title: High frequency of neurolymphomatosis as a relapse disease of intravascular large B-cell lymphoma.
abstract: 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), (18) F-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.
authors: Division of Hematology/Oncology, Department of Medicine, Kameda Medical Center, Kamogawa-shi, Chiba, Japan. kmatsue@kameda.jp
authors: Kosei
authors: K
authors: Matsue
authors: Brian Y
authors: BY
authors: Hayama
authors: Kan-Ichi
authors: K
authors: Iwama
authors: Takafumi
authors: T
authors: Koyama
authors: Hideaki
authors: H
authors: Fujiwara
authors: Masayuki
authors: M
authors: Yamakura
authors: Masami
authors: M
authors: Takeuchi
authors: Toshihiro
authors: T
authors: O'uchi
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000818:Animals; D003391:Cranial Nerves / Q000473:pathology; D005260:Female; D006801:Humans; D016403:Lymphoma, Large B-Cell, Diffuse / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008380:Marek Disease / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D008499:Medical Records; D009367:Neoplasm Staging; D010525:Peripheral Nerves / Q000473:pathology*; D049268:Positron-Emission Tomography; D011379:Prognosis; D015996:Survival Rate; D014057:Tomography, X-Ray Computed; D019043:Vascular Neoplasms / Q000150:complications* / Q000188:drug therapy / Q000473:pathology