What Leads To Neurofibromas?
Neurofibromas are very common benign tumors of the nerves. The disorganization of normal nerve tissue along with the overgrowths in the nerve leads to neurofibromas. Neurofibroma consists of nerve fibers, Schwann cells, blood vessels, mast cells, connective tissue, and extracellular matrix. Neurofibromas can be classified as diffuse, cutaneous/dermal, intramuscular and plexiform subtypes.(1)
Can Neurofibromas Be Cured?
Neurofibromas are benign tumors and can be removed surgically. The decision of whether to remove a neurofibroma depends on the location, patient’s age, symptoms and esthetic distortion caused by it. Neurofibromas are only removed when they grow larger and causing problems. They can be surgically excised and caution should be maintained as to not injure the nerve attached to it. Electrodesiccation should be used to remove a large number of tumors. It is difficult to remove diffuse neurofibromas and they should be monitored until they cause problems. Removing the plexiform tumor is also difficult without compromising the nerve involved and therefore of the risks and benefits should be carefully assessed before removing the tumor. Large tumors can be shrunk by embolization. Furthermore, MEK inhibitors have promising results that decrease and shrink the size of neurofibromas and prevent them from growing.(1)
Types Of Neurofibroma
Cutaneous or dermal neurofibroma is the most common type of neurofibroma. They are the tumors that commonly occur on the skin with clearly defined borders and the excision is easy in these tumors. The number of skin tumors varies from a solitary tumor to multiple tumors. Some patients have a lot of cutaneous tumors, although, a very large number of tumors is rare, it may be present in people with family history. They usually start appearing in adolescence or young adults and are rarely seen in children. The size and number of lesions increase with increasing age.(1)
Diffuse neurofibromas involve the whole of skin from the surface to the fascia; however, they do not go deeper than the fascia. They are a relatively uncommon type of neurofibroma, when present they are mostly seen on the scalp. They can feel soft and swishy with uneven margins. They are associated with diffuse hyperpigmentation and can look like a large café au lait spot. They are mostly present in early childhood and can enlarge with growing age.(1)
Intramuscular neurofibromas occur inside the muscles. They are tumors along very small nerves and can sometimes lead to pain. Although they can be removed, they often leave a scar behind. (1)
Plexiform neurofibromas are more commonly related to type 1 neurofibromatosis. They occur along the larger nerves and are mostly internal, but they can also involve smaller nerves and can affect superficial skin too. They are mostly seen in children and they can grow with time. Plexiform neurofibroma can further be divided into diffuse and nodular type. Diffuse type can affect the skin and extend beyond the fascia to the adjoining muscles and are devoid of clear margins. Nodular type of plexiform neurofibroma often involves a large nerve, such as sciatic nerve and can enlarge or thicken the nerve and produces clusters or nodules of the tumor along the nerve.(1)
Symptoms Of Neurofibroma
Although, neurofibromas are benign tumors that are mostly asymptomatic and can grow at a really slow pace. However, they can sometimes be itchy as they involve the mast cells or they can cause pain when touched or injured. A trauma to neurofibroma can lead to increased growth and swelling of the tumor. Severe pain can be a sign of the malignant transformation of a neurofibroma. Malignant transformation is noted in about 9% of all cases of plexiform neurofibroma. The malignant transformation of a plexiform neurofibroma is usually seen in adolescence or adult life. In rare Electrodesiccation diffuse neurofibromas can also have a malignant transformation; cutaneous neurofibromas do not become malignant. Although neurofibromas are tumors of nerves, they mostly never cause weakness unless spinal nerves are involved or it pushes along the spinal cord.(1)