar, negative acid-fast Bacilli, fungal cultures, and serology. Right ventriculoperitoneal shunt was performed and empirical antitubercular therapy was initiated at this hospital. The patient remained symptomatic with repeated bouts of headache and recurrent vomiting, despite repeated adjustments of shunt pressures by repeated reprogramming of shunt. Repeat MRI brain [Figure 1] revealed focal, nodular, well defined solid enhancing lesions in the right lateral ventricle and 4th ventricle. The patient had neurological decline with decline in sensorium, severe truncal ataxia, dual incontinence, and right gaze palsy with right 7th infra nuclear and 9th and 10th nerve palsy. Posterior fossa craniotomy and decompression of tumor in the 4th ventricle was performed and biopsy revealed round to oval and monomorphic cells with scanty rim of eosinophilic cytoplasm with large and hyperchromatic nucleoli with irregular nuclear membrane. Few mitotic figures were present, and tumor cells are positive for leukocyte common antigen and CD20 and were negative for CD3, synaptophysin, and glial fibrillary acidic protein. The mib1 labeling index was very high (80%), suggestive of non-Hodgkin's lymphoma (diffuse large B-cell type) [Figure 2]. In view of biopsy, further staging was performed with positron emission tomography MRI whole body which revealed metabolically active lesions in 4th ventricle and discrete lesions in bilateral lateral ventricles with no other systemic fluorodeoxyglucose uptake, suggestive of PCNSL. Bone marrow biopsy was normal.
Figure 1: Original magnetic resonance imaging images. (a and b) Flair images showing right lateral ventricular and 4th ventricular hyperintense nodular lesions. (c-e) Contrast enhancing nodular lesions in right lateral ventricle and 4th ventricle

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Figure 2: Original histopathology of 4th intraventricular tumor: (a) H and E staining revealed diffuse infiltration by sheets of atypical lymphoid cells. (b) The individual atypical lymphoid cells are moderately pleomorphic with round to oval hyperchromatic nuclei, inconspicuous nucleoli, and moderate amount of eosinophilic cytoplasm. Frequent mitosis is present. (c) Immunohistochemistry done for CD20 showed strong membranous positivity. (d) KI67/MIBI labeling index was very high (almost >80% positivity)

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   Discussion Top


CNS involvement in lymphoma can be secondary to a systemic lymphoma, which is the most common presentation. Alternatively, there can be isolated involvement of the CNS without any systemic lymphoma, which is the PCNSL variant. Secondary CNS lymphoma is almost always due to non-Hodgkin's lymphoma with spread to CNS which usually occurs after a median of 5–12 months after the primary diagnosis. Two-thirds of such secondary lymphoma infiltration occurs into the leptomeninges, subependymal, dural, or even cranial nerves and one-third present with CNS parenchymal infiltration.[1] PCNSL is a rare variant of extranodal non-Hodgkin's lymphoma and accounts for 1% of all primary brain tumors and is commonly a diffuse large B-cell type. PCNSL is usually associated with an immunodeficiency state, but may also be observed in immunocompetent individuals. As opposed to secondary CNS lymphoma, lesions of PCNSL are almost always found within the brain parenchyma and may be superficial (subpial) or deep-seated (sub ependymal).

PCNSL commonly presents within the brain parenchyma in superficial (subpial) or deep-seated (subependymal location). Intraventricular PCNSL is an extremely rare presentation with only few case reports.[2],[3],[4],[5],[6],[7],[8] Most of these case reports have described PCNSL occurrence in a single cerebral ventricle except the involvement of right lateral ventricle and 4th ventricle in one report,[9] which is also the presentation of our patient.

The imaging features of PCNSL are not characteristic and usually reveals hypo- or iso-intense lesions on T1-weighted MRI, iso- or hyper-intense lesions on T2-weighted images, and lesions show moderate to marked contrast enhancement with variable surrounding edema and hence, diagnosis is established only by a biopsy.[10]

Differential diagnosis for intraventricular CNS lesions includes central neurocytomas, meningiomas, ependymomas, choroid plexus papilloma, metastasis, neurocysticercosis, and rarely PCNSL. The role of imaging is 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. The standard treatment for PCNSL is biopsy followed by systemic chemotherapy or with intrathecal chemotherapy.[11] High-dose chemotherapy with autologous stem cell transplant may be utilized in patients with chemoresistance.[12]

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Haldorsen IS, Espeland A, Larsson EM. Central nervous system lymphoma: Characteristic findings on traditional and advanced imaging. AJNR Am J Neuroradiol 2011;32:984-92.  Back to cited text no. 1
    
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. 2
    
3.
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. 3
    
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
    
5.
Hassan HA, Ramli NM, Rahmat K. Primary intraventricular lymphoma with diffuse leptomeningeal spread at presentation. Ann Acad Med Singapore 2012;41:268-70.  Back to cited text no. 5
    
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. 6
    
7.
Lettau M, Laible M. Primary intraventricular non-Hodgkin's lymphoma of the CNS. Rofo 2012;184:261-3.  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.
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. 9
    
10.
Yap KK, Sutherland T, Liew E, Tartaglia CJ, Pang M, Trost N. Magnetic resonance features of primary central nervous system lymphoma in the immunocompetent patient: A pictorial essay. J Med Imaging Radiat Oncol 2012;6:179-86.  Back to cited text no. 10
    
11.
Deckert M, Engert A, Brück W, Ferreri AJ, Finke J, Illerhaus G, et al. Modern concepts in the biology, diagnosis, differential diagnosis and treatment of primary central nervous system lymphoma. Leukemia 2011;25:1797-807.  Back to cited text no. 11
    
12.
Omuro A, Correa DD, DeAngelis LM, Moskowitz CH, Matasar MJ, Kaley TJ, et al. R-MPV followed by high-dose chemotherapy with TBC and autologous stem-cell transplant for newly diagnosed primary CNS lymphoma. Blood 2015;125:1403-10.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2]



 

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