What are plexiform neurofibromas?
(PLEK-sih-form NOOR-oh-fy-BROH-muh) A tumor that forms in the tissue that covers and protects the nerves. Plexiform neurofibromas can occur anywhere in the body outside of the brain and spinal cord. They can occur on the face (including around the eye), neck, arms, legs, back, chest, abdomen, and internal organs.
What is multiple neurofibroma?
Neurofibromatoses are a group of genetic disorders that cause tumors to form on nerve tissue. These tumors can develop anywhere in the nervous system, including the brain, spinal cord and nerves. There are three types of neurofibromatosis: neurofibromatosis 1 (NF1), neurofibromatosis 2 (NF2) and schwannomatosis.
How is plexiform neurofibroma diagnosed?
How are plexiform neurofibromas diagnosed? Although diagnosis of these lesions is usually possible by examination of the child, the full extent of the lesion is best seen by MRI scan. MRI scans may show these lesions to be much more extensive than previously thought.
Can plexiform neurofibromas be removed?
If cancerous tumors develop with neurofibromatosis—for example, malignant plexiform neurofibromas, which can develop in the arms, legs, or trunk—they can also be surgically removed.
Which is seen in schwannoma?
A schwannoma is a type of nerve tumor of the nerve sheath. It’s the most common type of benign peripheral nerve tumor in adults. It can occur anywhere in your body, at any age. A schwannoma typically comes from a single bundle (fascicle) within the main nerve and displaces the rest of the nerve.
Do neurofibromas stop growing?
Neurofibromas often start to show up during puberty. They may keep getting bigger for a while but will eventually stop growing. Typically, people gradually develop new ones as they get older.
What kind of neurofibroma is plexiform type 1?
Plexiform neurofibromas represent an uncommon variant (30%) of neurofibromatosis type 1 (NF-1) in which neurofibromas arise from multiple nerves as bulging and deforming masses involving also connective tissue and skin folds.
Can a person with NF1 have multiple neurofibromas?
Neurofibromas may occur singly in genetically normal people at any point along the peripheral nervous system. Multiple neurofibromas are nearly pathognomonic for neurofibromatosis 1 (NF1). In patients with NF1, neurofibromas may be congenital and plexiform or, more commonly, may be smaller masses that begin to accumulate around the time of puberty.
What kind of tumor is a plexiform tumor?
Plex- originates from the Latin verb plectere meaning “to plait” or “interweave”. It is typically associated with tumors of neural derivation. Plexiform neurofibromas are considered the prototype of the plexiform pattern 1. 1. Abbas O, Bhawan J. Cutaneous plexiform lesions.
When do neurofibromas begin to accumulate in patients?
Multiple neurofibromas are nearly pathognomonic for neurofibromatosis 1 (NF1). In patients with NF1, neurofibromas may be congenital and plexiform or, more commonly, may be smaller masses that begin to accumulate around the time of puberty.