A case of pleomorphic adenoma of the nasal septum

We report a rare case of pleomorphic adenoma arising from the nasal septum. A 32-year-old woman presented with a 3-week-history of left-sided nasal obstruction. Computed tomographic scans revealed an oval mass, measuring about 30 mm in its greatest dimension, in the left anterior nasal cavity. The tumor arose from the anterior part of the nasal septum, and was removed endoscopically with endonasal approach. The microscopic finding showed a lobular and duct-like structures consisting of a loose chondromyxoid stroma. This lesion had histological characteristics of a pleomorphic adenoma, and this was confirmed by immunohistochemical expression of cytokeratin and S-100 protein. Her post-operative course was uneventful, and she is currently free from the disease 8 months after surgery. Diagnosis, clinical behavior and treatment of pleomorphic adenoma of the nasal septum are reviewed from perusal of the literature.     

Clinical investigation of genetic contributions to childhood-onset epilepsies and epileptic syndromes

Although there have been major advances in the understanding of the molecular bases of certain inherited epilepsy syndromes, clinical studies are still needed to verify the possible genetic contributions to common epilepsies. We examined the proportions of positive family histories of epilepsy (within second-degree relatives) and consanguinity (within first-degree relatives) in 311 probands with childhood-onset epilepsy, and found that they had high family history rates of epilepsy (19.3%) and consanguinity (6.1%). A positive family history of epilepsy was found more in probands with generalized epilepsy than in ones with localization-related epilepsy, and more in probands with idiopathic/cryptogenic epilepsy than in ones with symptomatic epilepsy. However, on analysis after the symptomatic epilepsies had been divided into two categories, probands with pre- or perinatal symptomatic generalized epilepsy and ones with postnatal symptomatic localization-related epilepsy showed high positive family history rates, similar to ones with idiopathic/cryptogenic epilepsy. On the other hand, a positive family history of consanguinity was noted more in probands with generalized epilepsy than in ones with localization-related epilepsy, but there was no significant difference between probands with idiopathic/cryptogenic epilepsy and ones with symptomatic epilepsy. These findings suggest that in addition to the hereditary effect on idiopathic/cryptogenic epilepsy, a genetic susceptibility may contribute to the development of pre- or perinatal symptomatic generalized epilepsy, and to that of postnatal symptomatic localization-related epilepsy. Furthermore, a genetic predisposition seems to have an influence through consanguinity on the etiologies of both idiopathic/cryptogenic and symptomatic generalized epilepsies.     

Incapacitating lower limb pain syndrome in cord blood stem cell transplant recipients with calcineurin inhibitor

Calcineurin-inhibitor induced pain syndrome (CIPS) is a newly described entity with a characteristic feature of sudden onset of severe lower limb pain, and high levels of cyclosporine or tacrolimus may be involved in the pathogenesis. This syndrome is rarely seen in recipients of hematopoietic stem cell transplantation (HSCT) compared with other organ transplant recipients, however, heightened awareness of this complication after HSCT may be needed for hematologists, as misdiagnosis can result in catastrophic consequences. We report herein two cases of lower limb pain syndrome, with some clinical features resembling CIPS, occurring during the early phase of cord blood stem cell transplantation for hematological malignancy.     

Contrast-enhanced MRA for investigation of cerebral arteriovenous malformations

We evaluated contrast-enhanced MRA (enhanced 3-D fast gradient-echo [efgre3d] with spectral inversion recovery) for identification of 15 intracranial arteriovenous malformations (AVMs) in 14 patients. Demonstration of the feeding arteries was classified as good for 16 examinations on maximum-intensity projections and multiprojection volume reconstruction images. The nidus was seen well in all patients. Definition of the draining veins was good or fair except for one poor result. Therapeutic effects were clearly demonstrated in three follow-up series. Contrast-enhanced MRA using efgre3d is useful for delineation of AVMs and for follow-up after treatment.     

Ionic interaction of [<Superscript>11</Superscript>C]-<Emphasis Type="Italic">N</Emphasis>, α-dimethylbenzylamine (DMBA) in rodent brain

The [S] enantiomer of [11C]-N,alpha-dimethylbenzylamine (DMBA) was synthesized by N-methylation of [S]-alpha-methylbenzylamine, and its biodistribution in mice was measured. [11C]-[S]-DMBA was rapidly distributed into the brain, heart and lungs, and considerable long-term retention in the brain was observed. The radioactive metabolites in the plasma were analyzed by liquid chromatography. Kinetic analysis using unmetabolized [11C]DMBA in the plasma as the input function was performed employing a simplified two-compartment model. The estimated distribution volumes (DV) of [11C]DMBA in the brain and heart were 6.05 and 3.95, respectively. The right striatum of the rat brain was lesioned with ibotenic acid 2 weeks before the tracer experiment. Both in vitro and in vivo autoragiographic studies were performed, and revealed significant reduction of the radioactivity levels in the lesioned striatum. On the other hand, the regional cerebral blood flow, as measured by [14C]iodoantipyrine, was not significantly altered in the lesioned striatum. These results indicate that the ionic binding component for DMBA exists mainly in neural cells rather than in glial cells. [11C]DMBA might be a useful radiotracer for detection of neural cell loss in the brain.     

Long-term results of stereotactic gamma radiosurgery of meningiomas

BACKGROUND

The early effects at a mean of 30 months and long-term results at 7 years after gamma radiosurgery for meningiomas were evaluated.

METHODS

Changes in tumor size were evaluated every 3 to 6 months after treatment using a five-point grading system, as well as changes in neurological signs and general status.

RESULTS

Early effects in 87 cases of benign meningioma showed a minimal size reduction of 16.1% and a response rate of 8.0%, but a higher control rate of 93%. The cavernous sinus meningioma showed a size reduction of 23.2%, a response rate of 11.1%, and control of 100%. A greater size reduction of 24.8% and response rate of 33.3%, but a lower control rate of 75% were obtained in 12 cases of malignant meningioma. Side effects were found in 12 cases (13.8%): radiation-induced edema in 9, hearing disturbance in 2, and visual deterioration in 1. Long-term results for 54 of 87 patients with benign tumors showed that response increased from 8% to 42.6% but control decreased slightly due to increased disease progression.

CONCLUSION

Gamma radiosurgery is effective and safe for meningiomas to control residual or recurrent tumors after surgery and initial tumors, with acceptable side effects and rate of tumor progression.     

Radiosurgery of cavernous hemangiomas in the cavernous sinus

BACKGROUND: Cavernous hemangiomas in the cavernous sinus are rare and demonstrate unique clinical courses. Although they rarely cause spontaneous bleeding, serious bleeding is not uncommon during operations. Total eradication of such tumors is very difficult because of the location and intraoperative bleeding. Consequently, alternatives to operative resection have been examined. METHODS: Three cases of cavernous hemangiomas in the cavernous sinus, presenting chiefly with ocular signs and facial pain, were treated by radiosurgery using a gamma knife. Two of the patients had been operated on before radiosurgery, while the third patient was diagnosed on the basis of neurological signs as well as radiological findings. RESULTS: MRI scans at the time of radiosurgery showed tumors in the cavernous sinus with low or iso-intensity on T1-weighted images and high signal intensity on T2-weighted images. All of the tumors intensely enhanced with gadolinium-DTPA. The tumors had diameters of 14 to 28 mm and were treated with a marginal dose of 14 to 17 Gy (mean 15.7 Gy). In the mean follow-up period of 27 months after radiosurgery, all of the tumors decreased in size (PR). Neurologically, none of the patients showed any deterioration, and one demonstrated an obvious improvement in ocular movement. CONCLUSIONS: Radiosurgery for cavernous hemangioma in the cavernous sinus is apparently safe and effective with consistent tumor shrinkage. Therefore, radiosurgery is an excellent alternative to operative intervention and may even replace operative procedures if the tumors are small in diameter or when they recur.