Atypical cauda equina presentation of an extranodal nasal type NK/T cell lymphoma: a case report

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Abstract

Background Extranodal NK/T-cell nasal type lymphoma, is an uncommon subtype of non-Hodgkin’s lymphoma. It is mostly located in the nasal cavity, but in some cases can be found outside of it. The central nervous system (CNS) is a rare site of primary or progression of this lymphoma. Only one similar case of cauda equina syndrome had been reported previously in a postmortem patient. Patients with this type of lymphoma have a poor prognosis and some authors have described a median overall survival of 8.5 months from diagnosis (range 3.8–24 months from CNS involvement). Case report We report a case of a 38-year-old female with diagnosis of extranodal NK/T-cell nasal lymphoma. After radiotherapy and chemotherapy, she was admitted to the emergency room presenting neurologic impairment in the limbs (2/5 in MRC grading scale). Brain neuroimaging did not show any abnormality and lumbar puncture was not possible despite multiple attempts. Thoracolumbar imaging showed an involvement of the cauda equina and intraoperative findings revealed thickening and swelling of the nerve roots confirming an infiltration by lymphoma in the pathology. To date, our patient has an overall survival of 18 months from diagnosis of lymphoma and 7 months from peripheral nervous system involvement (actually 5/5 in MRC grading scale). Conclusions In this particular case, the surgery goal was to obtain a diagnosis to guide treatment but not to relieve symptoms. We considered its diffusion and knowledge as imperative for all practitioners involved in the care of this patient.

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Keywords

Extranodal nasal type NK-T-Cell Lymphoma, Peripheral Nervous System, Cauda Equina Sydrome

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