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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 779-781

Case report: Sporadic primary Burkitt's lymphoma presenting with papilledema - A diagnostic challenge


1 Department of Ophthalmology and Visual Sciences, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, Kubang Kerian, Kelantan; Department of Ophthalmology, Hospital Raja Permaisuri Bainun, Ipoh, Perak, Malaysia
2 Department of Ophthalmology, Hospital Raja Permaisuri Bainun, Ipoh, Perak, Malaysia
3 Department of Neurology, Hospital Raja Permaisuri Bainun, Ipoh, Perak, Malaysia
4 Department of Ophthalmology and Visual Sciences, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, Kubang Kerian, Kelantan, Malaysia

Date of Submission30-Nov-2020
Date of Acceptance14-Mar-2021
Date of Web Publication09-Oct-2021

Correspondence Address:
Prof. W H Wan Hazabbah
Department of Ophthalmology and Visual Sciences, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, Kubang Kerian - 16150, Kelantan
Malaysia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_3572_20

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  Abstract 


Bilateral optic disc swelling with bilateral abducens nerve palsy as the first presenting signs of sporadic primary Burkitt's lymphoma (BL) is very rare. We report this in a young immunocompetent individual. Initial misdiagnosis of idiopathic intracranial hypertension was made due to normal brain imaging with elevated opening pressure on lumbar puncture. After 2 months, he developed neurological deficits and neck swellings. Biopsy of swelling revealed BL. He received chemotherapy but succumbed before the second cycle. Signs of raised intracranial pressure warrant meticulous investigation. Multiple high-volume taps of lumbar puncture are advised if clinical suspicion of neurolymphomatosis is high.

Keywords: Burkitt's lymphoma, idiopathic intracranial hypertension, neurolymphomatosis, optic disc swelling


How to cite this article:
Chin JJ, Chew CC, Chong MF, Cheah CF, Cheng YL, Ngoo QZ, Liza-Sharmini A T, Wan Hazabbah W H. Case report: Sporadic primary Burkitt's lymphoma presenting with papilledema - A diagnostic challenge. Indian J Ophthalmol Case Rep 2021;1:779-81

How to cite this URL:
Chin JJ, Chew CC, Chong MF, Cheah CF, Cheng YL, Ngoo QZ, Liza-Sharmini A T, Wan Hazabbah W H. Case report: Sporadic primary Burkitt's lymphoma presenting with papilledema - A diagnostic challenge. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 28];1:779-81. Available from: https://www.ijoreports.in/text.asp?2021/1/4/779/327676



Burkitt's lymphoma (BL) is a mature B-cell non-Hodgkin lymphoma (NHL), which is highly aggressive. It accounts for only 3%–5% of NHL in immunocompetent adults.[1] It can occur sporadically, endemically, or related to immunodeficiency.

We report a rare case of primary sporadic BL presenting with initial signs of raised intracranial pressure (ICP).


  Case Report Top


A 28-year-old previously healthy man presented with acute binocular horizontal diplopia for two weeks. He had headache but not vomiting. His vision was good in both eyes. He had history of right leg pain and numbness. There was no fever or trauma.

On examination, his visual acuity was 6/9 in both eyes. Extraocular movement examination showed bilateral lateral rectus palsy. There was no proptosis. Both anterior segments were unremarkable. Fundoscopy showed bilateral optic disc swelling with splinter hemorrhages and retinal vein tortuosity [Figure 1] and normal macula. Systemic examination showed no lymphadenopathy and no neurological deficits.
Figure 1: Fundus photo of the patient showing bilateral optic disc swelling with retinal hemorrhages and retinal vein tortuosity

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Computed tomography (CT) scan of the brain and orbit and CT Venogram were normal with no space-occupying lesion nor cerebral venous thrombosis. Lumbar puncture revealed an elevated opening pressure of 26 cm H20. Cerebrospinal fluid (CSF) analysis was normal (protein 407 mg/L, glucose 3.4 mmol/L, lactate 1.83 mmol/L, FEME clear, cell counts normal). Full blood count parameters were normal without cytopenia to suggest bone marrow involvement. Venereal Disease Research Laboratory and Human Immunodeficiency Virus serology were negative. Right leg pain was evaluated by orthopedics team. Deep vein thrombosis was ruled out as D-dimer test was normal. The provisional diagnosis was Idiopathic Intracranial Hypertension (IIH). Patient was started on oral acetazolamide 250 mg twice daily.

Magnetic resonance imaging (MRI) brain was planned. However, the patient defaulted. He presented only after 2 months with left-sided body weakness, left eye ptosis, multiple right facial swellings, right mastoid region pain, slurred speech and swallowing difficulties for a week. His initial diplopia persisted. Ocular examination revealed left eye ptosis with total ophthalmoplegia [Figure 2]. Fundus showed bilateral swollen optic discs with splinter hemorrhages. There was involvement of all cranial nerves sparing the olfactory, facial and spinal-accessory nerves. There was multiple firm to hard nodules on his face and neck.
Figure 2: Nine gazes of patient showing left eye proptosis with total ophthalmoplegia

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A repeated CT Scan of the brain revealed enhanced soft tissue densities at bilateral infra-temporal region, left high parietal and extraconal right lacrimal gland [Figure 3]. Biopsy was obtained from the soft tissues of the right maxilla and lower left alveolar ridge. Bone biopsy was also taken from the buccal of right maxilla. Histopathological examination revealed fragments of tumor tissue consisting of diffuse infiltration with neoplastic lymphoid cells in starry sky appearance [Figure 4]. The lymphoma cells exhibit uniformity, were medium sized with coarse chromatin and scanty cytoplasm. Mitotic figures and apoptotic bodies were seen. Immunohistochemistry studies showed the lymphoma cells were diffusely positive for CD20, CD79a, and CD10. They were negative for CD3, BCL-2, and TDT. The proliferation index estimated by Ki67 is almost 100% [Figure 5]. A diagnosis of Burkitt's lymphoma was made. FISH and cytogenetic studies were not done. There was no evidence of tumor lysis syndrome.
Figure 3: CECT Brain showing enhanced soft tissue densities at extraconal right lacrimal gland

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Figure 4: H and E × 4, ×10, ×20: Diffuse infiltration with neoplastic lymphoid cells in starry-sky appearance. The cells exhibit uniform, medium-size cells with coarse chromatin and scanty cytoplasm

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Figure 5: (a) CD20, (b) CD79a, (c) CD10: The lymphoma cells are diffuse, strong positive. (d) Ki67 proliferation index is almost 100%

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Subsequent bone marrow biopsy was done and showed no evidence of marrow infiltration. He received B-cell Acute lymphocytic leukemia protocol chemotherapy. Unfortunately, the patient succumbed prior to his second cycle of chemotherapy.


  Discussion Top


Our case was a sporadic disseminated BL (non-endemic area) in an immunocompetent individual. The sporadic and endemic forms are seen during childhood, contrary to our patient who was a middle-aged man.[2] It typically manifests as intra-abdominal tumor with bone marrow involvement. Ocular and orbital involvement as first presentation of the sporadic variant is unusual.[3],[4]

BL first involving the brain is infrequent, with less than ten cases found in the literature.[5],[6] Out of these, cranial nerves and meninges are the common sites of infiltration. Meningeal enhancement is better seen on contrasted T1-weighted images of the MRI rather than CT scans. There was a delay in requesting for an MRI brain for our patient due to limited facilities and our region not known to be endemic for BL.

Initial diagnosis of IIH was made because both CT brain and lumbar puncture were normal, with an elevated opening pressure. The frequency of IIH in men is remarkably low. In a recent meta-analysis by Gavin McCluskey et al., 87% out of a total of 889 patients were women.[7] There is also a positive correlation of IIH with obesity, whereas our patient was not obese.

Overdiagnosis of IIH was highlighted in a retrospective study by Adeniyi et al.[8] An alarming 39.5% of patients referred for presumed IIH prompted unnecessary tests. Out of these, only 9.3% had missed diagnoses that required further investigations.

CSF cytology at the early stage did not yield Burkitt's cells in our patient. CSF cytology has been reported to have a sensitivity of 75%–77% in leptomeningeal metastases, but more than two examinations were needed in 72% of positive cases.[9],[10] We propose multiple high-volume taps if neurolymphomatosis is strongly suspected.


  Conclusion Top


The combination of bilateral optic disc swelling with bilateral abducens nerve palsies as a false localizing sign signifies raised ICP. Urgent brain imaging particularly MRI along with multiple high-volume taps of lumbar puncture is advised if clinical suspicion of neurolymphomatosis is high. IIH remains a diagnosis of exclusion only after extensive investigation.

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 Top

1.
Johnson KA, Tung K, Mead G, Sweetenham J. The imaging of Burkitt's and Burkitt-like lymphoma, Clin Radiol 1998;53:835-41.  Back to cited text no. 1
    
2.
Van den Bosch CA. Is endemic Burkitt's lymphoma an alliance between three infections and a tumour promoter? Lancet Oncol 2004;5:738-46.  Back to cited text no. 2
    
3.
Ziegler JL. Burkitt's lymphoma. N Engl J Med 1981;305:735-45.  Back to cited text no. 3
    
4.
Zaman AG, Graham EM, Sanders MD. Anterior visual system involvement in non-Hodgkin's lymphoma. Br J Ophthalmol 1993;77:184-7.  Back to cited text no. 4
    
5.
Kobayashi H, Sano T, Li K, Hizawa K. Primary Burkitt-type lymphoma of the central nervous system. Acta Neuropathol 1984;64:12-4.  Back to cited text no. 5
    
6.
Monabati A, Rakei SM, Kumar P, Taghipoor M, Rahimi A. Primary Burkitt lymphoma of the brain in an immunocompetent patient. Case report. J Neurosug 2002;96:1127-9.  Back to cited text no. 6
    
7.
McCluskey G, Doherty-Allan R, McCarron P, Loftus AM, McCarron LV, Mulholland D, et al. Meta-analysis and systematic review of population-based epidemiological studies in idiopathic intracranial hypertension. Eur J Neurol 2018;25:1218-27.  Back to cited text no. 7
    
8.
Fisayo A, Bruce BB, Newman NJ, Biousse V. Overdiagnosis of idiopathic intracranial hypertension. Neurology 2016;86:341-50.  Back to cited text no. 8
    
9.
Mackintosh FR, Colby TV, Podolsky WJ, Burke JS, Hoppe RT, Rosenfelt FP, et al. Central nervous system involvement in non-Hodgkin's lymphoma: An analysis of 105 cases. Cancer 1982;49:586-95.  Back to cited text no. 9
    
10.
Straathof CS, De Bruin HG, Dippel DW, Vecht CJ. The diagnostic accuracy of magnetic resonance imaging and cerebrospinal fluid cytology in leptomeningeal metastasis. J Neurol 1999;246:810-4.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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