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Tumors: Neurofibromatosis
Also known as von Recklinghausen disease, neurofibromatosis is rarely cancerous. Neurofibromatosis grows around the nerve cells/sheaths and affects all nearby tissues: skin, the layer below the skin (subcutaneous) and bone.
Typically, neurofibromatosis is first noticed as patches of "café au lait" colored areas of skin which look like birthmarks. Then, the neurofibromatosis nodules themselves are noted. Neurofibromatosis may become evident in any of three forms: flat lesions of moderate size that follow a nerve and cause enlargement of surrounding tissues (plexiform neurofibroma), small, round nodules present in groups (mollusca fibrosa), or massive overgrowths of skin and associated soft tissues (elephantiasis neurofibromatosa). In the in the head, skull, and facial feature regions we often see deep involvement with several layers of skin, nerve and bone tissue in the eye area.
Usually the growth isn't painful, but requires treatment to avoid tissue and structure damage. The best improvement is achieved by surgically removing the growth. The tumor tissue tends to grow into and around the normal tissues so that removal is lengthy, complex and transient. Repeated surgeries will help control and eliminate further growth.
If the neurofibroma tumor grows in the face, brain, or skull region, it often pushes the eye out of its normal orbital position and infiltrates the eyelid tissue. It can extend into the bony area surrounding the eye sockets. Therefore, in addition to surgical removal of the abnormal tissues, reconstruction of bony tissue with bone grafts and bone contouring may be required.
Although a specific cause is not known, it is possible to inherit a predisposition to this condition.
