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Background  Resection of skull base malignancies poses complex pathological and treatment-related morbidities. Recent technological advancements of endoscopic endonasal surgery (EES) offer the ability to reexamine traditional treatment paradigms with endoscopic procedures. The utility of EES was quantitatively examined in a longitudinal series with attention to morbidities and postoperative outcomes. Methods  A single-center retrospective review was performed of all malignant sinonasal tumors from 2010 to 2018. Patients with purely EES were selected for analysis. Disease features, resection extent, complications, adjuvant treatment, recurrence, and survivability were assessed. Despite the mixed pathological cohort, analysis was performed to identify technical aspects of resection. Results  A total of 68 patients (47.6% males and 52.4% females, average age: 60.3 years) were included. A diversity of histotypes included clival chordoma (22.1%), olfactory neuroblastoma (14.7%), squamous cell carcinoma (11.8%), and adenoid cystic carcinoma (11.8%). Gross total resection (GTR) was achieved in 83.8% of cases. Infection (4.4%) and cerebrospinal fluid leak (1.5%) were the most common postoperative complications. Total 46 patients (67.6%) underwent adjuvant treatment. The average time between surgery and initiation of adjunctive surgery was 55.7 days. Conclusion  In our 8-year experience, we found that entirely endoscopic resection of mixed pathology of malignant skull base tumors is oncologically feasible and can be accomplished with high GTR rates. There may be a role for EES to reduce operative morbidity and attenuate time in between surgery and adjuvant treatment, which can be augmented through recent mixed reality platforms. Future studies are required to systematically compare the outcomes with those of open surgical approaches. [ABSTRACT FROM AUTHOR]

Objective  This article aims to characterize 14 patients who underwent purely endoscopic surgical debridement of acute invasive skull base fungal rhinosinusitis, and to evaluate postoperative outcomes and risk for recurrence. Design  Retrospective cohort study. Setting  Tertiary single-institution neurosurgery department. Participants  We performed a retrospective analysis of all patients with skull base fungal infections treated with a purely endoscopic surgical approach at Mount Sinai Hospital from 1998 to 2018. Main Outcome Measures  Clinical presentation, number of recurrences, and mortality rate. Results  The most common underlying medical comorbidities were hematologic malignancy in 8 (57.1%) patients and poorly controlled diabetes mellitus in 7 (50%) patients. Presenting symptoms included headache (50%), eye pain (35.7%), facial pain (28.6%), visual changes (21.4%), and nasal congestion (14.3%). The fungal organisms identified on culture were Aspergillus (42.9%), Mucorales (28.6%), Fusarium (14.3%), Penicillium (7.1%), and unspecified (7.1%). Eight (57.1%) patients developed recurrence and required multiple surgical debridements. Patients who had only a hematologic malignancy were more likely to require multiple surgical debridements compared with those who did not have a hematologic malignancy or those who had both hematologic malignancy and underlying diabetes mellitus (p  = 0.03). The mortality rate from surgery was 42.9%. Conclusion  Surgical endoscopic intervention is an option for definitive management of acute invasive skull base fungal rhinosinusitis; however, postoperative mortality and risk of recurrence requiring additional surgical interventions remains high. Patients with hematologic malignancy may be more susceptible to recurrent infection requiring multiple surgical debridements. We recommend early aggressive multimodal treatment. Multiple debridements may be warranted in most cases; close clinical surveillance is needed during neurosurgical intervention. [ABSTRACT FROM AUTHOR]

Background: Biliary tract cancers (BTCs) are highly fatal malignancies that make up less than 1% of all cancers. BTC is often diagnosed at an unresectable stage; surgical resection remains the only definitive treatment. Brain metastases (BMs) from BTC are extremely rare, and few studies on patients with BMs from BTC exist. The aim of this study was to identify clinical characteristics associated with poor prognosis for patients with BMs from BTC. Materials and Methods: We performed a retrospective review of electronic medical records for patients with BMs from BTC managed at Mount Sinai Hospital from 2000 to 2017. Data on patient characteristics, magnetic resonance imaging findings, treatment regimens, and clinical outcomes were analyzed. Results: We identified 1,910 patients with BTC. Nine patients developed BMs, with an incidence of 0.47%. Of these nine patients, six had intrahepatic cholangiocarcinoma, two had extrahepatic cholangiocarcinoma, and one had gallbladder cancer. Six (66.7%) patients had one BM, one (11.1%) patient had two BMs, and two (22.2%) patients had three or more BMs. Four (44.4%) patients underwent BM resection, and seven (77.8%) received BM radiation. Median overall survival from time of BM diagnosis was 3.8 months (95% confidence interval 0.1–16.9). Conclusion: Development of BMs from BTC is rare; however, prognosis is less than 4 months. BM diagnosis can occur within 2 years of primary diagnosis. As targeted therapeutics emerge, future studies ought to focus on identifying genomic BM markers associated with BTC subtypes. Implications for Practice: In the largest retrospective study of biliary tract cancer brain metastases, the clinical presentation and outcomes are reported of nine patients with an extremely rare clinical entity. The genomic literature and potential therapeutic targets for these patients with limited treatment options is comprehensively and exhaustively discussed. A better understanding of clinical outcomes of patients with biliary tract cancer who develop brain metastases. This article expands on the existing literature by describing outcomes for patients who developed brain metastases from biliary tract cancer within the context of the genomic era. [ABSTRACT FROM AUTHOR]

Background: Pilomyxoid astrocytoma (PMA) has recently been accepted as an aggressive variant of pilocytic astrocytoma with distinct histopathological features. PMAs have been frequently described in the pediatric population with a predilection for the hypothalamic/chiasmatic region. Case Description: A 72-year-old African American male presented with 6 months of memory loss, difficulty expressing himself, and a progressively worsening gait. Magnetic resonance imaging of the brain demonstrated a heterogeneously enhancing cystic mass centered within the cerebellar vermis with mass effect on the fourth ventricle and ventriculomegaly. The patient underwent placement of a ventriculoperitoneal shunt followed by a surgical resection of the lesion, which after immunohistopathologic evaluation, was diagnosed as a World Health Organization grade II PMA. The patient refused further treatment of the lesion and expired 11 months after initial symptom presentation and 4 months after surgery. Conclusion: To our knowledge, this is the first report of PMA of the cerebellar vermis in a previously unreported age group. This case report describes the natural history of this type of tumor in a patient who refused adjuvant therapy following surgical resection. [ABSTRACT FROM AUTHOR]

Cerebrospinal fluid leaks of the temporal bone are rare, often occult, and sometimes challenging to localize and repair. This is a retrospective study of eight patients with spontaneous cerebrospinal fluid leak and six patients with cerebrospinal fluid leak or encephalocele discovered during chronic ear surgery who were treated in a tertiary medical center over a 5-year period. All received preoperative temporal bone computed tomography, and six also underwent magnetic resonance imaging, one computed tomography cisternography, and one radionuclide cisternography. All patients initially underwent a transmastoid surgical approach. Additional exposure was necessary in three patients; two underwent middle fossa craniotomy and another required minicraniotomy. Primary surgical repair was successful in six of the eight patients with spontaneous leaks and in all six chronic ear patients. Both recurrences required intradural middle fossa repair. An individualized approach should be taken for repair of temporal bone cerebrospinal fluid leaks. In this series, most were successfully repaired in a single stage using a transmastoid or combined approach. The transmastoid approach provides information about the precise size and location of the dural defect. A primary transcranial approach is needed for defects that are multiple, located in the petrous apex, and in revision cases.

The persistent otic artery is a rare remnant of early fetal development that, to our knowledge, has been convincingly documented only once before with angiography. Other persistent vessels such as the trigeminal, hypoglossal, and proatlantal intersegmental arteries have been found to be associated with a variety of vascular anomalies. We believe we present the second case of a persistent otic artery documented by means of angiography and the first such case associated with multiple intracranial aneurysms.