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Arch Otolaryngol Head Neck Surg. 2007;133(3):304-305.

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Diagnosis: Plexiform neurofibroma

Plexiform neurofibromas are benign, slow-growing tumors that are found almost exclusively in patients with NF1. Neurofibromatosis 1 is an autosomal dominant condition that is caused by a defect on chromosome 17, affecting approximately 1 in 4000 births. It is characterized by the presence of numerous cutaneous or subcutaneous neurofibromas, optic gliomas, and café au lait spots. Approximately 17% to 33% of individuals with NF1 present with plexiform neurofibromas.^1-2

Neurofibromas can be of a plexiform or cutaneous type. While cutaneous lesions appear during preadolescence, plexiform neurofibromas are typically congenital. The majority of plexiform neurofibromas are found in the trunk, extremities, and head and neck area, with approximately 50% found in the latter. They are capable of developing anywhere along the length of a peripheral nerve and are often locally invasive. Muscle, bone, fascia, and internal organs may become involved.^{1, 3} Head and neck plexiform neurofibromas may cause disfigurement, compromise the airway, or . . . [Full Text of this Article]

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Arch Otolaryngol Head Neck Surg. 2007;133(3):302.
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