Bilateral Vestibular Schwannomas in a Patient with Ring Chromosome 22: Case Report and Review of the Literature. 2021

Penelope E Nussbaum, and Puja D Patel, and Leslie A Nussbaum, and Christopher W Hilton, and Eric S Nussbaum
Department of Neurosurgery, National Brain Aneurysm & Tumor Center, United Hospital, Saint Paul, Minnesota, USA.

BACKGROUND Ring chromosome 22 (r[22]) can lead to the development of intracranial tumors such as meningiomas, neurofibromas, and schwannomas similar to neurofibromatosis 2 (NF2). METHODS An 18-year-old female with r(22) and a history of global development delay and cognitive impairment presented with sudden hearing loss. MRI revealed bilateral vestibular schwannomas. Given documented audiologic decline in the patient's hearing, the larger tumor was treated with CyberKnife fractionated stereotactic radiosurgery, and the smaller tumor is being monitored. CONCLUSIONS This case provides further evidence that patients with r(22) can develop clinical features of NF2, including the development of bilateral vestibular schwannomas, and should be monitored for hearing disturbances starting in puberty as a warning sign for these tumors.

UI MeSH Term Description Entries
D008577 Meningeal Neoplasms Benign and malignant neoplastic processes that arise from or secondarily involve the meningeal coverings of the brain and spinal cord. Intracranial Meningeal Neoplasms,Spinal Meningeal Neoplasms,Benign Meningeal Neoplasms,Leptomeningeal Neoplasms,Malignant Meningeal Neoplasms,Meningeal Cancer,Meningeal Neoplasms, Benign,Meningeal Neoplasms, Intracranial,Meningeal Neoplasms, Malignant,Meningeal Tumors,Neoplasms, Leptomeningeal,Neoplasms, Meningeal,Benign Meningeal Neoplasm,Cancer, Meningeal,Cancers, Meningeal,Intracranial Meningeal Neoplasm,Leptomeningeal Neoplasm,Malignant Meningeal Neoplasm,Meningeal Cancers,Meningeal Neoplasm,Meningeal Neoplasm, Benign,Meningeal Neoplasm, Intracranial,Meningeal Neoplasm, Malignant,Meningeal Neoplasm, Spinal,Meningeal Neoplasms, Spinal,Meningeal Tumor,Neoplasm, Benign Meningeal,Neoplasm, Intracranial Meningeal,Neoplasm, Leptomeningeal,Neoplasm, Malignant Meningeal,Neoplasm, Meningeal,Neoplasm, Spinal Meningeal,Neoplasms, Benign Meningeal,Neoplasms, Intracranial Meningeal,Neoplasms, Malignant Meningeal,Neoplasms, Spinal Meningeal,Spinal Meningeal Neoplasm,Tumor, Meningeal,Tumors, Meningeal
D009464 Neuroma, Acoustic A benign SCHWANNOMA of the eighth cranial nerve (VESTIBULOCOCHLEAR NERVE), mostly arising from the vestibular branch (VESTIBULAR NERVE) during the fifth or sixth decade of life. Clinical manifestations include HEARING LOSS; HEADACHE; VERTIGO; TINNITUS; and FACIAL PAIN. Bilateral acoustic neuromas are associated with NEUROFIBROMATOSIS 2. (From Adams et al., Principles of Neurology, 6th ed, p673) Acoustic Neuroma,Melanocytic Vestibular Schwannoma,Schwannoma, Acoustic,Schwannoma, Vestibular,Acoustic Neuroma, Cerebellopontine Angle,Acoustic Tumor,Angle Tumor,Cerebellopontine Angle Acoustic Neuroma,Cerebellopontine Angle Tumor,Neurilemmoma, Acoustic,Neurilemoma, Acoustic,Neurinoma of the Acoustic Nerve,Neurinoma, Acoustic,Neuroma, Acoustic, Unilateral,Vestibular Schwannoma,Acoustic Neurilemmoma,Acoustic Neurilemmomas,Acoustic Neurilemoma,Acoustic Neurilemomas,Acoustic Neurinoma,Acoustic Neurinomas,Acoustic Neuromas,Acoustic Schwannoma,Acoustic Schwannomas,Acoustic Tumors,Angle Tumor, Cerebellopontine,Angle Tumors,Angle Tumors, Cerebellopontine,Cerebellopontine Angle Tumors,Melanocytic Vestibular Schwannomas,Neurilemmomas, Acoustic,Neurilemomas, Acoustic,Neurinomas, Acoustic,Neuromas, Acoustic,Schwannoma, Melanocytic Vestibular,Schwannomas, Acoustic,Schwannomas, Melanocytic Vestibular,Schwannomas, Vestibular,Tumor, Acoustic,Tumor, Angle,Tumor, Cerebellopontine Angle,Tumors, Acoustic,Tumors, Angle,Tumors, Cerebellopontine Angle,Vestibular Schwannoma, Melanocytic,Vestibular Schwannomas,Vestibular Schwannomas, Melanocytic
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D012303 Ring Chromosomes Aberrant chromosomes with no ends, i.e., circular. Chromosomes, Ring,Chromosome, Ring,Ring Chromosome
D016518 Neurofibromatosis 2 An autosomal dominant disorder characterized by a high incidence of bilateral acoustic neuromas as well as schwannomas (NEURILEMMOMA) of other cranial and peripheral nerves, and other benign intracranial tumors including meningiomas, ependymomas, spinal neurofibromas, and gliomas. The disease has been linked to mutations of the NF2 gene (GENES, NEUROFIBROMATOSIS 2) on chromosome 22 (22q12) and usually presents clinically in the first or second decade of life. Neurofibromatosis, Acoustic, Bilateral,Neurofibromatosis, Central, NF2,Neuroma, Acoustic, Bilateral,Schwannoma, Acoustic, Bilateral,Acoustic Neurinoma, Bilateral,Acoustic Schwannomas, Bilateral,Bilateral Acoustic Neurofibromatosis,Central Neurofibromatosis,Familial Acoustic Neuromas,NF2 (Neurofibromatosis 2),Neurofibromatosis II,Neurofibromatosis Type 2,Neurofibromatosis Type II,Neurofibromatosis, Central NF2,Neurofibromatosis, Central, NF 2,Neurofibromatosis, Type 2,Neurofibromatosis, Type II,Neurofibromatosis, central type,Acoustic Neurinomas, Bilateral,Acoustic Neurofibromatoses, Bilateral,Acoustic Neurofibromatosis, Bilateral,Acoustic Neuroma, Familial,Acoustic Neuromas, Familial,Acoustic Schwannoma, Bilateral,Bilateral Acoustic Neurinoma,Bilateral Acoustic Neurinomas,Bilateral Acoustic Neurofibromatoses,Bilateral Acoustic Schwannoma,Bilateral Acoustic Schwannomas,Central NF2 Neurofibromatoses,Central NF2 Neurofibromatosis,Central Neurofibromatoses,Familial Acoustic Neuroma,NF2s (Neurofibromatosis 2),Neurinoma, Bilateral Acoustic,Neurinomas, Bilateral Acoustic,Neurofibromatoses, Bilateral Acoustic,Neurofibromatoses, Central,Neurofibromatoses, Central NF2,Neurofibromatoses, Type 2,Neurofibromatoses, Type II,Neurofibromatosis IIs,Neurofibromatosis, Bilateral Acoustic,Neurofibromatosis, Central,Neuroma, Familial Acoustic,Neuromas, Familial Acoustic,Schwannoma, Bilateral Acoustic,Schwannomas, Bilateral Acoustic,Type 2 Neurofibromatoses,Type 2 Neurofibromatosis,Type II Neurofibromatoses,Type II Neurofibromatosis
D016634 Radiosurgery A radiological stereotactic technique developed for cutting or destroying tissue by high doses of radiation in place of surgical incisions. It was originally developed for neurosurgery on structures in the brain and its use gradually spread to radiation surgery on extracranial structures as well. The usual rigid needles or probes of stereotactic surgery are replaced with beams of ionizing radiation directed toward a target so as to achieve local tissue destruction. Gamma Knife Radiosurgery,Linear Accelerator Radiosurgery,Stereotactic Body Radiotherapy,Stereotactic Radiosurgery,CyberKnife Radiosurgery,LINAC Radiosurgery,Radiosurgery, Gamma Knife,Radiosurgery, Linear Accelerator,Radiosurgery, Stereotactic,Stereotactic Radiation,Stereotactic Radiation Therapy,CyberKnife Radiosurgeries,Gamma Knife Radiosurgeries,LINAC Radiosurgeries,Linear Accelerator Radiosurgeries,Radiation Therapy, Stereotactic,Radiation, Stereotactic,Radiosurgery, CyberKnife,Radiosurgery, LINAC,Radiotherapy, Stereotactic Body,Stereotactic Body Radiotherapies,Stereotactic Radiation Therapies,Stereotactic Radiations,Stereotactic Radiosurgeries,Therapy, Stereotactic Radiation

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