What Are The Different Types Of Neurofibromas?

Neurofibroma is the tumor growth in the connective tissue of the nerve sheath. Neurofibromas may be divided into various types based on their characteristics.

Diffuse Neurofibromas: A Diffuse neurofibroma is an uncommon form of neurofibroma. The tumor occurs in the trunk, head and neck region². Diffuse neurofibroma most occur in children and young adults.

Plexiform Neurofibromas: Plexiform neurofibroma is a type of neurofibromas which is made up of same cells of as dermal or cutaneous neurofibromas. It occurs in various parts of the body such as head, neck, around the spine areas and deep nerve in the body. This tumor grows slowly and produces pain. Serious brain dysfunction and impingement on other organs are complications of this tumor. Although the condition occurs deep into the tissue however it may occur on superficial nerves. Plexiform neurofibroma is divided in to diffuse plexiform and nodular plexiform neurofibroma.

Cutaneous Neurofibromas: Cutaneous neurofibromas are also called as dermal or discrete neurofibromas. This tumor originates from the small nerves in the skin and formed bumps or lumps on the skin⁴. Cutaneous neurofibroma is not removed through surgery. CO2 laser technique is used to remove this type of neurofibromas tumor.

The number of cutaneous neurofibromas varies in different patients. Some patients have very few while some had a large number of tumors. In case of a large number of tumors in cutaneous neurofibroma, the condition is generally linked to genetics.

Intramuscular Neurofibromas: This neurofibroma occurs on muscles and is present on small nerves. They can be removed but surgery may leave a scar.

Neurofibroma is a benign tumor of the peripheral nerve sheath. It arises from the proliferation of nerve sheath cells. Neurofibroma is made of nerve fibers, Schwann cells (cells that cover the nerve fibers), blood vessels, inflammatory white blood cells (mast cells), and fibroblasts (connective tissue)¹. It forms short bumps on or under the skin. Neurofibroma affects both men and women.

The symptoms of neurofibroma depend upon the size and location of the tumor. Following symptoms are experienced by the patient suffering from neurofibroma:

• Neurofibroma type 1(NF-1) produce skin discoloration
• Neurofibroma type-2(NF-2) may affect hearing
• Hearing loss or ringing in ears
• Soft and pea-size bump on or under the skin
• Sudden swelling due to trauma or injury of blood vessels associated with neurofibroma.

Various treatment strategies are implemented by a neurosurgeon for treating neurofibroma. Apart from removing the tumor, various other medications are also prescribed to manage the symptoms of neurofibroma. Like surgical procedures for other diseases, surgery in neurofibroma is also associated with various complications and side effects. Thus, it is best to avoid surgery if it is not essential. Monitoring through periodic evaluation is a common strategy implemented in cases where no severe symptoms experienced by the patient. Neuroblastoma can be treated with surgery or monitoring.

Surgical Procedure: Surgery is required in cases of neurofibroma where the patient experiences discomfort and tumor affect their daily life. The tumor of neurofibroma is removed by the surgery but in some cases, tumors are not fully removed and radiation therapy is used to relieve the pressure on the spinal cord³. An alternative surgical technique such as stereotactic spine surgery is also used to destroy the tumor. In this technique, a highly focused beam of radiation is used to destroy the tumor. The advantages of this surgery are:

• Minimal healthy tissue damage around the tumor
• Lower risk of side effects
• Precise surgical technique

Monitoring: Monitoring is the preliminary strategy implemented by neurologists in cases where there is no immediate threat to the life of the patient or when the quality of life is not significantly reduced because of the tumor. Periodic evaluation of the growth of the tumor is required during monitoring.

Various types of neurofibroma are cutaneous neurofibroma, plexiform neurofibroma, intramuscular neurofibroma, and diffuse neurofibroma. Plexiform neurofibroma is further subdivided into diffuse plexiform and nodular neurofibroma.

References:  

Also Read:

• Neurofibromatosis: Causes, Symptoms, Diagnosis, Treatment