Impact of Salvage Surgery and Re-irradiation for Radiation Failed Recurrent Skull Base Meningiomas

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Prognostic Molecular Biomarkers in Chordomas: A Systematic Review and Identification of Clinically Usable Biomarker Panels

INTRODUCTION AND OBJECTIVE: Despite the improvements in management and treatment of chordomas over time, the risk of disease recurrence remains high. Consequently, there is a push to develop effective systemic therapeutics for newly diagnosed and recurrent disease. In order to tailor treatment for individual chordoma patients and develop effective surveillance strategies, suitable clinical biomarkers need to be identified. The objective of this study was to systematically review all prognostic biomarkers for chordomas reported to date in order to classify them according to localization, study design and statistical analysis. METHODS: Using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, we systematically reviewed published studies reporting biomarkers that correlated with clinical outcomes. We included time-to-event studies that evaluated biomarkers in skull base or spine chordomas. To be included in our review, the study must have analyzed the outcomes with univariate and/or multivariate methods (log-rank test or a Cox-regression model). RESULTS: We included 68 studies, of which only 5 were prospective studies. Overall, 103 biomarkers were analyzed in 3183 patients. According to FDA classification, 85 were molecular biomarkers (82.5%) mainly located in nucleus and cytoplasm (48% and 27%, respectively). Thirty-four studies analyzed biomarkers with Cox-regression model. Within these studies, 32 biomarkers (31%) and 22 biomarkers (21%) were independent prognostic factors for PFS and OS, respectively. CONCLUSION: Our analysis identified a list of 13 biomarkers correlating with tumor control rates and survival. The future point will be gathering all these results to guide the clinical validation for a chordoma biomarker panel. Our identified biomarkers have strengths and weaknesses according to FDA\u27s guidelines, some are affordable, have a low-invasive collection method and can be easily measured in any health care setting (RDW and D-dimer), but others molecular biomarkers need specialized assay techniques (microRNAs, PD-1 pathway markers, CDKs and somatic chromosome deletions were more chordoma-specific). A focused list of biomarkers that correlate with local recurrence, metastatic spread and survival might be a cornerstone to determine the need of adjuvant therapies

Prevalence of Pituitary Hormone Dysfunction Following Radiotherapy for Sinonasal and Nasopharyngeal Malignancies.

BACKGROUND: There are limited studies and no surveillance protocols on pituitary dysfunction for adults who underwent anterior skull base radiation. METHODS: Cross-sectional study of 50 consecutive patients with sinonasal or nasopharyngeal cancer who underwent definitive radiotherapy. The mean radiation doses, prevalence of pituitary dysfunction, and associated factors were calculated. RESULTS: Pituitary hormone levels were abnormal in 23 (46%) patients, including 6 (12%) with symptomatic abnormalities requiring treatment. The most common hormonal abnormality was hyperprolactinemia (30%), central hypothyroidism (8%) and central hypogonadism (6%). Patients with abnormal pituitary hormone values received higher mean radiation doses to the pituitary gland (1143 cGy, P = 0.04), pituitary stalk (1129 cGy, P = 0.02), optic chiasm (1094 cGy, P = 0.01), and hypothalamus (900 cGy, P = 0.01). CONCLUSIONS: Nearly half of the patients had abnormal pituitary function, including over a tenth requiring treatment. There may be a dose-dependent association between hormonal dysfunction and radiation

Distinct Immune Signature Predicts Progression of Vestibular Schwannoma and Unveils a Possible Viral Etiology

BACKGROUND: The management of sub-totally resected sporadic vestibular schwannoma (VS) may include observation, re-resection or irradiation. Identifying the optimal choice can be difficult due to the disease\u27s variable progression rate. We aimed to define an immune signature and associated transcriptomic fingerprint characteristic of rapidly-progressing VS to elucidate the underpinnings of rapidly progressing VS and identify a prognostic model for determining rate of progression. METHODS: We used multiplex immunofluorescence to characterize the immune microenvironment in 17 patients with sporadic VS treated with subtotal surgical resection alone. Transcriptomic analysis revealed differentially-expressed genes and dysregulated pathways when comparing rapidly-progressing VS to slowly or non-progressing VS. RESULTS: Rapidly progressing VS was distinctly enriched in CD4 CONCLUSION: Rapid progression of residual vestibular schwannoma following subtotal surgical resection has an underlying immune etiology that may be virally originating; and despite an abundant adaptive immune response, T-cell immunosenescence may be associated with rapid progression of VS. These findings provide a rationale for clinical trials evaluating immunotherapy in patients with rapidly progressing VS

Chondromyxoid Fibroma of the Skull Base and Calvarium: Surgical Management and Literature Review

Chondromyxoid fibroma (CMF) is an exceedingly rare tumor that represents less than 1% of all primary bone neoplasms. Occurrence in the facial and cranial bones is extremely rare and frequently misdiagnosed. Case Reports We report two cases of CMF, one in the sphenoclival skull base and the other involving the parietal bone in two young female patients. Excision was performed in both cases. Presenting symptoms, treatment, and follow-up are reported. Methods A retrospective review of the literature on cranial CMF was performed. The location, demographics, presenting symptoms, and treatment of all calvarial and skull base CMF cases published since 1990 are summarized. Discussion In our literature review, we found 67 published cases of cranial CMF. Mean age of all calvarial and skull base CMFs at diagnosis was 38.2 years old. Of the cases affecting the cranium, the sinonasal structures were most commonly involved. To our knowledge we report only the second case of CMF involving the parietal bone published in an English-language journal. Total resection is the best treatment, and should be the goal of surgical intervention. Curettage results in high recurrence rates. Radiotherapy in the setting of subtotal resection or recurrence cannot be definitively recommended and needs further investigation

Chondromyxoid Fibroma of the Skull Base and Calvarium: Surgical Management and Literature Review

Chondromyxoid fibroma (CMF) is an exceedingly rare tumor that represents less than 1% of all primary bone neoplasms. Occurrence in the facial and cranial bones is extremely rare and frequently misdiagnosed. Case Reports We report two cases of CMF, one in the sphenoclival skull base and the other involving the parietal bone in two young female patients. Excision was performed in both cases. Presenting symptoms, treatment, and follow-up are reported. Methods A retrospective review of the literature on cranial CMF was performed. The location, demographics, presenting symptoms, and treatment of all calvarial and skull base CMF cases published since 1990 are summarized. Discussion In our literature review, we found 67 published cases of cranial CMF. Mean age of all calvarial and skull base CMFs at diagnosis was 38.2 years old. Of the cases affecting the cranium, the sinonasal structures were most commonly involved. To our knowledge we report only the second case of CMF involving the parietal bone published in an English-language journal. Total resection is the best treatment, and should be the goal of surgical intervention. Curettage results in high recurrence rates. Radiotherapy in the setting of subtotal resection or recurrence cannot be definitively recommended and needs further investigation

Functional Outcome in the Neurosurgical Patient and Its Impact on Quality of Life

Outcomes research has become an integral part of most clinical studies today. Extent of resection, increased median survival, and “time to progression” are no longer the only important end points in need of assessment. Identifying the long-term adverse effects of treatment has become increasingly important as patients try to resume previous activities and an independent lifestyle. The measurement of functional states and health-related quality of life issues are at the forefront of medicine and have become necessary measures of functional outcome following the treatment of a variety of medical and surgical disorders. In this article, we review the most recent studies on the functional outcome of patients undergoing neurological surgeries and its impact on quality of life

Resolution of Tonsillar Herniation and Cervical Syringomyelia Following Resection of a Large Petrous Meningioma: Case Report and Review of Literature

We describe a case of a large, petrous meningioma associated with tonsillar herniation and cervical syringomyelia. The patient, a 53-year-old woman, had a 6-month history of a dull, aching pain in the occipital region associated with numbness in the right C2 dermatome and left gaze evoked nystagmus. Magnetic resonance imaging (MRI) revealed a large tumor in the right posterior fossa associated with moderate supratentorial hydrocephalus. Secondary tonsillar herniation and cervical syringomyelia extending from C2 to C6 were also identified. The tumor, later confirmed to be a meningioma originating from the petrous region, was resected completely via a retrosigmoid approach. Postoperative MRI demonstrated total resolution of the tonsillar herniation and cervical syringomyelia. The radiological features, potential pathophysiological mechanisms, and treatment strategies are discussed in relation to the recent literature