Neurological Sciences

, Volume 33, Issue 6, pp 1429–1433 | Cite as

Central nervous system lymphoma occurring in a patient with neurofibromatosis type 1 (von Recklinghausen disease)

  • Marica EoliEmail author
  • Donata Bianchessi
  • Anna Luisa Di Stefano
  • Elena Prodi
  • Elena Anghileri
  • Gaetano Finocchiaro
Case Report


Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder, which confers an increased risk of a wide range of cancers, and malignant tumors are the most common cause of death in individuals with NF1. Although in children with NF1, the most common neoplasms are optic nerve gliomas and brain tumors, an elevated risk of myeloid leukemia and an increased relative risk of acute lymphoblastic leukemia and non-Hodgkin lymphoma were reported. In adults with NF1, the relative risk of brain tumor is 100 times higher than in the general population. Cases of malignant lymphoma occurring in NF1 adult patients have been reported. However, the association between NF1 and lymphoproliferative diseases is still debated. We report a case of CNS primitive lymphoma in an adult patient who resulted positive for NF1 at genetic testing. At present, only one case of CNS lymphoma in an adult patient displaying clinical criteria for NF1 diagnosis has been reported.


Neurofibromatosis 1 Central nervous system lymphoma 



This report was supported by grant RS18 of the Istituto Superiore di Sanità to GF. We thank Dr. B. Pollo for sharing information on patient histology and Maria Cristina Ibba for helping in genetic analysis.


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Copyright information

© Springer-Verlag 2012

Authors and Affiliations

  • Marica Eoli
    • 1
    Email author
  • Donata Bianchessi
    • 2
  • Anna Luisa Di Stefano
    • 2
  • Elena Prodi
    • 2
  • Elena Anghileri
    • 2
  • Gaetano Finocchiaro
    • 2
  1. 1.Fondazione I.R.C.C.S. Istituto Neurologico C. Besta, Unit of Molecular Neuro-OncologyMilanItaly
  2. 2.Fondazione I.R.C.C.S. Istituto Neurologico Nazionale C. MondinoPaviaItaly

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