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

Abstract

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.

Primary cauda equina lymphoma diagnosed by nerve biopsy: A case report and literature review

“…Major symptoms include lower back pain, muscle weakness, and numbness of lower limbs, with or without mild to severe urinary dysfunction. In some cases, the symptoms progress rapidly (18)(19)(20). Neurological examination showed cauda equina syndrome in 17 cases including our case (74%) (2,6-10,12,13,15,17-21,23,24), radiculopathy in three cases (13%) (16,22), and other symptoms (polyradiculoneuropathy, irritation, and facial numbness) in three cases (13%) (11,14,25).…”

“…Among primary NLs, invasion of the cauda equina by lymphoma cells is extremely rare. Twenty-three cases of primary CEL, including our case, have been reported so far (2,(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25). We reviewed these 23 cases regarding the clinical characteristics and imaging of primary CEL and summarized these data in Table I.…”

“…However, distinguishing spinal cord tumors from infections with these CSF findings is difficult. Nishida et al reported that cytology and immunophenotyping of CSF are effective diagnostic tools for CEL (20). In addition to general CSF examinations such as the cell count and protein and glucose levels, if atypical cells are observed with CSF cytology, immunophenotyping analysis could improve the accuracy of diagnosis of CEL.…”

Diffuse large B-cell lymphoma of the cauda equina

“…Direct infiltration of the cauda equina is rare and can be the result of a primary localised, disseminated or recurrent NHL. In a large study of 150 cauda equina tumours the incidence of lymphomas was 1.3%3 and over the years only a few case reports of primary cauda equina lymphoma have been published 2 4 5. Examination of the spine with MRI and intravenous gadolinium is recommended and cytomorphological examination of the CSF with immunophenotyping may give the final diagnosis of lymphoma 4.…”

“…In a large study of 150 cauda equina tumours the incidence of lymphomas was 1.3%3 and over the years only a few case reports of primary cauda equina lymphoma have been published 2 4 5. Examination of the spine with MRI and intravenous gadolinium is recommended and cytomorphological examination of the CSF with immunophenotyping may give the final diagnosis of lymphoma 4. However, others6 have also reported repeated inconclusive cytological examinations, where a final diagnosis could only be made after a nerve root biopsy.…”