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A malignant peripheral nerve sheath tumor is rare, but is one of the most common frequent soft tissue sarcoma in the pediatric population. About half of these cases also happen to occur along with neurofibromatosis type 1 (NF-1), which is a genetic mutation on the 17th chromosome which causes tumors along the nervous system.
A neurofibroma is a benign nerve-sheath tumor in the peripheral nervous system.In 90% of cases, they are found as stand-alone tumors (solitary neurofibroma, solitary nerve sheath tumor [1] or sporadic neurofibroma [1]), while the remainder are found in persons with neurofibromatosis type I (NF1), an autosomal-dominant genetically inherited disease.
Most peripheral nerve tumors occur for unknown reasons. Some, including schwannomatosis and neurofibromatosis (types 1 and 2), are associated with recognized hereditary disorders. Others may be caused by gene mutations. In the case of schwannomatosis and neurofibromatosis, tumors can grow on or close to nerves anywhere in the body.
Each nerve sheath tumor type has a few different associated features on MRI imaging. Neurofibromas and malignant peripheral nerve sheath tumors can be difficult to distinguish from each other and may require additional testing, including PET scans (18 FDG-PET). [1] Image-guided needle biopsies may be performed if there is concern for malignancy ...
Most common of these is a vestibular schwannoma, a tumor of the vestibulocochlear nerve that may lead to tinnitus and hearing loss on the affected side. Outside the cranial nerves, schwannomas may present on the flexor surfaces of the limbs. Rare occurrences of these tumors in the penis have been documented in the literature. [5]
Neurofibromatosis arise from the supporting cells of the nervous system rather than the neurons themselves. [1] In NF1, the tumors are neurofibromas (tumors of the peripheral nerves), while in NF2 and schwannomatosis tumors of Schwann cells are more common. [1] Diagnosis is typically based on symptoms, examination, medical imaging, and biopsy.
Acoustic neuroma - a slow-growing, benign tumor of the acoustic nerve. [2] Symptoms, which most often start after the age of 30, can include dizziness, headache, vertigo, loss of balance, ringing sensations, and numbness. [3] Ganglioneuroma - a tumor of the sympathetic nerve fibers arising from neural crest cells. [4]
Granular cell tumor is a tumor that can develop on any skin or mucosal surface, but occurs on the tongue 40% of the time. It is also known as Abrikossoff's tumor, [1] granular cell myoblastoma, [1] granular cell nerve sheath tumor, [1] and granular cell schwannoma. [1] Granular cell tumors (GCTs) affect females more often than males. [2]
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