Neurofibromatosis Type 2 and Other Bilateral Cerebellopontine Angle Tumors

  • Madjid Samii
  • Venelin Gerganov


Neurofibromatosis type 2 is a dominantly inherited tumor predisposition syndrome characterized by a tendency for developing bilateral VSs and other CNS tumors (meningiomas, ependymomas, astrocytomas, or other schwannomas). Typically the syndrome manifests itself during young adulthood—in the second or third decade of life. The severity of the disease ranges from mild to aggressive in regard to age of onset, tumor types, and biological aggressiveness. The treatment of patients with bilateral VSs is different from that of patients with sporadic unilateral VSs. The lifelong tendency to formation of new CNS tumors predetermines the impossibility of definitive cure of NF-2 patients. Therefore, management is focused on life prolongation, preservation or rehabilitation of cranial nerve function, and maintenance of quality of life. The treatment should be individualized and performed in a specialized treatment center in close collaboration with the patient and his family. The patient’s clinical presentation, attitude, and expectations, as well as the tumor characteristics, should guide the decision-making process. The tumor size and the function of the cochlear nerves on both sides are issues of paramount importance. Other bilateral CP angle lesions have been rarely reported: choroid plexus papillomas, endolymphatic sac tumors, primary central nervous system lymphoma, arachnoid cysts, osteomas, or metastases.


Facial Nerve Cochlear Implant Inferior Colliculus Primary Central Nervous System Lymphoma Cochlear Nucleus 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • Madjid Samii
    • 1
  • Venelin Gerganov
    • 2
  1. 1.International Neuroscience Institute INIHannoverGermany
  2. 2.Department of NeurosurgeryInternational Neuroscience InstituteHannoverGermany

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