Neurolymphomatosis of the median nerve, optic nerve, L4 spinal nerve root and cauda equina in patients with B-cell malignancies: a case series

A Al Battah,L Szabados,A Ellahie,A Fadul,A Gameil,M Yassin,R Taha,L J Fernyhough,M Bakr,H El Sabah,S Alazawi,H Elomri,M T Abdelhamid,K Aboudi

doi:10.1186/s13256-021-02714-8

Abstract

BackgroundNeurolymphomatosis is rare. Neoplastic lymphocytes are seen to invade nerves (cranial or peripheral), nerve roots or other related structures in patients with hematological malignancy. It is a separate entity from central nervous system lymphoma. Neurolymphomatosis has most commonly been described in association with B-cell non-Hodgkin lymphoma. Neurolymphomatosis in the context of Burkitt lymphoma and the post-renal transplant setting has not been described before.Case reportsWe report for the first time in the Arabian Gulf countries and nearby Arab states four cases of neurolymphomatosis (one Asian, and the other 3 are from Arabic nationals) occurring between 2012 and 2017 involving the median nerve, optic nerve, nerve root and cauda equina in patients with Burkitt lymphoma, Philadelphia chromosome-positive B-cell acute lymphoblastic leukemia and diffuse large B-cell lymphoma.ConclusionsNeurolymphomatosis is rare and can be difficult to diagnose by biopsy but reliably confirmed by a combined imaging approach. Prior treatment with high-dose dexamethasone might suppress 18F-fluorodeoxyglucose (FDG) activity and decrease the sensitivity of positron emission tomography/computed tomography (PET/CT). The prognosis is generally poor but using high-dose methotrexate as well as high-dose chemotherapy and autologous stem cell transplantation may be an effective way to treat neurolymphomatosis.

Highlights

Neurolymphomatosis is rare and can be difficult to diagnose by biopsy but reliably confirmed by a combined imaging approach
The addition of rituximab (R) to CHOP chemotherapy has been shown to significantly improve the survival in other patients with diffuse large B-cell lymphoma (DLBCL) [27, 28], other reported cases suggest that rituximab does not improve the outcome in NL [29]
In this report we describe for the first time, to our knowledge, one patient who developed NL from Burkitt lymphoma presenting as a post-renal transplant lymphoproliferative disorder (PTLD)