Abstract
Spinal involvement in lymphomas is often associated with advanced disease. Primary spinal non-Hodgkin’s lymphoma is a rare entity. A 47-year-old male presented with a history of neck pain followed by progressive quadriparesis and bowel bladder involvement over a 5-month period. The magnetic resonance imaging was suggestive of an intradural extramedullary lesion at the C1–C2 vertebra level. A surgical excision was done and the histopathology revealed atypical lymphoid cells, which are immunopositive for CD45, CD20, MUM-1, and BCL6, while negative for BCL2, EBV (LMP-1 and CISH), Cyclin D1 and confirmed the diagnosis of Burkitt’s lymphoma. The patient received chemotherapy in the form of CODOX-M/IVAC (cyclophosphamide, vincristine, doxorubicin, high-dose methotrexate/ifosfamide, etoposide, high-dose cytarabine) regimen. Primary spinal intradural extramedullary Burkitt’s lymphoma is a rare diagnosis that may often be difficult to differentiate radiologically from other causes of intradural extramedullary lesions. A thorough histological examination is warranted in such cases.
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Introduction
Burkitt’s lymphoma (BL) is a B cell non-Hodgkin lymphoma that is rapidly growing and follows an aggressive course [1]. The spine is an unusual and rare primary site for BL [1]. However, when involved the spine is primarily involved, it often presents as an epidural or intramedullary lesion [2,3,4,5,6,7,8,9,10]. Primary intradural extramedullary (IDEM) lesions of BL are even rarer with only a few reports described [3]. In this report we present the case of a 47-year-old male presenting with quadriparesis with an IDEM lesion at C1–C2 level that turned out to be a BL.
Case
A 47-year-old male presented to us with a history of pain in the neck for the past 5 months. It was associated with numbness and weakness of all four limbs for the past 3 months and urinary retention with a catheter in situ for the past 2 months. The patient did not have any comorbidity or any other significant past history.
On examination, the patient was conscious and oriented. There were no cranial nerve deficits. Tone was increased in all four limbs. The patient had left-sided hemiplegia and on the right side, the power was 1/5 for right elbow flexion and 2/5 for right extensor halluces longus. The power in other muscle groups on the right side was 0/5. All upper and lower limb deep tendon reflexes were exaggerated. The Hoffman sign was positive in both upper limbs and planters were up going on both sides. The patient had graded sensory loss below C3 dermatome. The patient also had a grade two bedsore in the sacral region. The breath holding time was 20 s.
The radiographs of the patient were unremarkable. The MRI was suggestive of T1 and T2 isointense, STIR hyperintense, and a contrast-enhancing IDEM lesion at C1 and C2 levels with extension to and widening of left lateral neural foramina at C1–C2 involving exiting nerve root (Figs. 1 and 2). There was also cord edema present extending to the upper C5 level. Based on the imaging findings, a differential diagnosis of Schwannoma was kept.
The patient was operated under general anesthesia in a prone position and a standard midline exposure was done. A rim of the foramen magnum was removed and C1–C2 laminectomy was done. On opening the duramater, there was an IDEM tumor in the region of left side C1 and C2. The tumor was adherent to the left side duramater with extension to C2 neural foramen and anterior extension was also noticed. It was firm, moderately vascular in close relation to C2 nerve root. C1, C2 nerve root and dentate ligament of left side sacrificed to approach the anterior/foraminal part of the tumor. Near-total excision was achieved. A small part of the tumor in neural foramina was left behind. A lax duroplasty was done and a C2 laminoplasty was performed. The postoperative course was uneventful and the patient had some neurological recovery with the attainment of 3/5 power across all joints on the right side. However, there was no recovery on the left side.
The histopathological examination reveals sheets of atypical lymphoid cells, which are mildly pleomorphic and have round and oval hyperchromatic nuclei (Fig. 3). These atypical lymphoid cells were immunopositive for CD45, CD20, MUM-1, and BCL6, while negative for CD10, BCL2, EBV (LMP-1 and CISH), and Cyclin D1 (Figs. 4–6). CD3 highlights the presence of background T cells. MIB-1 labeling index was approximately 92%. These findings were consistent with a diagnosis of BL. A contrast-enhanced CT scan of the neck, chest, abdomen, and pelvis was done that was negative for any lymphadenopathy at any other site. The patient received chemotherapy in the form of CODOX-M/IVAC (cyclophosphamide, vincristine, doxorubicin, high-dose methotrexate/ifosfamide, etoposide, high-dose cytarabine) regimen. The patient died 4 months post surgery at home due to unknown causes.
Discussion
The abdomen and pelvis are common sites of involvement of BL and spinal involvement has only been reported in advanced stages of the disease [1]. Primary spinal BL is rare and most reports have suggested an epidural or an intramedullary involvement [2,3,4,5,6,7,8,9,10]. A literature review of cases of primary spinal BL is presented in Table 1. Only one case of a single primary spinal IDEM BL has been reported so far [3]. We report a second such case in the present article.
Weakness, numbness, and difficulty in walking are often the presenting complaints of spinal lymphoma [11]. They are often solitary and involve the thoracic spine more often than the cervical and lumbar spine [11, 12]. In our case, the patient presented with neck pain that was followed by weakness and quadriparesis.
A contrast-enhanced MRI is often the preferred modality for the evaluation of spinal tumors [13]. The radiological features of the lesion like contrast enhancement, STIR hyperintensity, IDEM location, and extension along the C2 nerve root with the widening of the neural foramina were greatly in favor of the diagnosis of a benign nerve sheath tumor [13]. The presence of neural foraminal widening also brings in the possibility that the lesion may have been present and growing for quite some time before symptoms appeared and clinical attention was sought. The histopathology was strongly supportive of a non-Hodgkin’s lymphoma and IHC markers with strong positivity for CD20, CD45, BCL6 confirmed the diagnosis of BL [14].
All the reports of primary spinal BL have been treated by surgical decompression followed by chemotherapy with or without radiation [2,3,4,5,6,7,8,9]. A similar approach was followed in our case. High-dose methotrexate-based chemotherapy is often the first-line treatment for spinal lymphomas and a similar approach in the form of CODOX-M/IVAC regimen was used in our case [15]. The approach of surgery followed by chemoradiation may be due to the rarity and diagnostic dilemma of these lesions in the spine. A few reports have recently suggested that spinal lymphomas may respond well to radiation alone; however, the exact role is yet to be explored [16]. The patient in our case expired 4 months post surgery while chemotherapy was ongoing. The prognosis of primary spinal lymphomas can be highly variable and survival can range from a few months to years (Table 1). The variability could be due to a number of factors like age, functional status, location of the lesion, neurological status, and response and tolerance to chemotherapy. A high cervical lesion with quadriparesis and poor respiratory reserve may have been possible causes of poor prognosis in this case.
Conclusion
Primary spinal IDEM BL is a rare diagnosis that may often be difficult to differentiate radiologically from other causes of IDEM lesions. A thorough histological examination becomes essential in such cases due to different postoperative courses based on etiology.
Data availability
The authors confirm that the data supporting the findings of this study are available within the article. Further details, if needed, can be elucidated from the corresponding author on request.
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BT, SS, SM, and BS contributed to concept and design, data analysis, manuscript preparation, manuscript editing, and approval of final version of manuscript. BT and SS contributed to data acquisition.
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Bansal, T., Sahu, S., Sharma, M.C. et al. Primary intradural extramedullary spinal Burkitt’s lymphoma mimicking a nerve sheath tumor: a case report. Spinal Cord Ser Cases 8, 53 (2022). https://doi.org/10.1038/s41394-022-00520-y
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DOI: https://doi.org/10.1038/s41394-022-00520-y
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