Neurofibroma is a subtype of peripheral nerve sheath tumors, in addition to schwannomas and malignant peripheral nerve sheath tumors. Neurofibromas and schwannomas are the benign varieties of peripheral nerve sheath tumors and are by far the most commonly encountered tumors. Neurofibromas are painless, slow-growing soft tissues tumors arising from adjoining nerves. Neurofibroma is further divided into localized, diffuse and plexiform varieties. Neurofibromas are mostly observed in children or young adults.(1)
Coping With Plexiform Neurofibroma In Type 1 Neurofibromatosis
Plexiform neurofibromas are benign peripheral nerve sheath tumors growing along a nerve that is commonly observed in neurofibromatosis type 1. The manifestation of plexiform neurofibromas in type 1 neurofibromatosis can result in severe chronic pain. Plexiform neurofibromas can lead to nerve, airway and spinal cord compression.
Furthermore, it can also cause leg length discrepancies and scoliosis. The pain associated with plexiform neurofibroma has a tendency to grow rapidly during childhood and it can also occur after the removal of the tumor. Despite pain management with pain medications, the pain may still be prevalent. The pain, functional disability, as well as aesthetic concerns related to plexiform neurofibroma, can have significant physiological, psychological and social impact. These patients may even suffer from anxiety, depression, social stress, and other social-emotional functioning. This may reduce the overall quality of life of a patient suffering from type 1 neurofibromatosis along with cutaneous manifestations, such as plexiform neurofibroma.(3)
These patients require over the counter as well as prescribed pain medications, medications to deal with anxiety and depression, psychological counseling as well as multidisciplinary therapies to lead a normal life that improves their quality of life.(3)
Localized neurofibroma is the most common subtype accounting for approximately 90% of all neurofibromas. In contrast to diffuse and plexiform subtype, localized neurofibroma is not associated with type 1 neurofibromatosis. Localized neurofibroma mostly accounts for approximately 5% of all benign soft tissue tumors and is observed in young adults between the ages 20-30 years. Most of these lesions are superficial and well-defined lesions originating from smaller nerves; deeper lesions are rare, which mostly arise from larger nerves.(1)
Plexiform neurofibroma is a rare form of neurofibroma but is most commonly associated with type 1 neurofibromatosis. It is most commonly seen in early childhood. It is also associated with malignant peripheral nerve sheath tumors and lifetime risk of malignant transformation is about 8-12%. In contrast to localized neurofibroma, which is well defined, plexiform neurofibroma can expand and distort a large segment of nerve and its branches that creates a ‘bag of worms’ appearance. The nerve expansion not only involves the nerve but with a growing tumor, the surrounding soft tissue is also affected. When it involves a limb, it may induce elephantiasis neuromatosa that is associated with enlargement of the affected extremity along with bone hypertrophy and redundant skin.(1)
Diagnosis And Management Of Neurofibroma
A neurofibroma usually has no known cause, but on occasions, it may be associated with a genetic condition known as type 1 neurofibromatosis. The diagnosis of neurofibroma is based on clinical examination, medical history and imaging (CT scan or MRI). A PET scan or biopsy may be done to know whether it is benign.(2)
Since, neurofibroma is a benign lesion, a solitary lesion less than 2 cm does not require any treatment. If the tumor does not cause any problem and is very small without showing any signs of growth, just monitoring is enough. Surgical intervention is required when the lesion becomes symptomatic in cases where it is pressing or damaging nearby organs. The type of surgery depends on the location and size of the tumor. However, the objective of the surgery is to extirpate as much tumor as possible without causing any further nerve damage. Physical rehabilitation may be required after surgery to keep muscles and joints active, to prevent stiffness and to restore the function of the affected body extremity.(2)
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