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Special mention of tumors
D. Neurofibroma
A neurofibroma is a benign nerve sheath tumor in the peripheral nervous system. Usually found in individuals with neurofibromatosis type 1 (NF1), a genetically-inherited disease, they can result in a range of symptoms from physical disfiguration and pain to cognitive disability. Neurofibromas arise from Schwann cells in the NF1 gene that codes for the protein neurofibromin.[1] This protein is responsible for regulating the RAS-mediated cell growth pathway. In contrast to, another type of tumor arising from Schwann cells, neurofibromas incorporate many additional types of cells and structural elements in addition to Schwann cells, making it difficult to identify and understand all the mechanisms through which they originate and develop.
Neurofibromas have been subdivided into two broad categories: dermal and plexiform. Dermal neurofibromas are associated with a single peripheral nerve, while plexiform neurofibromas are associated with multiple nerve bundles. According to the World Health Organization classification system, dermal and plexiform neurofibromas are grade I tumors.
Types of Neurofibroma
Dermal Neurofibromas
Dermal Neurofibroma, sometimes referred to as cutaneous neurofibroma, typically arise in the teenage years and are often associated with the onset of puberty. They continue to increase in number and size throughout adulthood, although there are limits to how big they get. They look like lumps on or under the skin. While dermal neurofibromas can lead to stinging, itching, pain and disfiguration. They do not undergo malignant transformation.
Plexiform Neurofibromas
Plexiform neurofibromas are often congenital defects, and are the more troublesome type. They can be very large and can cause pain, disfigurement, neurological and other clinical deficits. While dermal neurofibromas originate in nerves in the skin, plexiform neurofibromas can grow from nerves in the skin or from more internal nerve bundles.
Internal plexiform neurofibromas are very difficult to remove completely because they extend through multiple layers of tissue and the attempt would damage healthy tissue or organs. Plexiform neurofibromas also have the potential to cause severe clinical complications if they occur in certain areas. [7] In addition, about 10% of plexiform neurofibromas undergo malignant transformation. This transformation turns the plexiform neurofibroma into a malignant peripheral nerve sheath tumor (MPNST).
Treatment
Surgery is the most preferred choice of treatment . Dermal neurofibromas are not removed unless they are painful or causing disfigurement.
Team of Specialists:
- Dr. Arun Saroha Head - Neurosurgery (Unit I) View Profile
- Dr. Alok Gupta Head - Neurosurgery (Unit II) View Profile
- Dr. Praveen Gupta Head - Neurology View Profile