Tumors develop on nerve tissue as a result of a series of hereditary diseases called von recklinghausen's disease. Anywhere in the neurological system, including the brain, spinal cord, and nerves, these tumors can form. Von recklinghausen's disease comes in three different forms: schwannomatosis, neurofibromatosis 1 (NF1), and neurofibromatosis 2 (NF2). While NF2 and schwannomatosis are typically detected in early adulthood, NF1 is typically diagnosed in childhood.
Risk factors of von recklinghausen's disease
These are the risk factors -
1. A family history of von recklinghausen's disease is the main risk factor for the condition. About half of those with NF1 and NF2 received the condition from a parent who had it. People with NF1 and NF2 who do not have affected relatives are more likely to have a novel gene mutation.
2. Both NF1 and NF2 are autosomal dominant diseases, which means that there is a 50% probability that the genetic mutation will be passed down to any offspring of an affected parent.
3. It is unclear how von recklinghausen's disease is inherited. According to current research, there is a 15% chance of inheriting schwannomatosis from a parent who has the condition
Management of the von recklinghausen's disease
For the management of the condition, routine monitoring is crucial. All patients are advised to have annual physical exams, as well as regular eye tests and blood pressure checks. Annual ophthalmologic examinations, routine measurements of head circumference, and developmental evaluations are all advised for kids with NF1. Depending on the location and symptoms, advanced imaging (MRI, PET, or PET/CT) may be indicated to monitor internal tumors. The use of whole-body MRI as a technique for tracking internal cancers is being researched. For females with NF1, annual breast cancer screening (either by mammography or breast MRI) is advised to start at age 30.
Six or more café-au-lait spots of at least 5 millimeters [mm] in size (before puberty) or 15 mm in size (after puberty),Freckling in the underarms (axillary) or groin (inguinal) regions,Two or more neurofibromas of any type or one plexiform neurofibroma,Optic pathway glioma
Increased risk of developing noncancerous (benign) and cancerous (malignant) tumors
Neurontin, Gralise, Horizant) or pregabalin (Lyrica),Tricyclic antidepressants such as amitriptyline