Neurofibroma is a tumor formed from the multiplication of neural or perineural tissue. It is a benign tumor commonly presenting on the skin in the form of nodule or bump. It is soft in consistency and can be compressed by fingers. The reason for the soft consistency of the neurofibroma is the absence of the collagen in the dermis layer of the skin. On compression, there is no resistance felt under the tumor which is identified as analogous to a buttonhole hence neurofibromas are sometimes referred to as buttonhole sign.
Neurofibromatosis is a hereditary syndrome occurring due to genetic defects and presents as cutaneous, nervous, and skeletal system abnormalities. Neurofibromas can develop in this syndrome but are not mandatory to be found in it. There are other cutaneous features in neurofibromatosis like cafe au lait macules, axillary freckling, hyperpigmented patches[1], etc. The cafe au lait macules are characteristic of the neurofibromatosis syndrome. These macules can have a smooth border or rough border. Smooth bordered macules are known as the coast of California whereas roughly bordered macules are known as the coast of Maine.
The nervous system involvements include various tumors like optic glioma, neurofibromas, vestibular schwannomas, etc. Optic glioma and neurofibromas are commonly associated with neurofibromatosis type 1 whereas vestibular schwannomas are commonly associated with neurofibromatosis type 2. Spinal cord tumors are also associated in a few cases where they are referred to as dumbbell-shaped tumors because of their specific shape seen[2].
Skeletal system abnormalities found in neurofibromatosis are osteoporosis, scoliosis, multiple fractures, osteopenia, bone dysplasias, pathological joint fusions, etc. The commonly affected bones are the long bones like femur, humerus, tibia toetc.
Since neurofibromatosis is a collagen disorder there are various vascular abnormalities also associated with it. Vascular stenosis and hypertension are seen systematically in cases of neurofibromatosis. These may also be the presenting features of it as a clinical case which could be diagnosed after complete body examination.
Difference Between Neurofibroma And Neurofibromatosis
Usually, the neurofibromas are of benign origin with minimal symptoms and occur singularly without any correlation with other systemic symptoms whereas neurofibromatosis occurs with a group of symptoms one of which can be the formation of neurofibromas. These neurofibromas occur at various sites haphazardly and are multiple in numbers associated with other cutaneous symptoms like hyperpigmented macules etc.
Neurofibromas, when occur separately without any syndromic association, are easy to treat with no malignant transformation seen among them in maximum cases. But in cases associated with neurofibromatosis, the neurofibromas can undergo malignant change or can involve multiple nerves or nerve bundles to form plexiform neurofibroma which is difficult to treat and is also having a detrimental effect on the quality of life.
The loss of the skin sensations, autonomic symptoms etc. is minimal with the neurofibromas whereas these problems are increased in cases of syndromic association with neurofibromatosis. Neurofibromatosis is also associated with hypertension symptoms due to vessel involvement which is not seen in the case of neurofibroma.
Neurofibroma alone can be treated via laser treatment easily without complications whereas neurofibromatosis has its predominant treatment as in form of surgical treatment although in many cases of neurofibromas also, the surgical resection is done in absence of laser therapy. The surgical treatment of the neurofibroma leads to less scarring and no recurrence in singular neurofibroma tumor. The surgical resection is more leading to more scarring and high recurrence rate in neurofibromatosis associated tumors.
Conclusion
Neurofibromas are a nerve involving tumor which could occur as a single identity or can also occur in association with other symptoms in hereditary disorders like neurofibromatosis. Neurofibroma, being a benign tumor is a less dangerous and treatable disease whereas neurofibromatosis is more dangerous because of the possibility of malignancy as well as multiple organ and organ system involvement in it. As these two disease identities can create confusion amongst them, therefore complete demarcation is necessary between the case of neurofibroma and neurofibromatosis to start appropriate treatment at the earliest for the same.
Also Read:
- What Is The Best Way To Get Rid Of Neurofibromas?
- What Leads To Neurofibromas & Can They Be Cured?
- Best Exercises & Activities For Neurofibroma Patients
- How Common Is Neurofibroma or Is It A Rare Disease?
- Is There A Surgery For Neurofibroma & What Is The Life Expectancy With It?
- What Are The Different Types Of Neurofibromas?
- What Is Myxoid Neurofibroma & What Are Its Symptoms, Treatment Procedures?
