Neurofibromas are benign (non-cancerous) tumours that grow on nerves and cause bumps on or under the skin. They can grow on any nerve in the body, the biggest or smallest, and are found on most patients with NF1.<\/p>\n\n\n\n
WHAT TYPES ARE THERE?<\/strong><\/p>\n\n\n\n There are two types of neurofibromas: cutaneous and plexiform ones. Cutaneous ones are the more common type that develop over time. Both are benign tumours that develop on nerves or under the skin.<\/p>\n\n\n\n WHAT ARE PLEXIFORM NEUROFIBROMAS?<\/strong><\/p>\n\n\n\n A plexiform neurofibroma is a tumour that forms in the tissue covering and protecting the nerves. Plexiform neurofibromas can occur anywhere in the body, outside of the brain and spinal cord. They can develop on the face (including around the eye), neck, arms, legs, back, chest, abdomen, and internal organs. Plexiform neurofibromas can also affect the appearance of the skin (darker skin colour, unusual hair growth in the affected area, thicker skin). Large tumours can affect the structure of a nearby bone, skin, and muscle. Plexiform neurofibromas can cause severe pain, mobility problems, vision and hearing loss, or obstruction (a handicap). Plexiform neurofibromas are usually not malignant (or, in simpler words, cancerous), but some may turn into malignant peripheral nerve sheath tumour (MPNST). Some patients with NF1 are at higher risk of developing MPNSTs. That is why genetic testing for NF1 and counselling is useful.<\/p>\n\n\n\n About 30 to 50% of NF1 patients develop plexiform neurofibromas. They start developing from a young age and some even have them from birth. They can often disfigure a person. However, neurofibromas can be surgically removed if they were discovered early on. If the surgeon succeeds to fully cut the tissue out, in most of the cases the Plexiform Neurofibroma will completely go away. If the surgeon can only remove a small part of the tumour, then it is very likely that the plexiform neurofibroma will grow back to its original form. <\/p>\n\n\n\n What\u2019s interesting and a glimmer of hope is a new medication that is being tested which reduces the size and intensity of plexiform neurofibromas. This happens through MEK inhibitors. In 2020, the medication was approved by the Food and Drug Administration. The drug, that comes in form of a pill, is for children from the age of 2 years on and has to be taken orally twice a day. The US National Cancer Institute found that in 70% of children the medication helped to reduce the plexiform neurofibromas from 20 to 60% in size. However, the medication is still in its testing phase and has shown some side effects like abdominal pain, nausea, fever, headaches that need to be further investigated.<\/p>\n","protected":false},"excerpt":{"rendered":" The rare genetic disease Neurofibromatosis (NF) comes in all shapes and forms and progresses differently on everybody. Some affected people experience multiple symptoms while others only have a specific one. But every symptom is valid, therefore NFPU will look more closely at a different symptom in every edition. This month it\u2019s plexiform neurofibromas. WHAT IS A NEUROFIBROMA? Neurofibromas are benign (non-cancerous) tumours that grow on nerves and cause bumps on or under the skin. They can grow on any nerve in the body, the biggest or smallest, and are found on most patients with NF1. WHAT TYPES ARE THERE? There\u2026<\/p>\n","protected":false},"author":1,"featured_media":6883,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"advgb_blocks_editor_width":"","advgb_blocks_columns_visual_guide":""},"categories":[1],"tags":[],"acf":[],"author_meta":{"display_name":"magazineadmin","author_link":"https:\/\/www.nf-patients.eu\/magazine\/author\/magazineadmin\/"},"featured_img":"https:\/\/www.nf-patients.eu\/magazine\/wp-content\/uploads\/2023\/10\/Megan_Plexiform_Neurofibroma-edited-300x200.jpg","coauthors":[],"tax_additional":{"categories":{"linked":["Uncategorized<\/a>"],"unlinked":["Uncategorized<\/span>"]}},"comment_count":"0","relative_dates":{"created":"Posted 3 years ago","modified":"Updated 2 years ago"},"absolute_dates":{"created":"Posted on 26\/09\/2023","modified":"Updated on 12\/10\/2023"},"absolute_dates_time":{"created":"Posted on 26\/09\/2023 14:30","modified":"Updated on 12\/10\/2023 15:49"},"featured_img_caption":"","series_order":"","_links":{"self":[{"href":"https:\/\/www.nf-patients.eu\/magazine\/wp-json\/wp\/v2\/posts\/6703"}],"collection":[{"href":"https:\/\/www.nf-patients.eu\/magazine\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.nf-patients.eu\/magazine\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.nf-patients.eu\/magazine\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/www.nf-patients.eu\/magazine\/wp-json\/wp\/v2\/comments?post=6703"}],"version-history":[{"count":21,"href":"https:\/\/www.nf-patients.eu\/magazine\/wp-json\/wp\/v2\/posts\/6703\/revisions"}],"predecessor-version":[{"id":7107,"href":"https:\/\/www.nf-patients.eu\/magazine\/wp-json\/wp\/v2\/posts\/6703\/revisions\/7107"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/www.nf-patients.eu\/magazine\/wp-json\/wp\/v2\/media\/6883"}],"wp:attachment":[{"href":"https:\/\/www.nf-patients.eu\/magazine\/wp-json\/wp\/v2\/media?parent=6703"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.nf-patients.eu\/magazine\/wp-json\/wp\/v2\/categories?post=6703"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.nf-patients.eu\/magazine\/wp-json\/wp\/v2\/tags?post=6703"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}![\"Megan](\"https:\/\/www.nf-patients.eu\/magazine\/wp-content\/uploads\/2023\/09\/Megan_Plexiform_Neurofibroma-scaled.jpg\")
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