Surgical treatment of trigeminal schwannomas

Schwannomas that arise from the trigeminal nerve are rare, but this nerve is the second most frequent intracranial site of schwannoma occurrence next to the vestibular nerve. The advent of microsurgical techniques and skull-base approaches has greatly enhanced the surgical management of these tumors, and outcomes have improved markedly. This report documents 18 cases of histologically verified schwannomas that arose from the trigeminal nerve and were treated surgically in our clinic between January 1992 and July 2005. The patients were ten women and eight men of age 39.7 years (range, 22-62 years). The tumor was located in the middle fossa (type A) in five cases, in the middle and posterior fossae (type C) in nine cases, in the posterior fossa (type B) in two cases, and in the branches of the trigeminal nerve (type D) in two cases. Total excision was achieved in 17 cases, and there was no mortality in the series. Our results indicate that trigeminal schwannomas, regardless of type, can be removed via skull-base approaches. We present an algorithm for surgical management of trigeminal schwannomas based on our experience and information from the literature

Changing treatment strategy of cavernous sinus meningiomas: experience of a single institution

Background: Oncological treatment of a neoplasm is more than surgical removal of the tumor. Probably, this truth is the reason for the ongoing discussion on cavernous sinus meningiomas in the last decade. Debate on optimal management of cavernous sinus meningiomas aims to compare the different treatment strategies: (a) radical surgical resection and (b) conservative surgical resection complemented with radiosurgical treatment. Materials and Methods: Natural history of the change in the management strategy of cavernous sinus meningiomas in our department before and after GK facility became available in 1997 allowed us to compare the 2 aforementioned strategies. Before installation of a Leksell GK unit at the hospital in 1997, the neurosurgical team at Marmara University Institute of Neurological Sciences and Faculty of Medicine (Istanbul, Turkey) treated patients with cavernous sinus meningioma using radical resection (radical strategy, group A, 10 patients). After 1997, the same neurosurgical team used understanding of surgical removal of the extracavernous sinus tumor component with GK irradiation of the intracavernous part (conservative strategy, group B, 12 patients). Another group of patients, who were treated with GK as a first-step treatment, was analyzed (GK group, group C, 26 patients). Results: At the end of the third year, more stable tumor volume control was achieved in groups B and C; after the second year, an incline in the tumor volume-time graph was detected. Group B resulted in less cranial nerverelated complications; a certain degree of improvement in cranial nerve deficits was observed. Conclusion: Comparing 2 different management strategies for cavernous sinus meningiomas in the same hospital setting using the same neurosurgical group, we conclude that extracavernous resection followed by GK is as effective as radical surgery. Considering cranial nerve complications and third-year tumor volume control achievement, conservative approach yielded better results. Longer follow-up with larger series is necessary. (c) 2005 Elsevier Inc. All rights reserved

Gamma-knife radiosurgery in the treatment of trigeminal schwannomas

Background. Trigeminal nerve schwannomas account for 0.07%-0.28% of all intracranial tumours. Advances in skull base surgery have led to more aggressive resection of these tumours, but surgery may associated with development of new neurological deficits. Methods. In this report, we analyse the long-term results 15 patients with newly diagnosed or residual/recurrent trigeminal schwannoma who underwent gamma-knife treatment. Findings. During a mean 61 months of follow-up, MRI revealed reduction of tumour size in 13 and no size change in 2 patients. The tumour growth control rate was 100% and only 1 patient had transient facial numbness and diplopia. Conclusions. For patients with small to moderate size trigeminal schwannomas, gamma-knife radiosurgery is associated with good tumour control and a minimal risk of adverse radiation effects

Apparently novel genetic syndrome of pachygyria, mental retardation, seizure, and arachnoid cysts

We report on an apparently new syndrome in a consanguineous family with seven members, three of whom have cerebral anomalies including pachygyria and arachnoid cysts along with mental retardation and seizures. The two patients with seizure disorders also had multiple enlarged perivascular spaces seen in the white matter of the centrum semiovale. Our data provide a contribution to the acacumulating knowledge on familial cerebral anomalies including arachnoid cysts and lissencephaly. Given the lack of mutation in known lissencephaly genes such as LIS1, 14-3-3 epsilon, and DCX, this syndrome may constitute a new phenotype with autosomal recessive inheritance.(c) 2007 Wiley-Liss, Inc

Gamma knife radiosurgery for cavernous sinus plasmacytoma in a patient with breast cancer history

Background: Multiple myeloma (MM) presentation with cerebral mass lesion is unusual. Gamma knife radiosurgery for plasmacytoma has not been reported so far. Case Report: We report a 70-year-old female with a medical history of infiltrative ductal carcinoma of the breast. She developed cavernous sinus syndrome (CSS) 5 months before admission to the hospital. The magnetic resonance imaging revealed an isointense solitary mass in the left cavernous sinus in noncontrast T1-weighted images. The lesion was highly enhancing with gadolinium-diethylenetriaminopentaacetic acid. She was operated by using Dolenc technique, and the tumor was partially resected. The pathological examination of the tumor tissue revealed a plasmacytoma. Systemic evaluation was positive for the diagnosis of MM. She underwent gamma knife radiosurgery for the residual cavernous sinus tumor. Chemotherapy with prednisolone and melphalan was given. Follow-up magnetic resonance images 6 months after the treatment demonstrated complete tumor disappearance. However, she died of sepsis 26 months after the diagnosis. Conclusion: This is an unusual MM case with a history of breast cancer, which had CSS and which demonstrated an excellent response to gamma knife radiosurgery. (C) 2005 Elsevier Inc. All rights reserved

A novel heterozygous deletion within the 3 ' region of the PAX6 gene causing isolated aniridia in a large family group

Paired box gene 6 (PAX6) is the causative gene of aniridia. it is a dominantly inherited eye abnormality characterized by partial or complete absence of the iris. The PAX6 gene is located on chromosome 11 p13 and contains 14 exons. It is expressed mainly in the developing eye and central nervous system. Submicroscopic copy number variations are common in the human genome. Submicroscopic deletions may cause several human diseases, either by disrupting coding sequences or by eliminating regulatory elements essential for expression of the gene in question. Over the past several years, array-based comparative genomic hybridization has become an increasingly useful too] for both identifying normal cytogenetic variations and characterizing chromosomal abnormalities associated with developmental delays and cancer. Our results support the notion that assessing copy number variation of the PAX6 gene itself and also of flanking regions, may contribute to the molecular diagnosis of aniridia. (C) 2009 Elsevier Ltd. All rights reserved

Low grade oligodendroglioma seeding around the 4th ventricle

We report a 45 years old female patient with a left temporal grade II oligodendroglioma that recurred on the wall of the fourth ventricle at grade II oligodendroglioma. © 2019, © 2019 The Neurosurgical Foundation

New candidate chromosomal regions for chordoma development

Background: Chordomas are rare, slow growing, infiltrative tumors thought to arise from vestigial or ectopic notochord. Chordoma can occur along the axial skeleton, predominantly in the sphenooccipital, vertebral, and sacrococcygeal regions. Although most chordomas are sporadic, familial cases have also been reported. The most common molecular cytogenetic abnormalities in these tumors are monosomy of chromosome I and gain of chromosome 7. In addition, a variety of other chromosomal changes, which are associated with losses and gains of different chromosomes, have also been described in chordomas, such as 1q, 2p, 3p, 5q, 9p, 10, l2q, 13q, 17, and 20q. Methods: In this study, using molecular cytogenetics (iFISH), we have studied 1p36, 1q25, 3p13p14, 7q33, 17p13.1 (p53 gene locus), 2p13 (TGF-alpha locus), 6p12 (VEGF locus), and 4q26-q27 (bFGF/FGF2 locus) loci in chordoma tissues from seven patients with 7 primary tumors and 11 recurrences. Results: We found that chromosomes 1p36, 1q25, 2p13, and 7q33 are affected in primary chordomas, and these aberrations persist in recurrences. However, the chromosome 6p12 aberration was seen only in primary chordomas, but not in recurrences, indicating that this locus may be associated with chordoma genesis. Conclusions: Our descriptive data from interphase FISH analyses suggest that future studies should incorporate a larger number of patients and should focus on identifying the candidate genes in chordoma pathogenesis. Such studies may use a whole-genomic approach, in addition to the regions identified in this study and others. (C) 2007 Elsevier Inc. All rights reserved