Neurofibromatosis Tumors

Neurofibromatosis (NF), which is a combination of three genetic diseases; NF1, NF2 and Schwannomatosis all result in the slow growth of typically Benign, non-Cancerous tumors, that can start to grow typically at birth, even if none are symptomatic till years later. While each type of NF results in different nerve types and formations, the commonality between NF types is the result of nerve damage from tumor growth. Individuals with NF1 and NF2 tumors can become Malignant, Cancerous.

Neurofibroma like Schwannoma grow attached to nerves, but in a different way. Cutaneous tumors are tumors that grow over the skin and but generally are not painful. Subcutaneous are tumors that grow anywhere under the skin and generally are painful.

1. NF Tumors by NF Type

While there can be more, the following are typical tumor types for each forms of NF.

Neurofibromatosis Type 1

NF1 results in Neurofibroma, Optic Glioma and Lisch nodules

1. Skin Surface: Cutaneous Neurofibroma and Subcutaneous Neurofibroma
2. Peripheral Nerves Neurofibroma: These are Plexiform Neurofibroma, tumors on nerves deep in the body

Neurofibromatosis Type 2

The majority of NF2 tumors are within the Central Nervous System; brain and Spinal cord. NF2 results in Schwannoma, Meningioma, Ependymoma and Astrocytoma

1. Bilateral Vestibular Schwannoma (VS), sometimes referred to as Acoustic Neuroma (AN):
   Schwannoma growth on both, the left and right, Vestibular Nerve
2. Additional Brain Tumors: Schwannoma, Meningioma and Glioma (Astrocytoma or Ependymoma)
• Schwannoma on Cranial Nerves:
  Schwannoma growth in the brain is rarely limited to just the Vestibular Nerves
• Intracranial Meningioma:
  Meningioma can easily develop throughout the brain.
• Cerebellum (lower brain):
  Glioma (Astrocytoma or Ependymoma)
3. Spine Cord Tumors: Schwannoma, Meningioma and Glioma (Astrocytoma or Ependymoma)
4. Peripheral Nerve Schwannoma:
   Schwannoma on nerves outside of the brain and spine which can only be seen with MRI.
5. Skin Surface: Cutaneous Schwannoma (also called Dermal Schwannoma) and Subcutaneous Schwannoma

Schwannomatosis

Schwannomatosis results in Schwannoma - Schwannomatosis tumors are primarily:

• Peripheral Nerves: Schwannoma, tumors on nerves deep in the body
• Skin Surface: Cutaneous Schwannoma and Subcutaneous Neurofibroma

2. Reference Sources

Plotkin, S. R., Bredella, M. A., Cai, W., Kassarjian, A., Harris, G. J., Esparza, S., ... & Mautner, V. F. (2012). Quantitative assessment of whole-body tumor burden in adult patients with neurofibromatosis. PloS one, 7(4), e35711. http://dx.plos.org/10.1371/journal.pone.0035711