Primary central nervous system diffuse large B-cell lymphoma with MYC and BCL2 rearrangements associated with deep brain stimulation device.

in Virchows Archiv : an international journal of pathology by Kirill A Lyapichev, Sri Bharathi Kavuri, Patrick J Karas, Laura Wu, Nahyun Jo, John Heymann, Hadi Yaziji, Jianli Dong, Farkhod Tursunbaev, Rasha Alfattal, Michelle Madden Felicella

TLDR

  • A 70-year-old woman with a history of DBS therapy for essential tremor developed a rare CNS lymphoma with MYC and BCL2 rearrangements around the DBS electrode.

Abstract

Primary central nervous system (CNS) diffuse large B-cell lymphoma with MYC and BCL2 rearrangements is a very rare lymphoma subtype. Deep brain stimulation is an effective minimally invasive therapeutic option for the treatment of refractory movement disorders, as well as some psychiatric disorders and chronic pain syndromes. Herein, we report a case of CNS lymphoma, which developed around an electrode of a deep brain stimulation (DBS) device. A 70-year-old woman with drug-resistant essential tremor was treated with bilateral thalamic DBS with significant symptomatic improvement over the following year. She presented to the emergency department with recurrent tremor, blurred vision, and confusion. Imaging showed a 4 cm heterogeneously enhancing centered around the DBS electrode. Pathologic evaluation and systemic workup confirmed a diagnosis of primary CNS diffuse large B-cell lymphoma. MYC and BCL2 rearrangements were identified. To our knowledge, this is the first reported case of a CNS lymphoma associated with a DBS electrode.

Overview

  • The case study reports a rare case of primary central nervous system (CNS) diffuse large B-cell lymphoma with MYC and BCL2 rearrangements developing around an electrode of a deep brain stimulation (DBS) device.
  • The patient was a 70-year-old woman with drug-resistant essential tremor who underwent bilateral thalamic DBS and showed significant symptomatic improvement over the following year.
  • The patient presented with recurrent tremor, blurred vision, and confusion, and imaging revealed a 4-cm heterogeneously enhancing lesion centered around the DBS electrode.

Comparative Analysis & Findings

  • The case study presents a unique occurrence of a CNS lymphoma associated with a DBS electrode, with no reported cases in the literature.
  • The patient's initial presentation with recurrent tremor, blurred vision, and confusion was attributed to the lymphoma and not the DBS device itself.
  • The diagnosis was confirmed through pathologic evaluation and systemic workup, revealing MYC and BCL2 rearrangements in the lymphoma tissue.

Implications and Future Directions

  • The case highlights the importance of monitoring patients with DBS devices for potential complications, including the development of CNS lymphoma.
  • Future research should focus on understanding the relationship between DBS devices and the development of CNS lymphoma, as well as identifying potential risk factors.
  • The findings of this case emphasize the need for careful patient selection and follow-up for patients undergoing DBS therapy, particularly those with pre-existing conditions that may increase their risk of lymphoma development.