Neurolymphomatosis in Recrudescent Diffuse Large B-cell Lymphoma

Document Type : Case report

Authors

1 Drexel University College of Medicine, Philadelphia, PA, USA

2 Department of Pathology, Massachusetts General Hospital, Boston, MA, USA

3 Department of Radiology, Division of Nuclear Medicine and Molecular Imaging, Massachusetts General Hospital, Boston, MA, USA

Abstract

Neurolymphomatosis is an uncommon manifestation of lymphoma, often presenting with painful polyneuropathy or polyradiculopathy and concomitant distal extremity weakness. Differentiation from other etiologies resulting in similar neuropathic symptoms such as compressive or inflammatory pathologies can be difficult and often results in delayed diagnosis. Here we describe a case of neurolymphomatosis affecting a 64-year-old man with a history of diffuse large B-cell lymphoma (DLBCL) in remission presenting with a right-sided foot drop following a gunshot wound. MRI at that time demonstrated thickening and enhancement of the cauda equina nerve roots. Over the course of the subsequent eight months, he developed left lower extremity sensory symptoms, left-sided foot drop and signs of upper motor neuron involvement, including left facial weakness, dysphonia, and dysphagia. 18F-FDG PET/CT revealed intensely avid left lumbosacral nerve roots, bilateral lower extremity and left upper extremity neurovascular bundles. Left sural nerve biopsies showed infiltration of DLBCL and confirmed neurolymphomatosis. We highlight the role of 18F-FDG PET/CT, with histological verification, for the diagnosis of an extended course of neurolymphomatosis occurring in the absence of typical painful neuropathy but with cranial and peripheral neuropathies.

Keywords


  1. Levin N, Soffer D, Grissaru S, Aizikovich N, Gomori JM, Siegal T. Primary T-cell CNS lymphoma presenting with leptomeningeal spread and neurolymphomatosis. J Neurooncol. 2008; 90(1):77-83.
  2. Baehring JM, Damek D, Martin EC, Betensky RA, Hochberg FH. Neuro-lymphomatosis. Neuro Oncol. 2003; 5(2):104-15.
  3. Lagarde S, Tabouret E, Matta M, Franques J, Attarian S, Pouget J, et al. Primary neurolymphomatosis diagnosis and treat-ment: a retrospective J Neurol Sci. 2014; 342(1-2):178-81.
  4. Grisariu S, Avni B, Batchelor TT, van den Bent MJ, Bokstein F, Schiff D, et al. Neurolymphomatosis: an International Primary CNS Lymphoma Collaborative Group report. Blood. 2010; 115(24): 5005-11.
  5. Shree R, Goyal MK, Modi M, Gaspar BL, Radotra BD, Ahuja CK, et al. The Diagnostic Dilemma of Neuro-lymphomatosis. J Clin Neurol. 2016; 12 (3):274-81.
  6. Bourque PR, Warman Chardon J, Bryanton M, Toupin M, Burns BF, Torres C. Neurolymphomatosis of the Brachial Plexus and its Branches: Case Series and Literature Can J Neurol Sci. 2018; 45(2):137-43.
  7. Lim AT, Clucas D, Khoo C, Parameswaran BK, Lau E. Neuro-lymphomatosis: MRI and (18) FDG-PET features. J Med Imaging Radiat Oncol. 2016; 60(1):92-5.
  8. De Vries AH, Howe BM, Spinner RJ, Broski SM. B-cell peripheral neurolymphomatosis: MRI and (18)F-FDG PET/CT imaging characteristics. Skeletal Radiol. 2019; 48(7):1043-50.
  9. Davidson T, Kedmi M, Avigdor A, Komisar O, Chikman B, Lidar M, et al. FDG PET-CT evaluation in neuro-lymphomatosis: imaging characteristics and clinical outcomes. Leuk Lymphoma. 2018; 59(2):348-56.
  10. Jeong J, Kim SW, Sung DH. Neuro-lymphomatosis: a single-center experience of neuromuscular manifestations, treat-ments, and outcomes. J Neurol. 2021; 268(3):851-9.
  11. Kamiya-Matsuoka C, Shroff S, Gilder-sleeve K, Hormozdi B, Manning JT, Woodman KH. Neurolymphomatosis: a case series of clinical manifestations, treatments, and J Neurol Sci. 2014; 343(1-2):144-8.
  12. Khurana A, Novo M, Nowakowski GS, Ristow KM, Spinner RJ, Hunt CH, et al. Clinical manifestations of, diagnostic approach to, and treatment of neurolymphomatosis in the rituximab era. Blood Adv. 2021; 5(5):1379-87.
  13. Li V, Jaunmuktane Z, Cwynarski K, Carr A. Diagnostic delay in a case of T-cell neurolymphomatosis. BMJ Case Rep. 2019; 12(12): e232538.
  14. 1Canh NX, Tan NV, Tung TT, Son NT, Maurea S. (18)F-FDG PET/CT in Neurolymphomatosis: Report of 3 Cases. Asia Ocean J Nucl Med Biol. 2014; 2(1):57-64.
  15. Yamada S, Tanimoto A, Nabeshima A, Tasaki T, Wang KY, Kitada S, et al. Diffuse large B-cell lymphoma presenting with neurolymphomatosis and intravascular lymphoma: a unique autopsy case with diverse neurological symptoms. Diagn Pathol. 2012; 7:94.
  16. Lahoria R, Dyck PJ, Macon WR, Crum BA, Spinner RJ, Amrami KK, et al. Neurolymphomatosis: A report of 2 cases representing opposite ends of the clinical Muscle Nerve. 2015; 52(3):449-54.
  17. Baehring JM, Batchelor TT. Diagnosis and management of neurolymphoma-tosis. Cancer J. 2012; 18(5):463-8.
  18. Gan HK, Azad A, Cher L, Mitchell PL. Neurolymphomatosis: diagnosis, manage-ment, and outcomes in patients treated with rituximab. Neuro Oncol. 2010; 12(2): 212-5.