Multiple meningiomas in brain and lung due to acquired mutation of the NF2 gene.
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Adult↗
Biomedical subjects
R Ojemann
Publications and source records attributed to R Ojemann.
Pedicled rhinotomy for clival chordomas invaginating the brainstem.
OBJECT: Clival chordomas are frequently midline lesions whose posterior growth may breach the dura and invaginate the brainstem. This precludes safe delivery of potentially curative high-dose fractionated proton radiotherapy. To avoid this problem, the authors performed pedicled rhinotomy to resect chordomas in 10 patients. METHODS: Pedicled rhinotomy is a midface transnasal route to the intercarotid sella and clivus from the tuberculum sellae to the mid-C-2 level. It involves a lateral rhinotomy incision, osteotomies of nasal bones and cartilage, lateral rotation of the nose, removal of the nasal septum and medial maxillary walls, opening of ethmoid and sphenoid sinuses, and dissection of nasopharynx and oropharynx to expose the clivus and craniovertebral junction. Tumors involving the sella, medial cavernous sinuses, middle and lower clivus, and C-1 arch and dens can be removed even if they traverse the dura. Closure involves dural repair, grafting of fat and split-thickness skin, rotation of a vascularized mucosal pedicle, and reattachment of nasal cartilage. Ten clival chordomas in adult patients were surgically removed via a pedicled rhinotomy approach. Seven patients had previously undergone a total of nine skull base procedures. In eight of the 10 patients, tumors compressing the brainstem were completely removed using this technique. One patient required an additional subtemporal resection of a suprasellar tumor before definitive radiotherapy could be undertaken. No patient sustained any new neurological deficit; in eight patients headache, diplopia, or hemiparesis improved. One patient developed postoperative cerebrospinal fluid leakage and meningitis that were successfully treated with antibiotic agents and shunt placement. CONCLUSIONS: Pedicled rhinotomy provides excellent shallow-field exposure of midline clival chordomas and permits relief of brainstem compression and the postoperative administration of potentially curative proton beam irradiation.
Adult↗
Management of atypical and malignant meningiomas: role of high-dose, 3D-conformal radiation therapy.
OBJECTIVE: Atypical and malignant meningiomas are at high risk for local failure. The role of radiation therapy (RT) and dose levels required to improve tumor control are poorly defined. This study reviews our experience with RT. MATERIAL AND METHODS: Thirty-one patients underwent fractionated RT for atypical (AM, 15 patients) or malignant meningioma (MM, 16 patients) of the cranium. Sixteen patients presented with primary and 15 with recurrent disease. Eight patients received RT following total resection, 21 patients after subtotal resection and 2 patient following biopsy only. RT was given using megavoltage photons in 15 patients and combined photons and 160 MeV protons in 16 patients. Total target doses ranged from 50 to 68 (AM, mean 62) and from 40 to 72 (MM, mean 58) Gy or CGE (= cobalt-gray-equivalent). RESULTS: With mean observation time of 59 months (range: 7-155 months) actuarial local control rates at 5- and 8-years were similar for both histologies (38% and 19% for AM and 52 and 17% for MM). However, significantly improved local control was observed for proton versus photon RT (80% versus 17% at 5 years, p = 0.003) and target doses > or = 60 Gy for both, atypical (p = 0.025) and malignant meningioma (p = 0.0006). At time of analysis, 14/15 patients (93%) with AM and 6/16 (38%) with MM were alive. Three patients (19%) with MM developed distant metastasis. Actuarial 5- and 8-year survival rates for MM were significantly improved by use of proton over photon RT and radiation doses > 60 CGE. Three patients developed symptomatic radiation damage after 59.3, 68.4 and 72 Gy/CGE. CONCLUSION: Conformal, high dose RT resulted in significant improvement of local control for atypical and malignant meningiomas. Increased local control resulted also in improved rates of survival for patients with malignant meningioma.
Adolescent↗
A point mutation associated with a severe phenotype of neurofibromatosis 2.
Neurofibromatosis 2 (NF2) is an autosomal dominant disease characterized by bilateral vestibular schwannomas and other nonmalignant tumors of the brain, spinal cord, and peripheral nerves. Although the average age of onset of NF2 is 20 years, some individuals may become symptomatic in childhood. We studied 5 unrelated NF2 patients who became symptomatic before age 13. All 5 had multiple tumors in addition to vestibular schwannoma, and none had a positive family history. Sequence analysis of the NF2 gene revealed identical nonsense mutation of exon 6 in 3 patients. Because this mutation destroys a restriction enzyme recognition site, genomic DNA from the 2 other children was directly tested for this change and identical alterations were detected. Although the work of our laboratory and others has not, in general, detected identical mutations in unrelated patients, this mutation seems to occur particularly frequently in the pediatric population and thus may be associated with an especially severe phenotype. Restriction analysis in children with NF2 may be a cost effective way of identifying their mutation. Further work is needed to characterize the effects of this change on the NF2 protein product and its relationship to this severe phenotype.
Adolescent↗
Molecular genetic screening for children at risk of neurofibromatosis 2.
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Adolescent↗
Meningiomas.
This article reviews the surgical management of meningiomas. Variations in surgical techniques for meningiomas in specific areas are presented. The author also reviews the role of adjunctive radiation therapy, especially in the treatment of recurrent tumors.
Humans↗
Acute quadriplegia due to bilateral cerebral metastases.
A 51-year-old man developed severe quadriparesis without sensory loss after a fall on the occiput. When he was treated with corticosteroids, the weakness worsened slightly for 2 days, but he then progressively improved and ultimately walked unassisted. Two months later, postmortem examination disclosed metastatic tumors in the superior portion of each precentral gyrus. The subjacent white matter was edematous. No spinal cord abnormalities were found. The clinical, radiologic, and neuropathologic findings suggest that the quadriplegia resulted from these bilateral cerebral lesions and not from spinal cord dysfunction. The rapid development of cerebral edema, perhaps precipitated by trauma, seemed to account for the acute onset of symptoms in this case.
Brain Edema↗
Intracerebral hemorrhage following carotid endarterectomy: a hypertensive complication?
Two patients with transient ischemic attacks and subsequent minor cerebral infarction had repair of very tight carotid stenosis, 4 and 5 weeks respectively after their stroke. Each developed intracerebral hemorrhage when hypertension was uncontrolled during the post-operative period. Hypertension is a significant complication of carotid endarterectomy, and may be a prominent factor in the development of intracerebral hemorrhage post-carotid endarterectomy.
Arteriosclerosis↗