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High frequency of neurolymphomatosis as a relapse disease of intravascular large B-cell lymphoma
Version of Record online: 29 MAR 2011
Copyright © 2011 American Cancer Society
Volume 117, Issue 19, pages 4512–4521, 1 October 2011
How to Cite
Matsue, K., Hayama, B. Y., Iwama, K.-I., Koyama, T., Fujiwara, H., Yamakura, M., Takeuchi, M. and O'uchi, T. (2011), High frequency of neurolymphomatosis as a relapse disease of intravascular large B-cell lymphoma. Cancer, 117: 4512–4521. doi: 10.1002/cncr.26090
- Issue online: 16 SEP 2011
- Version of Record online: 29 MAR 2011
- Manuscript Accepted: 7 FEB 2011
- Manuscript Revised: 21 JAN 2011
- Manuscript Received: 29 SEP 2010
- intravascular large B-cell lymphoma;
- 18F-fluoro-2-deoxy-D-glucose-positron emission tomography/computed tomography;
- magnetic resonance imaging;
- high-dose methotrexate
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.
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.
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.
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. Cancer 2011. © 2011 American Cancer Society