Neurolymphomatosis in the Cauda Equina Diagnosed by an Open Biopsy

Abstract

Neurolymphomatosis is a rare form of extranodal malignant lymphoma defined as the infiltration of malignant lymphocytes into the central or peripheral nerve. We herein report a case of neurolymphomatosis in the cauda equina diagnosed by an open surgical biopsy. He presented with muscle weakness, atrophy, numbness and hypoesthesia in the bilateral lower extremities with the accumulation of fluoro-2-deoxyglucose (FDG) in the bilateral cauda equina. Cerebrospinal fluid cytology (three times) and flow cytometry (two times) and biopsies of the left sural nerve, bone marrow, paranasal sinus and left testis were all negative for malignancy, so finally we performed a surgical open biopsy of the cauda equina by laminectomy and diagnosed him with diffuse large B-cell lymphoma in the cauda equina. He was successfully treated with the disappearance of the FDG accumulation for a long time. The present case suggested that an early open biopsy of the cauda equina may be considered for cases of suspected neurolymphomatosis in the cauda equina for a good outcome.

Primary Extranodal NK/T-Cell Lymphoma Presenting as Neurolymphomatosis Involving Multiple Cranial Nerves: A Case Report

“…Although CSF PCR immunoglobulin and T-cell receptor gene rearrangement studies may be a useful adjunct in the detection of NL more generally, such studies would be of limited value in the detection of ENKTL, as most ENKTLs are of true NK lineage, with T-cell receptor genes in germline configuration [8]. While incorporating flow cytometry in CSF analysis improves sensitivity for detection of CNS lymphoma [27], in NL, CSF flow cytometry studies may be repeatedly negative, even in biopsy-proven disease [28].…”

Primary Central Nervous System Lymphoma with Peripheral Nerve Involvement: Case Report

“…Khandelwal et al presented a case of NL in the left C4 and C5-C8, and L4-L5 and S1-S2 [2]. Broen et al [1], Sasaki et al [3], and Marquardt et al [7] reported cases of NL in the cauda equina or lumbosacral root/plexus. Misdraji et al reported four cases of NL in the sciatic nerve (two cases), radial nerve, or sympathetic chain and spinal nerve [8].…”

“…Only 1% of primary CNS NHL emerges in the spinal cord. A syndrome of lymphoma and leukemic infiltration of the cranial or peripheral nerves is called neurolymphomatosis (NL) [2-3]. It is a rare clinical entity, with an incidence of 0.2% in all NHL patients [3].…”

“…A syndrome of lymphoma and leukemic infiltration of the cranial or peripheral nerves is called neurolymphomatosis (NL) [2-3]. It is a rare clinical entity, with an incidence of 0.2% in all NHL patients [3]. Several studies have reported primary and secondary NL with single and multiple cranial or spinal nerve involvement [1-9].…”

The Prevalence of Vitamin D Deficiency and its Correlation to Obesity- Related Complications in Obese Children from West Virginia

“…7,8,9 In the setting of disease progression and non-diagnostic testing (including advanced imaging modalities), the importance of tissue diagnosis cannot be overstated. 10,11,12 Even if a diagnosis of NL is obtained, management decisions are purely anecdotal in the literature. Current systemic chemotherapy treatment options include intravenous high dose methotrexate, high dose cytarabine, or combination chemotherapy including rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone.…”