Primary spinal epidural lymphoma: a rare entity with an ambiguous management

Pinheiro, João Páscoa; Rato, Joana R.; Rebelo, Olinda; Costa, G

doi:10.1136/bcr-2019-233442

Cited by 6 publications

(5 citation statements)

References 22 publications

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“…A total of 65 studies 3,13,17,30–91 with 72 cases of primary spinal B-cell lymphoma were included (Fig. 1).…”

Section: Resultsmentioning

confidence: 99%

Surgical Intervention for Primary B-cell Lymphoma of the Spine

Gendreau,

Patel,

Brown

et al. 2023

Clinical Spine Surgery: A Spine Publication

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Study Design: Systematic review and meta-analysis. Objective: To perform a systematic review of the clinical symptoms, radiographic findings, and outcomes after spinal decompression in B-cell lymphoma. Summary of Background Data: B-cell lymphoma is a potential cause of spinal cord compression that presents ambiguously with nonspecific symptoms and variable imaging findings. Surgical decompression is a mainstay for both diagnosis and management, especially in patients with acute neurological deficits; however, the efficacy of surgical intervention compared with nonoperative management is still unclear. Methods: The databases of Medline, PubMed, and the Cochrane Database of Systemic Reviews were queried for all articles reporting spinal B-cell lymphoma. Data on presenting symptoms, treatments, survival outcomes, and histologic markers were extracted. Using the R software “survival” package, we generated bivariate and multivariate Cox survival regression models and Kaplan-Meier curves. Results: In total, 65 studies were included with 72 patients diagnosed with spinal B-cell lymphoma. The mean age was 56.22 (interquartile range: 45.00–70.25) with 68% of patients being males and 4.2% of patients being immunocompromised. Back pain was the most common symptom (74%), whereas B symptoms and cauda equina symptoms were present in 6% and 29%, respectively. The average duration of symptoms before presentation was 3.81 months (interquartile range: 0.45–3.25). The most common location was the thoracic spine (53%), with most lesions being hyperintense (28%) on T2 magnetic resonance imaging. Surgical resection was performed in 83% of patients. Symptoms improved in 91% of patients after surgery and in 80% of patients treated nonoperatively. For all 72 patients, the overall survival at 1 and 5 years was 85% (95% CI: 0.749–0.953; n = 72) and 66% (95% CI: 0.512–0.847; n = 72), respectively. Conclusion: Although surgery is usually offered in patients with acute spinal cord compression from B-cell lymphoma, chemotherapy and radiation alone offer a hopeful alternative to achieve symptomatic relief, particularly in patients who are unable to undergo surgery.

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“…A total of 65 studies 3,13,17,30–91 with 72 cases of primary spinal B-cell lymphoma were included (Fig. 1).…”

Section: Resultsmentioning

confidence: 99%

Surgical Intervention for Primary B-cell Lymphoma of the Spine

Gendreau,

Patel,

Brown

et al. 2023

Clinical Spine Surgery: A Spine Publication

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“…Out of all the primary central nervous system (CNS) tumors (which also includes spinal lymphomas), primary DLBCL of the CNS constitutes only 0.5% cases, occurring in approximately two cases per 10 million people. Spinal cord location is particularly rare 6 …”

Section: Discussionmentioning

confidence: 99%

Primary spinal epidural non‐Hodgkin's diffuse large B‐cell lymphoma: A case report

Pokhrel

Prasad

Paudel

et al. 2020

Clinical Case Reports

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A 24-year-old man presented with radiating back pain and progressive neurological deficit. MRI revealed a spinal epidural tumor at thoracolumbar junction. He underwent decompression and excisional biopsy. Histopathology and immunohistochemistry identified it as diffuse large B-cell lymphoma. He received chemotherapy and is asymptomatic at 1-year follow-up. Primary spinal epidural lymphomas (PSELs) comprise a group of tumors, which are present only in the spinal epidural space, with a histopathological picture of lymphoma and negative diagnostic workup for lymphoma at other sites. 1 The epidural location of lymphomas, both Hodgkin's and non-Hodgkin's, is infrequent and challenging to diagnose. About 24%-48% of non-Hodgkin's lymphomas (NHLs) were found to have an extranodal origin. 2,3 However, PSEL accounts for only 0.9% of all extranodal NHLs. 4 There are many cell types of NHLs. Primary spinal epidural DLBCL is the most common form which accounts for only 1.8% all diffuse large B-cell lymphomas. 5 It is easily missed and maybe misdiagnosed leading to a lack of timely intervention and unhindered tumor progression. This report describes the clinical features, imaging characteristics, and histopathological features of the rare case of primary spinal epidural diffuse large B-cell lymphoma (DLBCL). To the best of our knowledge, this is the first reported case of primary spinal epidural DLBCL from Nepal. It also emphasizes the importance of a multidisciplinary approach for the successful treatment of this disease. 2 | CASE REPORT A 24-year-old man presented with a 3-month history of low back pain associated with the burning sensation of bilateral lower limbs. For the last 12 days, he was unable to walk due to gradually progressive weakness in bilateral lower limbs. These symptoms were not accompanied by fever, headache, and night sweats. He could not recall any history of trauma in the past. His past medical and surgical history was not significant. On examination, there was a localized tenderness over the thoracolumbar region. Lower limb motor power varied across muscle groups: hip flexors (2/5 bilaterally), knee extensors (2/5 bilaterally), ankle dorsiflexors (2/5 bilaterally), long toe

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“…Spinal cord location is particularly rare. 6 The pathogenesis of primary central nervous system lymphomas (PCNSLs) has been a matter of considerable debate among experts. Some authors mention that PCNSL arise de novo in nervous system tissues due to the presence of lymphoid precursor cells in this location.…”

Section: Discussionmentioning

confidence: 99%

Primary spinal epidural non-Hodgkin’s diffuse large B-cell lymphoma: a case report

Pokhrel

Prasad

Paudel

et al. 2020

Preprint

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A 24-year-old male presented with radiating back pain and progressive neurological deficit. MRI revealed spinal epidural tumor at the thoracolumbar junction. He underwent decompression and excisional biopsy. Histopathology and immunohistochemistry identified it as diffuse large B-cell lymphoma. He received chemotherapy and is asymptomatic at one-year follow-up. Key clinical message Rarer diseases like primary spinal epidural diffuse large B-cell lymphomas should also be considered as the differential in those patients who complain of back pain with rapid neurological deterioration in lower limbs.

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Primary spinal epidural lymphoma: a rare entity with an ambiguous management

Cited by 6 publications

References 22 publications

Surgical Intervention for Primary B-cell Lymphoma of the Spine

Surgical Intervention for Primary B-cell Lymphoma of the Spine

Primary spinal epidural non‐Hodgkin's diffuse large B‐cell lymphoma: A case report

Primary spinal epidural non-Hodgkin’s diffuse large B-cell lymphoma: a case report

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