High frequency of neurolymphomatosis as a relapse disease of intravascular large B-cell lymphoma

Kosei Matsue, Brian Y. Hayama, Kan Ichi Iwama, Takafumi Koyama, Hideaki Fujiwara, Masayuki Yamakura, Masami Takeuchi, Toshihiro O'Uchi

Research output: Contribution to journalArticlepeer-review

37 Citations (Scopus)


BACKGROUND: Intravascular large B-cell lymphoma (IVL) is characterized by lymphoma cell proliferation in the lumina of small vessels in various organs. A high incidence of neurologic symptoms associated with the central nervous system has been reported, but peripheral nerve involvement (neurolymphomatosis [NL) rarely has been described. METHODS: The medical records from patients who were diagnosed with IVL over the past 4 years were reviewed. A diagnosis of NL was made based on the combination of neurologic symptoms and their correspondence with imaging studies, such as magnetic resonance imaging (MRI), 18F-fluoro-2-deoxy-D-glucose (FDG)-positron emission tomography/computed tomography (PET/CT), and/or the histologic confirmation of lymphoma cells within the peripheral nerves, nerve root/plexuses, or cranial nerves. RESULTS: Four patients with NL were identified among 11 patients who had IVL. All cases of NL occurred as relapsed disease during or shortly after the completion of chemotherapy. Although MRI studies of the brains and whole spines revealed nerve infiltration by gadolinium enhancement in 2 patients, the technology was not sensitive enough to detect such infiltration in the remaining 2 patients. In contrast, FDG-PET/CT studies successfully revealed cranial or peripheral nerve lesions in all 4 patients and was useful for evaluating therapeutic response. Patients received treatment with high-dose methotrexate with or without other systemic chemotherapy, which achieved varied success. Further studies will be needed to determine the optimal treatment. CONCLUSIONS: Considering the rarity of IVL and NL, the current observations suggested that IVL may have a predilection not only for the vessels but also for both the central and peripheral nervous systems.

Original languageEnglish
Pages (from-to)4512-4521
Number of pages10
Issue number19
Publication statusPublished - Oct 1 2011
Externally publishedYes


  • F- fluoro-2-deoxy-D-glucose-positron emission tomography/computed tomography
  • high-dose methotrexate
  • intravascular large B-cell lymphoma
  • magnetic resonance imaging
  • neurolymphomatosis

ASJC Scopus subject areas

  • Oncology
  • Cancer Research


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