Brachial and sacral plexus neurolymphomatosis - unusual regions for disease relapses

Neurolinfomatose nos plexos braquial e sacral - uma apresentação clínica atípica para recidiva

A 49-year-old man who was diagnosed with diffuse large B cell lymphoma was treated with cyclophosphamide, doxorubicin, vincristine, and prednisone, plus the monoclonal antibody rituximab (R-CHOP protocol) and achieved a confirmed complete response. However, six months later, he complained of superior (upper) and inferior (lower) limb paresthesias. An MRI of the brachial plexus (Figure 1) and sacral plexus (Figure 2) showed diffuse root enlargement that also demonstrated hypermetabolism on PET-CT (Figure 1D and Figure 2D and 2E), characterizing a disease relapse.

Figure 1
Neuroimaging of the brachial plexus. Brachial plexus MRI coronal T1 WI (A), T2/STIR WI (B) and DWI (C) demonstrating brachial plexus root infiltration with abnormal MR signal intensìty (arrows). Note the fusiform aspect in most of the masses, following the larger axis of roots and trunks longitudinally. Enlarged axillar nodes are also depicted (*). PET-CT coronal images (D) reveal noticeable fluorodeoxyglucose hypermetabolism in the brachial plexus.

Figure 2
Neuroimaging of the sacral plexus. Sacral plexus MRI coronal T1 WI (A), coronal DWI (B) and axial T2/STIR (C) images also show fusiform masses along the sacral plexus roots and trunks (arrows). PET-CT coronal (D and E) show sacral plexus fluorodeoxyglucose hypermetabolism.

Despite the low frequency of occurrence, the clinician should also be aware of neuropathy as a symptom for disease recurrence/relapse, allowing for an early diagnosis/treatment. Neurolymphomatosis is a rare lymphoma presentation that occurs when there is direct infiltration of the endoneurium by lymphoma cells^1,2.

• Ethical approval:
  All procedures performed in the studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

References

• ¹
  Gan HK, Azad A, Cher L, Mitchell PL. Neurolymphomatosis: diagnosis, management, and outcomes in patients treated with rituximab. Neuro Oncol. 2010 Feb;12(2):212-5. https://doi.org/10.1093/neuonc/nop021
  » https://doi.org/10.1093/neuonc/nop021
• ²
  Tomita M, Koike H, Kawagashira Y, Iijima M, Adachi H, Taguchi J, et al. Clinicopa thol ogical features of neuropathy associated with lymphoma. Brain. 2013 Aug;136(8):2563-78. https://doi.org/10.1093/brain/awt193
  » https://doi.org/10.1093/brain/awt193

Publication Dates

• Publication in this collection
  05 Dec 2019
• Date of issue
  Nov 2019

History

• Received
  02 Feb 2019
• Reviewed
  06 Mar 2019
• Accepted
  23 Mar 2019