Management of multiple tumors in neurofibromatosis type 2 patients

Neurochirurgie. 2018 Nov;64(5):364-369. doi: 10.1016/j.neuchi.2014.11.012. Epub 2015 Jun 10.


Introduction: Neurofibromatosis type 2 is characterized by the presence of bilateral vestibular schwannomas. However, other nervous system tumors may also occur. Therefore, the management of NF2 patients is complex and requires a multidisciplinary discussion in a specialized center.

Materials and methods: All recent articles concerning tumors other than vestibular schwannoma in NF2 disease were reviewed, using PubMed databases.

Results: Intracranial meningiomas occur in 50% of NF2 patients, and are often multiple. Surgery remains the main treatment and should be performed in cases of growing tumors. The role of antiangiogenic therapy is currently under evaluation and the role of radiosurgery still remains to be defined in NF2 disease. Spinal tumors occur in about half of NF2 patients. Surgery should be discussed when radiological tumor progression is demonstrated, even if spinal tumors are asymptomatic, in order to preserve neurological function and good quality of life. As regards lower cranial nerve schwannomas, radiosurgery appears to be a more appropriate treatment for growing tumor with a small volume in order to avoid post-operative complications, especially swallowing disorders. Facial nerve schwannomas may appear, on MRI, like vestibular schwannomas. The diagnosis should be suspected when the facial palsy is an early symptom during cerebello-pontine tumor progression. Trigeminal schwannomas are frequent in NF2 disease and fortunately they are often asymptomatic. Among major neurofibromatosis types, peripheral nerve sheath schwannomas are only present in patients with NF2 disease and schwannomatosis. Surgical resection is required when the cutaneous schwannomas is painful or when tumor progression is observed and causes symptoms.

Conclusion: Tumors other than vestibular schwannoma are also associated with a poor prognosis in NF2 patients. Surgery remains the main treatment in most cases. Each treatment decision in NF2 disease requires a complete evaluation of all cranial and spinal locations of the disease in order to establish surgical priorities and strategies.

Keywords: Ependymoma; Meningioma; Méningiome; NF2; Schwannoma; Schwannome; Spinal tumor; Tumeur spinale; Épendymome.

MeSH terms

  • Disease Progression
  • Humans
  • Magnetic Resonance Imaging / methods
  • Meningioma / diagnosis
  • Meningioma / surgery
  • Neurilemmoma / diagnosis
  • Neurilemmoma / surgery
  • Neurofibromatoses / diagnosis
  • Neurofibromatoses / surgery*
  • Neurofibromatosis 2 / diagnosis
  • Neurofibromatosis 2 / pathology*
  • Neurofibromatosis 2 / surgery*
  • Postoperative Complications / surgery
  • Radiosurgery / methods
  • Skin Neoplasms / diagnosis
  • Skin Neoplasms / surgery
  • Spinal Neoplasms / surgery*
  • Treatment Outcome

Supplementary concepts

  • Schwannomatosis