Primary EBV-associated cauda equina lymphoma

Giobbia, Mario; Carniato, Antonella; Scotton, Pier Giorgio; Vaglia, A.; Marchiori, G. C.

doi:10.1007/s004150050445

Cited by 15 publications

(10 citation statements)

References 1 publication

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Diffuse thickening of the cauda equina nerve roots or intradural nodular masses with enhancement of the lesions on post contrast MRI were the most common radiological findings [2,3,5,11,13]. In our patient high resolution contrast enhanced MR neurography was inconclusive and repeat CSF analysis was normal.…”

Section: Dear Sirsmentioning

confidence: 52%

“…So far only a few cases of primary malignant lymphoma of the lumbar spine with disease apparently confined to the leptomeninges have been reported [2-6, 8, 11, 13, 15]. Generally patients presented with a brief history of progressive lower limb weakness and, on admission, neuroimaging depicted leptomeningeal disease in most cases [3,5,6,11,15].…”

Section: Dear Sirsmentioning

confidence: 99%

See 1 more Smart Citation

Primary leptomeningeal lymphoma of the cauda equina: a rare cause of radiculopathy

et al. 2010

View full text Add to dashboard Cite

Primary leptomeningeal lymphoma (PLML) is a particularly rare neoplasm, accounting for only 7% of all primary central nervous system lymphomas. PLML is defined as a syndrome of lymphomatous meningeal infiltration without identification of systemic lymphoma or parenchymal CNS lymphoma in the clinical course of the disease [6,12]. The diagnosis is challenging and requires a high index of suspicion. Early diagnosis, however, is crucial as immediate treatment may be beneficial. We here report a case of PLML of the cauda equina to raise awareness for this disorder and reduce the delay to diagnosis in similar cases.A 69-year-old immunocompetent man complained about increasing low back pain and moderate weakness of the left leg, which had slowly developed over 1 year. Neurologic examination and electrodiagnostic studies indicated a left-sided L5-S1 radiculopathy. There was no evidence of polyneuropathy. MRI of the spine, the pelvis, and the brain, including contrast-enhanced sequences, was unremarkable. Routine lumbar puncture yielded normal findings. The patient underwent an extensive laboratory and image work-up to rule out malignant, autoimmune, or infectious disease. Due to negative findings, he was discharged to home. High-resolution, contrast-enhanced MR neurography [7, 10] and a second lumbar tap were performed 36 days later. Analysis of the cerebrospinal fluid (CSF) revealed 0 cells/ll with a normal glucose and protein content and imaging findings were again inconclusive ( Fig. 1a and b). Short inversion time inversion recovery (STIR) images, however, then illustrated hyperintensity and contrast enhancement of the left piriformis muscle and the left medial gluteal muscle, suggesting acute denervation (Fig. 1c). Subsequent treatment with intravenous methylprednisolone 10 mg/kg/day for 5 days was without clinical benefit at that point.Only 9 months later, the patient was readmitted with a pronounced flaccid lower extremity paraparesis and severe low back pain. Contrast-enhanced MRI of the lumbar spine then disclosed abnormal thickening and marked linear enhancement coating multiple cauda equina nerve roots highly suggestive of leptomeningeal infiltration by tumor (Fig. 2a-c). Urgent analyses of the CSF revealed elevated protein (439 mg/dl; normal range: 15-45) and a lymphocytic pleocytosis (70 cells/ll) with many atypical cells consistent with lymphoma. A biopsy of an anterior cauda equina nerve root as highlighted by immunohistochemistry with S100 (Fig. 2d) and NSE revealed a massive diffuse interstitial infiltrate of atypical lymphocytic cells, that reached the marginal area of the small specimen with epithelial membrane antigen (EMA) positive cells indicating leptomeningeal infiltration [14]. The cells expressed CD20 and CD79, but were negative for Kl-1, EMA, CD56,

show abstract

Section: Dear Sirsmentioning

confidence: 52%

Section: Dear Sirsmentioning

confidence: 99%

Primary leptomeningeal lymphoma of the cauda equina: a rare cause of radiculopathy

et al. 2010

View full text Add to dashboard Cite

show abstract

“…Most previous cases were reported to have relapsed and died in the early phase, within several months after the initial diagnosis. Their therapeutic options were not as intensive as our case [1][2][3][4][5][6][7][8][9][10]. So, we performed combination chemotherapy with high-dose Ara-C, in addition to high-dose MTX, followed by radiotherapy to remain the patient in CR without any signs of recurrence.…”

Section: Discussionmentioning

confidence: 99%

“…Less than 1% of primary central nervous lymphoma occurs in the spinal cord and previously, there have been only 12 reported cases of malignant lymphoma of the cauda equina, so the optimal treatment strategy for this condition has not been established yet and the prognosis is thought to be poor [1][2][3][4][5][6][7][8][9][10].…”

Section: Discussionmentioning

confidence: 99%

“…Examination of the spinal MRI with intravenous Gd has been shown to be helpful in detecting the extent of the disease lesions of the spinal cord and the thickened cauda equina and nerve roots are clearly demonstrated after the Gd enhancement [1][2][3][4][5][6][7][8][9]. The differential diagnosis of primary intradural tumors at the level of the cauda equina consist first of tumors, such as metastases, neurofibromas, ependymomas, and schwannomas, secondly of infections, including tuberculosis, toxoplasmosis, cryptococcal granulomatas, inflammatory polyradiculoneuropathy, vasculitis, and arachnoiditis, Lastly, Guillain-Barre syndrome, sarcoidosis, and so on [1][2][3][4][5][6][7][8]. In our case, the detection of the thickened cauda equina with markedly increased Gd enhancement on the spinal MRI examination was the main finding, highly for suspicious of the diagnosis of malignant tumors and especially, the patient's 10-day history of rapidly progressive clinical symptoms absolutely required us of the early definitive diagnosis to start the therapy and reverse the patient's muscle weakness as soon as possible, so the examination of the CSF was quickly performed, which demonstrated the appearance of CD20 positive atypical large lymphoma cells, as described above (Fig.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Primary B-cell lymphoma of the cauda equina, successfully treated with high-dose methotrexate plus high-dose cytarabine: a case report with MRI findings

2011

View full text Add to dashboard Cite

Primary malignant lymphoma of the cauda equina is an extremely rare disease. Previously, there have been only 12 reported cases of malignant lymphoma of the cauda equina, and most cases relapsed early in the clinical course. So, the optimal treatment strategy for this condition has not been established yet and the prognosis is thought to be poor. We experienced a case of B-cell malignant lymphoma of the cauda equina, with rapid progression of the muscle weakness of the bilateral lower extremities, successfully treated with high-dose methotrexate plus high-dose cytarabine (Ara-C) chemotherapy, followed by radiotherapy and in complete remission without any recurrence signs, 1.5 years after the initial diagnosis. Intrathecal chemotherapy with MTX, Ara-C, and predonisolone was simultaneously performed. We should carefully continue to monitor the clinical course of our case, with the examinations of magnetic resonance imaging and cerebrospinal fluid in order not to overlook any subtle neurological changes or other clinical symptoms.

show abstract

Disseminated enteropathy-type T-cell lymphoma: cauda equina syndrome complicating coeliac disease

Doran

Plessis

Larner

2005

Clinical Neurology and Neurosurgery

View full text Add to dashboard Cite

Primary EBV-associated cauda equina lymphoma

Cited by 15 publications

References 1 publication

Primary leptomeningeal lymphoma of the cauda equina: a rare cause of radiculopathy

Primary leptomeningeal lymphoma of the cauda equina: a rare cause of radiculopathy

Primary B-cell lymphoma of the cauda equina, successfully treated with high-dose methotrexate plus high-dose cytarabine: a case report with MRI findings

Disseminated enteropathy-type T-cell lymphoma: cauda equina syndrome complicating coeliac disease

Contact Info

Product

Resources

About