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Acoustic neuroma, [1] ... located on chromosome 22, ... the ideal treatment for symptomatic patients with vestibular schwannoma is the total excision of the tumor in ...
Neurofibromatosis type II (also known as MISME syndrome – multiple inherited schwannomas, meningiomas, and ependymomas) is a genetic condition that may be inherited or may arise spontaneously, and causes benign tumors of the brain, spinal cord, and peripheral nerves.
Schwannomatosis is an extremely rare genetic disorder closely related to the more-common disorder neurofibromatosis (NF). Originally described in Japanese patients, [1] it consists of multiple cutaneous schwannomas, central nervous system tumors, and other neurological complications, excluding hallmark signs of NF.
The three types of neurofibromatosis are caused by different mutations on chromosomes. NF1 is caused by a mutation on the NF1 gene on the arm of chromosome 17. [4] NF2 is caused by a mutation on the NF2 tumor suppressor gene on chromosome 22. [4] Schwannomatosis is caused by various mutations on chromosome 22. [4]
The translabyrinthine approach was developed by William F. House, M.D., [2] who began doing dissections in the laboratory with the aid of magnification and subsequently developed the first middle cranial fossa and then the translabyrinthine approach for the removal of acoustic neuroma.
A common subtype is the vestibular schwannoma (acoustic neuroma) [5]. Neurofibromas : Benign tumors that grow on nerves, commonly associated with neurofibromatosis type 1 (NF1) [ 2 ] . Malignant Peripheral Nerve Sheath Tumors (MPNST) : Rare but aggressive cancers that arise from peripheral nerves or nerve sheath cells [ 6 ] .
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Patient response to treatment will vary based on age, health, and the tolerance to medications and therapies. Metastasis occurs in about 39% of patients, most commonly to the lung. Features associated with poor prognosis include a large primary tumor (over 5 cm across), high grade disease, co-existent neurofibromatosis, and the presence of ...