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Abstract Atypical teratoid/rhabdoid tumors (AT/RTs) are highly malignant CNS neoplasms that typically occur in children <2 years of age. These are characterized by high-grade histologic features and mutations of the INI1/SMARCB1 gene readily detected by loss of expression by immunohistochemistry. Among adults, the majority of AT/RTs occurs in the cerebral hemispheres. A small number of adult AT/RTs involving the sellar and suprasellar region reported in the literature suggest a distinct clinical course for this group. Here, we describe detailed clinical and genetic characterization of 5 adult patients with AT/RTs involving the sellar and suprasellar region, and provide a review of the available clinical and genetic features of 22 previously reported cases in order to help increase our understanding of this unusual entity.

Objective Facial nerve preservation is a critical measure of clinical outcome after vestibular schwannoma treatment. Gamma Knife radiosurgery has evolved into a practical treatment modality for vestibular schwannoma patients, with several reported series from a variety of centers. In this study, we report the results of an objective analysis of reported facial nerve outcomes after the treatment of vestibular schwannomas with Gamma Knife radiosurgery. Materials and methods A Boolean Pub Med search of the English language literature revealed a total of 23 published studies reporting assessable and quantifiable outcome data regarding facial nerve function in 2,204 patients who were treated with Gamma Knife radiosurgery for vestibular schwannoma. Inclusion criteria for articles were: (1) Facial nerve preservation rates were reported specifically for vestibular schwannoma, (2) Facial nerve functional outcome was reported using the House–Brackmann classification (HBC) for facial nerve function, (3) Tumor size was documented, and (4) Gamma Knife radiosurgery was the only radiosurgical modality used in the report. The data were then aggregated and analyzed based on radiation doses delivered, tumor volume, and patient age. Results An overall facial nerve preservation rate of 96.2% was found after Gamma Knife radiosurgery for vestibular schwannoma in our analysis. Patients receiving less than or equal to 13 Gy of radiation at the marginal dose had a better facial nerve preservation rate than those who received higher doses (≤13 Gy = 98.5% vs. >13 Gy = 94.7%, P  < 0.0001). Patients with a tumor volume less than or equal to 1.5 cm 3 also had a greater facial nerve preservation rate than patients with tumors greater than 1.5 cm 3 (≤1.5 cm 3 99.5% vs. >1.5 cm 3 95.5%, P  < 0.0001). Superior facial nerve preservation was also noted in patients younger than or equal to 60 years of age (96.8 vs. 89.4%, P  < 0.0001). The average reported follow up duration in this systematic review was 54.1 ± 31.3 months. Conclusion Our analysis of case series data aggregated from multiple centers suggests that a facial nerve preservation rate of 96.2% can be expected after Gamma knife radiosurgery for vestibular schwannoma. Younger patients with smaller tumors less than 1.5 cm 3 and treated with lower doses of radiation less than 13 Gy will likely have better facial nerve preservation rates after Gamma Knife radiosurgery for vestibular schwannoma.

Cranial chordomas are rare tumors that have been difficult to study given their low prevalence. Individual case series with decades of data collection provide some insight into the pathobiology of this tumor and its responses to treatment. This meta-analysis is an attempt to aggregate the sum experiences and present a comprehensive review of their findings. We performed a comprehensive review of studies published in English language literature and found a total of over 2,000 patients treated for cranial chordoma. Patient information was then extracted from each paper and aggregated into a comprehensive database. The tumor recurrences in these patients were then stratified according to age (<21 vs. >21 years), histological findings (chondroid vs. typical) and treatment (surgery and radiation vs. surgery only). Data was analyzed via Pearson chi-square and t -test. A total of 464 non-duplicated patients from 121 articles treated for cranial chordoma met the inclusion criteria. The recurrence rate among all patients was 68% (314 patients) with an average disease-free interval of 45 months (median, 23 months). The mean follow-up time was 39 months (median, 27 months). The patients in younger group, patients with chordoma with chondroid histologic type, and patients who received surgery and adjuvant radiotherapy had significantly lower recurrence rate than their respective counterparts. The results of our systematic analysis provide useful data for practitioners in objectively summarizing the tumor recurrence in patients with cranial chordomas. Our data suggests that younger patients with chondroid type cranial chordoma treated with both surgery and radiation may have improved rates of tumor recurrence in the treatment of these tumors.

Given its rarity, appropriate treatment for pineocytoma remains variable. As the literature primarily contains case reports or studies involving a small series of patients, prognostic factors following treatment of pineocytoma remain unclear. We therefore compiled a systematic review of the literature concerning post-treatment outcomes for pineocytoma to better determine factors associated with overall survival among patients with pineocytoma. We performed a comprehensive search of the published English language literature to identify studies containing outcome data for patients undergoing treatment for pineocytoma. Kaplan–Meier analysis was utilized to determine overall survival rates. Our systematic review identified 168 total patients reported in 64 articles. Among these patients, 21% underwent biopsy, 38% underwent subtotal resection, 42% underwent gross total resection, and 29% underwent radiation therapy, either as mono- or adjuvant therapy. The 1 and 5 year overall survival rates for patients receiving gross total resection versus subtotal resection plus radiotherapy were 91 versus 88%, and 84 versus 17%, respectively. When compared to subtotal resection alone, subtotal resection plus radiation therapy did not offer a significant improvement in overall survival. Gross total resection is the most appropriate treatment for pineocytoma. The potential benefit of conventional radiotherapy for the treatment of these lesions is unproven, and little evidence supports its use at present.