[Neurinomas of the trigeminal nerve in neurofibromatosis. Apropos of two cases and review of the literature]. 1992

M Kchouk, and L Aouidj, and R Gouider, and S Oueslati, and S Touibi, and M Khaldi
Service de Neuroradiologie, Institut National de Neurologie, La Rabta, Tunis, Tunisie.

We report two cases of trigeminal neuroma in patients with Von Recklinghausen neurofibromatosis (NF1). Despite the high frequency of intracranial neuroma in neurofibromatosis, the localization on the fifth cranial nerve is an uncommon findings. In our two cases, neuromas were asymptomatic clinically and electrophysiologically, but computed tomography showed a small nodular lesion in the cerebellopontine cistern, independent of the seventh and eight nerve and which can only correspond to the fifth nerve. MR imaging very clearly demonstrates intracranial neuromas of all cranial nerves.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009442 Neurilemmoma A neoplasm that arises from SCHWANN CELLS of the cranial, peripheral, and autonomic nerves. Clinically, these tumors may present as a cranial neuropathy, abdominal or soft tissue mass, intracranial lesion, or with spinal cord compression. Histologically, these tumors are encapsulated, highly vascular, and composed of a homogenous pattern of biphasic fusiform-shaped cells that may have a palisaded appearance. (From DeVita Jr et al., Cancer: Principles and Practice of Oncology, 5th ed, pp964-5) Neurinoma,Schwannoma,Schwannomatosis, Plexiform,Neurilemoma,Neurilemmomas,Neurilemomas,Neurinomas,Plexiform Schwannomatoses,Plexiform Schwannomatosis,Schwannomas
D009456 Neurofibromatosis 1 An autosomal dominant inherited disorder (with a high frequency of spontaneous mutations) that features developmental changes in the nervous system, muscles, bones, and skin, most notably in tissue derived from the embryonic NEURAL CREST. Multiple hyperpigmented skin lesions and subcutaneous tumors are the hallmark of this disease. Peripheral and central nervous system neoplasms occur frequently, especially OPTIC NERVE GLIOMA and NEUROFIBROSARCOMA. NF1 is caused by mutations which inactivate the NF1 gene (GENES, NEUROFIBROMATOSIS 1) on chromosome 17q. The incidence of learning disabilities is also elevated in this condition. (From Adams et al., Principles of Neurology, 6th ed, pp1014-18) There is overlap of clinical features with NOONAN SYNDROME in a syndrome called neurofibromatosis-Noonan syndrome. Both the PTPN11 and NF1 gene products are involved in the SIGNAL TRANSDUCTION pathway of Ras (RAS PROTEINS). Peripheral Neurofibromatosis,Recklinghausen Disease of Nerve,von Recklinghausen Disease,Cafe-au-Lait Spots with Pulmonic Stenosis,Molluscum Fibrosum,NF1 (Neurofibromatosis 1),Neurofibromatosis I,Neurofibromatosis Type 1,Neurofibromatosis Type I,Neurofibromatosis, Peripheral Type,Neurofibromatosis, Peripheral, NF 1,Neurofibromatosis, Peripheral, NF1,Neurofibromatosis, Type 1,Neurofibromatosis, Type I,Pulmonic Stenosis with Cafe-au-Lait Spots,Recklinghausen Disease, Nerve,Recklinghausen's Disease of Nerve,Recklinghausens Disease of Nerve,Watson Syndrome,von Recklinghausen's Disease,Cafe au Lait Spots with Pulmonic Stenosis,Neurofibromatoses, Peripheral,Neurofibromatoses, Type I,Neurofibromatosis, Peripheral,Peripheral Neurofibromatoses,Pulmonic Stenosis with Cafe au Lait Spots,Syndrome, Watson,Type 1 Neurofibromatosis,Type 1, Neurofibromatosis,Type I Neurofibromatoses,Type I, Neurofibromatosis,von Recklinghausens Disease
D003390 Cranial Nerve Neoplasms Benign and malignant neoplasms that arise from one or more of the twelve cranial nerves. Cranial Neuroma, Benign,Benign Cranial Nerve Neoplasms,Benign Cranial Nerve Tumors,Cranial Nerve Neoplasms, Benign,Cranial Nerve Neoplasms, Malignant,Cranial Nerve Tumors, Benign,Cranial Nerve Tumors, Malignant,Malignant Cranial Nerve Neoplasms,Malignant Cranial Nerve Tumors,Neoplasms, Cranial Nerve,Neoplasms, Cranial Nerve, Benign,Neoplasms, Cranial Nerve, Malignant,Tumors, Cranial Nerve, Benign,Tumors, Cranial Nerve, Malignant,Benign Cranial Neuroma,Benign Cranial Neuromas,Cranial Nerve Neoplasm,Cranial Neuromas, Benign,Neoplasm, Cranial Nerve,Neuroma, Benign Cranial,Neuromas, Benign Cranial
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D014057 Tomography, X-Ray Computed Tomography using x-ray transmission and a computer algorithm to reconstruct the image. CAT Scan, X-Ray,CT Scan, X-Ray,Cine-CT,Computerized Tomography, X-Ray,Electron Beam Computed Tomography,Tomodensitometry,Tomography, Transmission Computed,X-Ray Tomography, Computed,CAT Scan, X Ray,CT X Ray,Computed Tomography, X-Ray,Computed X Ray Tomography,Computerized Tomography, X Ray,Electron Beam Tomography,Tomography, X Ray Computed,Tomography, X-Ray Computer Assisted,Tomography, X-Ray Computerized,Tomography, X-Ray Computerized Axial,Tomography, Xray Computed,X Ray Computerized Tomography,X Ray Tomography, Computed,X-Ray Computer Assisted Tomography,X-Ray Computerized Axial Tomography,Beam Tomography, Electron,CAT Scans, X-Ray,CT Scan, X Ray,CT Scans, X-Ray,CT X Rays,Cine CT,Computed Tomography, Transmission,Computed Tomography, X Ray,Computed Tomography, Xray,Computed X-Ray Tomography,Scan, X-Ray CAT,Scan, X-Ray CT,Scans, X-Ray CAT,Scans, X-Ray CT,Tomographies, Computed X-Ray,Tomography, Computed X-Ray,Tomography, Electron Beam,Tomography, X Ray Computer Assisted,Tomography, X Ray Computerized,Tomography, X Ray Computerized Axial,Transmission Computed Tomography,X Ray Computer Assisted Tomography,X Ray Computerized Axial Tomography,X Ray, CT,X Rays, CT,X-Ray CAT Scan,X-Ray CAT Scans,X-Ray CT Scan,X-Ray CT Scans,X-Ray Computed Tomography,X-Ray Computerized Tomography,Xray Computed Tomography
D014276 Trigeminal Nerve The 5th and largest cranial nerve. The trigeminal nerve is a mixed motor and sensory nerve. The larger sensory part forms the ophthalmic, mandibular, and maxillary nerves which carry afferents sensitive to external or internal stimuli from the skin, muscles, and joints of the face and mouth and from the teeth. Most of these fibers originate from cells of the TRIGEMINAL GANGLION and project to the TRIGEMINAL NUCLEUS of the brain stem. The smaller motor part arises from the brain stem trigeminal motor nucleus and innervates the muscles of mastication. Cranial Nerve V,Fifth Cranial Nerve,Nerve V,Nervus Trigeminus,Cranial Nerve, Fifth,Fifth Cranial Nerves,Nerve V, Cranial,Nerve Vs,Nerve, Fifth Cranial,Nerve, Trigeminal,Trigeminal Nerves,Trigeminus, Nervus

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