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| CASE REPORT |
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| Year : 2019 | Volume
: 2
| Issue : 2 | Page : 131-134 |
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Primary central nervous lymphoma of cerebellopontine angle germinal center type: Report of a rare case
Venkateswarao Kommu1, Rachanakehra2, Sudha S Murthy2, Krishnamohan Mallavarapu3, Tasneem Rushdi4, Rashmi Sudhir5
1 Department of Neurosurgery and Neuro-Ocology, Basavatarakam Indoamerican Cancer Hospital and Research Institute, Hyderabad, Telangana, India
2 Department of Lab Medicine, Basavatarakam Indoamerican Cancer Hospital and Research Institute, Hyderabad, Telangana, India
3 Department of Medicaloncology, Basavatarakam Indoamerican Cancer Hospital and Research Institute, Hyderabad, Telangana, India
4 Department of Radiation Oncology, Basavatarakam Indoamerican Cancer Hospital and Research Institute, Hyderabad, Telangana, India
5 Department of Radiodiagnosis, Basavatarakam Indoamerican Cancer Hospital and Research Institute, Hyderabad, Telangana, India
| Date of Submission | 17-May-2019 |
| Date of Acceptance | 03-Nov-2019 |
| Date of Web Publication | 10-Jan-2020 |
Correspondence Address:
Dr. Venkateswarao Kommu
Department of Neurosurgery and Neuro.oncology, Basavatarakam Indoamerican Cancer Hospital and Research Institute, Hyderabad, Telangana
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/IJNO.IJNO_7_19
Lymphoma of the central nervous system is uncommon, accounting for approximately 0.2-2% of all brain tumors. Primary lymphoma of the cerebellopontine angle (CPA) is rare in the central nervous system. Computed tomography (CT) and magnetic resonance imaging (MRI) reliably describe the typical features of vestibular schwannomas and meningiomas. In various types of cerebellopontine angle mass lesions lymphoma should be considered one of the differential diagnosis. To our knowledge, there have only been 20 cases reported worldwide so far.
Keywords: Braintumor, cerebellopontine angle, malignant lymphoma
How to cite this article:
Kommu V, Rachanakehra, Murthy SS, Mallavarapu K, Rushdi T, Sudhir R. Primary central nervous lymphoma of cerebellopontine angle germinal center type: Report of a rare case. Int J Neurooncol 2019;2:131-4 |
| Introduction | |  |
Primary central nervous system (CNS) lymphomas (PCNSLs) are relatively rare, accounting for approximately 2.4%–3% of all intracranial tumors. They usually present in supratentorial locations with a predilection for deep white mater such as corpus callosum, basal ganglia, thalami, and paraventricular region.[1] PCNSL presenting as a cerebellopontine angle (CPA) mass is rare and can be mistaken for other diseases such as vestibular schwannomas or meningiomas, which constitute vast majority of mass lesions in the CPA; only 20 cases have been reported so far in the literature, to the best of our knowledge. We report of a case of CPA lymphoma with detailed work-up with emphasis on need to reach an accurate diagnosis for proper management of these cases.
| Case Report | | |
A 24-year-old male patient presented with gradually progressive headache, associated with multiple episodes of vomiting. On examination, the patient was found to be having left cerebellar signs. All routine investigations were within normal limits. Computed Tomography (CT) and Magnetic resonance imaging (MRI) brain showed left CPA mass lesion [Figure 1] and [Figure 2]a [Figure 2]b [Figure 2]c [Figure 2]d [Figure 2]e. Radiological impression was schwannoma. The patient was taken for surgery; intraoperatively, a well-defined mass lesion involving the middle cerebellar peduncle was seen. Tumor was soft and suckable and pale gray. Tumor tissue was sent for frozen section study which suggested possibility of lymphoma; hence, surgical decompression was stopped. Histopathological examination revealed fragmented bits of glial tissue harboring a cellular lesion arranged in diffuse sheets, comprising large cells having vesicular nuclei and moderate cytoplasm. Dilated blood vessels were seen. On immunohistochemistry, tumor cells were positive for LCA, CD20, CD10, and BCL6, and Ki67 was 85% [Figure 3]. A final diagnosis of diffuse large B-cell lymphoma (DLBCL)-germinal center type was rendered. The patient has a smooth postoperative course without neurological deficit, with marked improvement in his headache and gait disturbances. Postoperatively, the patient received local radiotherapy whole brain radiotherapy (WBRT) followed by chemotherapy with six cycles of cyclophosphamide, doxorubicin, vincristine, and prednisolone. Follow-up MRI [Figure 2]f after 1 year showed complete resolution of the mass with only posttreatment gliotic changes. | Figure 1: Noncontrast computed tomography brain axial image showed a well-defined lobulated intra-axial hyperdense mass at left cerebellopontine angle involving in the left middle cerebellar peduncle with perilesional moderate edema
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 | Figure 2: (a-e) Preoperative magnetic resonance imaging brain (T1-weighted, T2-weighted axial, and postcontrast images in axial, sagittal, and coronal planes) showed well-defined lobulated mass in the left middle cerebellar peduncle and cerebellar vermis, isointense to gray matter on T1-weighted and T2-weighted images, and heterogeneous enhancement on postcontrast imaging with perilesional moderate edema and mass effect on the fourth ventricle. Follow-up magnetic resonance imaging (f) after 1 year, coronal section showed fluid collection in the left cerebellum with perifocal mild vasogenic edema. No viable residual tumor
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 | Figure 3:(a) Perivascular arrangement of discrete large cells with vesicular chromatin and prominent nucleoli (H and E, ×400). (b) Tumor cells showing diffuse membranous expression of CD20 (×400). (c) CD3 expression seen only in background T-lymphocytes; large tumor cells are negative (×400). (d) CD10 expression seen in more than 30% of tumor cells (×400). (e) Immunohistochemical for BCL 6 showing weak nuclear expression by tumor cells (×400), (f) Ki67 was 85% (×400)
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| Discussion | | |
Lymphomas of CNS are relatively rare and can be classified as primary and secondary depending on the involvement of extracranial sites. PCNSL accounts for 2.4%–3% of all intracranial tumors and 4%–6% of all extranodal lymphomas.[1] PCNSL may occur in both immunocompromised patients at an average age of 30–33 years and immunocompetent persons at age of 50–55 years. Our patient was immunocompetent and presented at a young age. DLBCL is the most common subtype of PCNSL.[2]
PCNSL usually presents deep within the cerebral hemispheres; often occurs in thalami, periventricular region, corpus callosum, and basal ganglia; and is occasionally found in the cerebellum, and rarely occurs in the CPA.
CPA tumors account for 6%–10% of all intracranial tumors in adults.[3],[4] Most common tumors of the CPA are schwannoma (80%–90%).[5] Of the nonacoustic tumors, the majority were meningiomas (5%–10%), epidermoid inclusion cyst (5%–7%), primary cholesteatomas, and glomus jugulare tumors. A variety of unique lesions are reported at this location however together constitute <10% of all CPA tumors. Solitary CPA lymphoma is very rare; however, it must be kept in differential diagnosis as accurate diagnosis is imperative for proper management of the patient.
Only 20 cases of CPA lymphoma described so far;[6] the present case represents the 21st case in the literature. Most CNS lymphomas are characterized as homogeneously isointense/hypointense to the cortex on T1-weighted images, homogeneously isointense/hypointense with mild perifocal edema on T2-weighted images, and strongly homogeneously enhanced with contrast; in rare instances, CPA lymphoma can erode the internal auditory canal. In our case, radiological features are not mimicking the vestibular schwannoma.
Immunohistochemically, tumor cells express B-cell markers, CD20, PAX5. BCL 6 is expressed in 60% cases and CD10 is expressed in less than 10% of PCNSL. Since expression of CD10 is more commonly reported in systemic DLBCL, it is advisable to carry out complete workup to rule out CNS involvement by systemic DLBCL.[1] Our case expressed CD10 in more than 30% of tumor cells and was classified as DLBCL-germinal center type according to the Hans algorithm.[7]
Systemic workup done did not reveal any other site of lymphadenopathy, and bone marrow examination was also negative for the involvement by DLBCL.
PCNSL is believed to have worse outcomes as compared to systemic DLBCL. Currently, the use of high-dose methotrexate used either singly or in combination with other agents with or without radiotherapy forms the mainstay of treatment; however, currently, there is no uniform treatment consensus on optimal management of PCNSL.[8] Chemotherapy with judicious use of radiotherapy has been associated with longer survival rates for patients with PCNSL.[9] Our patient received postoperative chemoradiotherapy and doing fine at 1-year follow-up.
| Conclusion | | |
We report a rare case of DLBCL of CPA. It is difficult to diagnose presurgically and usually misdiagnosed as acoustic neuroma or meningioma. With this case report, our aim is to present a rare case report and emphasize the importance of keeping this differential diagnosis in mind for proper management of such cases, and we can avoid the radical resection of CPA tumor to avoid postoperative morbidity.
Declaration of patient consent
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 | | |
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| 7. | Hans CP, Weisenburger DD, Greiner TC, Gascoyne RD, Delabie J, Ott G, et al. Confirmation of the molecular classification of diffuse large B-cell lymphoma by immunohistochemistry using a tissue microarray. Blood 2004;103:275-82. |
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[Figure 1], [Figure 2], [Figure 3]
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