Characteristics and overall survival in pediatric versus adult skull base chordoma: a population-based study.

PURPOSE: Less than 5% of chordomas occur in pediatric patients. While many studies have explored the treatment and outcomes of skull base chordomas, few have focused on the differences between pediatric and adult populations. The aim of this study is to analyze the epidemiological variables and clinical outcomes between pediatric and adult skull base chordomas using a large-sample, population-based cancer database. METHODS: The National Cancer Database was queried between 2004 and 2015 for skull base chordomas. We stratified patients as pediatric (<18 years) and adults (≥18 years). We compared several clinical covariates between the two groups. RESULTS: Our cohort consisted of 658 patients, 61 pediatric (9.3%), and 597 adults (90.7%). Pediatric patients were more likely to have larger tumor size (41.4 ± 15.7 mm versus 34.1 ± 15.8 mm, p < 0.01) and universally treated at academic facilities. There was no significant difference in overall survival. CONCLUSIONS: Pediatric skull base chordomas are rare tumors that are managed with aggressive surgical resection, followed by radiation. While there may be difference between tumor presentation, outcomes between pediatric and adult patients are similar.

Intraoperative neuromonitoring techniques in the surgical management of acoustic neuromas.

Unfavorable outcomes such as facial paralysis and deafness were once unfortunate probable complications following resection of acoustic neuromas. However, the implementation of intraoperative neuromonitoring during acoustic neuroma surgery has demonstrated placing more emphasis on quality of life and preserving neurological function. A modern review demonstrates a great degree of recent success in this regard. In facial nerve monitoring, the use of modern electromyography along with improvements in microneurosurgery has significantly improved preservation. Recent studies have evaluated the use of video monitoring as an adjunctive tool to further improve outcomes for patients undergoing surgery. Vestibulocochlear nerve monitoring has also been extensively studied, with the most popular techniques including brainstem auditory evoked potential monitoring, electrocochleography, and direct compound nerve action potential monitoring. Among them, direct recording remains the most promising and preferred monitoring method for functional acoustic preservation. However, when compared with postoperative facial nerve function, the hearing preservation is only maintained at a lower rate. Here, the authors analyze the major intraoperative neuromonitoring techniques available for acoustic neuroma resection.

Ergonomics of Endoscopic Skull Base Surgery: A Systematic Review.

OBJECTIVE: There has been a significant expansion in endonasal endoscopic skull base surgery (EES) that has been used to address a wide range of intracranial and sinonasal pathologies. Although there exists a large amount of literature on approaches and patient outcomes, there is a paucity of data describing ergonomics in this field. Our goal was to evaluate and summarize the literature on ergonomics in EES. METHODS: We systematically reviewed all published, peer-reviewed, English language literature in the PubMed and Web of Science databases as screened by multiple reviewers describing ergonomics as related to EES. RESULTS: A total of 50 articles were found that described significant conclusions and descriptions on ergonomics in EES. We found and summarized the different technical aspects of ergonomics as pertaining to EES and provided evidence-based suggestions on operating room and surgeon setup. CONCLUSIONS: There are several improvements in EES ergonomics that can decrease fatigue, improve efficiency, and overall surgeon well-being.

Characteristics and overall survival in pediatric versus adult esthesioneuroblastoma: A population-based study.

BACKGROUND: Esthesioneuroblastoma (ENB) is an uncommon sinonasal malignancy and is even less common in the pediatric population. OBJECTIVE: The purpose of this study is to compare characteristics and outcomes of ENB between adult and pediatric patients. METHODS: The National Cancer Database was queried for patients with histologically proven ENB of the nasal cavity and paranasal sinuses, and then baseline characteristics, treatment, and survival data compared between the pediatric (age < 18 years) and adult (age ≥ 18 years) populations. RESULTS: 1411 patients were identified, with 45 in the pediatric cohort and 1366 in the adult cohort. Ten-year overall survival (OS) in the pediatric cohort was improved compared to the adult cohort, 87% and 66%, respectively (p < 0.05). Adjuvant chemotherapy was more commonly utilized in the pediatric cohort (p < 0.001). Race was associated with decreased OS in the pediatric cohort (p = 0.013). Pediatric patients had shorter length of stay (p = 0.009) and lived closer to their provider (p = 0.044) than adult ENB patients. CONCLUSION: Treatment of ENB in pediatric patients more commonly includes chemotherapy and more commonly occurs at academic medical centers. OS is improved in pediatric ENB compared to adults as well, but larger studies are necessary.

The role of regulatory T-cells in glioma immunology.

Despite recent advances in treatment, the prognosis for glioblastoma multiforme (GBM) remains poor. The lack of response to treatment in GBM patients may be attributed to the immunosuppressed microenvironment that is characteristic of invasive glioma. Regulatory T-cells (Tregs) are immunosuppressive T-cells that normally prevent autoimmunity when the human immune response is evoked; however, there have been strong correlations between glioma-induced immunosuppression and Tregs. In fact, induction of Treg activity has been correlated with glioma development in both murine models and patients. While the exact mechanisms by which regulatory T-cells function require further elucidation, various cytokines such as interleukin-10 (IL-10) and transforming growth factor-ß (TFG-ß) have been implicated in these processes and are currently under investigation. In addition, hypoxia is characteristic of tumor development and is also correlated with downstream induction of Tregs. Due to the poor prognosis associated with immunosuppression in glioma patients, Tregs remain a promising area for immunotherapeutic research.

Hearing preservation after LINAC radiosurgery and LINAC radiotherapy for vestibular schwannoma.

Linear accelerators (LINAC) can deliver both radiosurgery and fractionated radiotherapy. In this systematic analysis, we compare hearing preservation in patients with vestibular schwannomas (VS) treated with either LINAC-based radiotherapy (SRT) or LINAC-based radiosurgery (SRS), with an emphasis on the prognostic implications of tumor size and patient age. A total of 400 patients met our criteria for LINAC SRS, with an average hearing preservation rate of 66.3%. Patients with smaller tumors (<3.0 cm(3)) treated with SRS had similar hearing preservation rates to those with larger tumors. However, younger patients (<55 years) demonstrated improved hearing preservation compared to older patients (≥55 years). When comparing LINAC SRS to LINAC SRT directly, hearing preservation was similar in patients with smaller tumors. However, patients with larger tumors (≥3.0cm(3)) who received SRT had higher hearing preservation rates than those who received SRS. A total of 629 patients met our criteria for LINAC SRT, with an average hearing preservation rate of 75.3%. Patients with larger tumors who received SRT had better hearing outcomes than those with smaller tumors, while there was no significant difference in hearing preservation in younger patients compared to older patients. When comparing LINAC SRS to LINAC SRT directly, younger patients had similar hearing preservation rates. However, older patients who received SRT had improved hearing preservation compared to those who received SRS. In a direct comparison of average hearing preservation, patients who received SRT had higher hearing preservation rates than those who underwent SRS. Prospective studies will be needed to further characterize radiation dose and other variables.

Post-transplantation primary central nervous system lymphoma: A case report and review of the literature.

BACKGROUND: Post-transplantation primary central nervous system lymphoma (PT-PCNSL) is a rare neoplasm that can develop within months to years after transplantation, and imaging often reveals multiple lesions with homogeneous or ring enhancement. The clinical and imaging presentation of PT-PCNSL can often be nonspecific and present a diagnostic challenge. CASE DESCRIPTION: A 56-year-old woman presented to a tertiary university emergency room with altered mental status 15 months after undergoing renal transplantation. On brain MRI, she was found to have three rim-enhancing mass lesions, and biopsy revealed PT-PCNSL. CONCLUSION: There has been a steady increase in the number of patients living following organ transplantation in the United States and an increasing likelihood that PT-PCNSL will increasingly be encountered in neurosurgical practice. We present here a case of PT-PCNSL and a brief review of the relevant clinical characteristics, treatment options, and prognosis of PT-PCNSL.