Management of large intraventricular meningiomas with minimally invasive port technique: a three-case series.

The use of minimally invasive transcranial ports for the resection of deep-seated lesions has been shown to be safe and effective. To date, most of the literature regarding the tubular retractors used in brain surgery is comprised of individual case reports that describe the successful resection of deep-seated lesions such as thalamic pilocytic astrocytomas, colloid cysts in the third ventricle, hematomas, and cavernous angiomas. The authors describe their experience using a tubular retractor system with three different cases involving large intraventricular meningiomas and examine radiographic and patient outcomes. A single-institution, retrospective case series was performed from a skull base database. Patients who underwent resection of intraventricular > 4-cm meningiomas with port technology were identified. The authors reviewed three cases to illustrate the feasibility of minimal access port surgery for the resection of these lesions. Complete resection was achieved in all cases. None of the patients developed permanent neurological deficits. There were no major complications related to surgery and no mortalities. Good clinical and surgical outcomes for atrium meningiomas can be achieved through the minimally invasive port technique and tumor size does not appear to be a limitation.

Generalized Dystonia Treated With Deep Brain Stimulator: An Institutional Single Surgeon Experience.

Introduction Dystonia can cause severe disability when left untreated. Once a patient has exhausted medical management, surgical intervention may be the only treatment option. Although not curative, deep brain stimulation has been shown to be beneficial for patients affected by this condition. Our study sought to review patients undergoing deep brain stimulation for medically refractory dystonia to assess outcomes. Methods Our institution's operative database was reviewed retrospectively for all patients undergoing deep brain stimulator placement over the last six years. These medical records were reviewed for the severity of dystonia preoperatively and followed postoperatively for 24 months, focusing on the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). Patients with less than two-year postoperative follow-up were excluded from the study. The patients were further stratified by age into Group A, consisting of patients less than 40 years old, and Group B, patients greater than or equal to 40 years old. Other attributes such as age, sex, age of disease onset, disease duration at the time of surgery, genetic tests for dystonia-related genes, and any complication associated with surgery were also reviewed. Results Four hundred fifty-five operative cases for deep brain stimulator placement were reviewed, and 16 patients met inclusion criteria for the study. The mean age for our patient cohort was 43.75 years, with four males and 12 females. The average time from the age of disease onset to time of surgery was 9.7 years for Group A and 10.8 years for Group B; the overall average was 10.3 years. All patients had globus pallidus interna (GPi) as their surgical target. The first incidence of a statistically significant decrease in BFMDRS score was noted at three months postoperatively (p<0.001) when compared to preoperative values. Fourteen patients in our cohort underwent preoperative genetic testing for DYT gene mutations, out of which four were found to have a mutation. Conclusion Our review of outcomes for primary generalized dystonia at our institution found that deep brain stimulator targeting the GPi is safe and effective. We found an overall 88% response rate with younger patients (< 40-year-old) showing a better response at two years than older patients.

Improving Function in Cavernous Sinus Meningiomas: A Modern Treatment Algorithm.

Background: The efficacy and safety of radiosurgery led to paradigm shift in the management of cavernous sinus meningiomas. Nevertheless, patients are still significantly affected by cranial nerve deficits related to the mass effect of these tumors. Our management strategy involves the combination of a functional surgical decompression followed by radiation therapy. Methods: We reviewed a single institution's cohort of patients who underwent endoscopic endonasal decompression (EED) for symptomatic meningiomas primarily involving the cavernous sinus (CS) from 2010 to 2016. The preoperative neuro-ophthalmological exam was compared to the 1- and 6-month postoperative exams. The patient's length of hospital stay, complications, and radiological and clinical follow-up were noted. Results: A total of 17 patients underwent EED for CS meningiomas that fit our radiological criteria. The final outcome at the 6-month visit showed five patients (62.5%) with normalization of deficit and three patients (37.5%) with partial improvement of the CNII deficit. Out of the 12 patients who had cavernous sinus cranial nerves (CSCN) deficits, the final outcome at the 6-month visit showed four patients (33.33%) with normalization of deficit, seven patients (58.3%) with partial improvement, and one patient (8.33%) with no improvement. There were no intraoperative complications. Conclusion: The EED for CS meningiomas is a valuable technique when addressing acute/subacute CNII and CSCN deficits. This conservative surgical approach showed good functional outcomes, low morbidity, and low complication rates. However, it does not exempt the need for radiosurgery/radiation therapy for control of tumor growth.

Pituitary carcinomas: review of the current literature and report of atypical case.

Introduction: Pituitary carcinomas are poorly understood, rare entities. They are distinguished from adenomas not by histopathological features but rather by the presence of metastases.Objective: We discuss the diagnosis, mechanism of dissemination and pathogenesis based on a review of the literature and illustrated by a singular case.Case Report: A 59-year-old male presented with a dural-based posterior fossa lesion. He had been diagnosed with a pituitary chromophobe adenoma 43 years earlier that was treated at the time with surgery and radiation therapy. A presumptive diagnosis of a radiation-induced meningioma was made and surgery was recommended. At surgery the tumour resembled a pituitary adenoma. Histopathology, laboratory findings, and the patient's medical history confirmed the final diagnosis of a prolactin-secreting pituitary carcinoma. To our knowledge, this is the longest reported interval between the pituitary adenoma and metastatic lesion diagnosis (43 years).Conclusion: Management should be tailored to individual patient and may include a combination of treatments (surgery, radiation therapy, chemotherapy, and hormone-targeted therapy). Functionally active tumours may be monitored with hormone levels as tumour markers.

Infradentate Approach to the Fourth Ventricle.

BACKGROUND: The use of minimally invasive transcranial ports for the resection of deep-seated lesions has been shown to be safe and effective for supratentorial lesions. The routine use of this surgical modality for posterior fossa masses has not been well established in the literature. In particular, fourth ventricular tumors are not the typical target for neuro-port surgery because of potential injury to the dentate nucleus. OBJECTIVE: To describe the use of a tubular retractor system to reach the fourth ventricle while sparing the cerebellar vermis and the dentate nucleus. Three cases illustrations are presented. METHODS: Surgical access to the fourth ventricle was developed sparing the cerebellar vermis and the dentate nucleus. The authors reviewed 3 cases to illustrate the feasibility of minimal access transcerebellar port surgery for the resection of these lesions using an infradentate access. RESULTS: None of the patients developed new neurological deficits and the pathology was successfully resected in all cases. There were no major complications related to surgery and no mortalities. CONCLUSION: The infradentate approach obviates the need for traditional approaches to the fourth ventricle, thus making this challenging target in the posterior fossa more accessible to neurosurgeons. The authors observed successful removal of lesions involving the fourth ventricle while avoiding any associated morbidity or mortality.

Somatic SMARCB1 Mutation in Sporadic Multiple Meningiomas: Case Report.

Background: Multiple intracranial meningiomas account for <10% of all meningiomas. Familial multiple meningiomas have been linked to germline mutations in two genes: neurofibromatosis type 2 (NF2) and SWIch/Sucrose Non-Fermentable (SWI/SNF)-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1 (SMARCB1). Sporadic multiple meningiomas have been associated with somatic NF2 mutations and, to date, there has been no case related to somatic SMARCB1 mutations. Here, we describe the first case. Case Report: A 45-year-old female suffered a head trauma while snowboarding. Subsequent to her injury, she experienced persistent headache, nausea, vomiting, dizziness, and flashing lights in the right eye. Magnetic resonance imaging (MRI) of her brain revealed multiple intracranial meningiomas. She underwent a two-staged craniotomy to remove frontal/parietal/temporal and occipital extra-axial tumors. Pathology confirmed the masses as meningiomas, WHO Grade I. Tumor genetic testing was positive for SMARCB1 mutation but blood genetic testing was negative for SMARCB1 mutation. Conclusion: In sporadic multiple meningiomas, somatic NF2 mutations are usually the suspected genetic alternations. Our case illustrates that somatic SMARCB1 mutation is another genetic risk factor for sporadic multiple meningiomas, albeit rare.

A modern series of subdiaphragmatic craniopharyngiomas.

OBJECTIVE: The endoscopic endonasal approach has been proposed as a primary surgical strategy for select craniopharyngiomas. However, those tumors that arise from the sella have not been classified with the other craniopharyngioma subtypes in terms of surgical nuances, intraoperative findings, and postoperative outcomes. The authors describe their experience with a select subtype of craniopharyngioma arising within the sella subjacent to the diaphragma sellae and refer to these tumors as type 0. METHODS: After obtaining IRB approval, three institutions retrospectively reviewed their data collected from 2005 to 2017. Patients eligible for inclusion in the study were those who had tumors that originated from the sella inferior to the diaphragma sellae. Demographic, clinical, radiological, surgical, and follow-up data were examined and analyzed. RESULTS: Twenty-eight patients (average age 19.3 years, range 3-60 years) were included in this study. Sixteen patients (57%) were younger than 18 years of age. All patients had characteristic imaging features of an expanded sella. Seventy-five percent of the patients presented with some form of visual field deficit (89% had radiographic optic apparatus compression) and 39% with hypopituitarism. The average maximal tumor diameter in the axial, coronal, or sagittal plane was 3.1 cm. Gross-total resection was achieved in 82% of the patients. Twenty-one percent of patients experienced an iatrogenic complication, and there were only two cases (7%) of postoperative cerebrospinal fluid (CSF) leakage. Only two patients (7%) required the use of a nasoseptal flap as part of their original reconstruction. Pathology was uniformly the adamantinomatous subtype. Postoperative objective visual outcomes were improved in 71% of the patients with visual symptoms or visual field deficits on presentation and stable in 24%. Mean follow-up was 45.1 months (range 3-120 months) with an 18% recurrence rate at a mean of 44.4 months (range 10-84 months). One patient was lost to follow-up. Thirty-six percent of patients received postoperative radiation to treat recurrence or residual tumor. Endocrine data are also presented. CONCLUSIONS: Craniopharyngiomas that originate within the sella below the diaphragma sellae are a select subtype characterized by 1) an enlarged sella, 2) an intact diaphragma sellae at surgery, and 3) an adamantinomatous pathology. These tumors can be treated transnasally without the absolute need for neurovascular flap reconstruction, as there is a low risk of CSF leakage.

Endoscopic trans-sphenoidal surgery for petroclival and clival meningiomas.

Petroclival meningiomas are challenging lesions to manage independently of the selected surgical approach and are unique tumors in the type of pathological displacement of the surrounding anatomy. They also represent one of the most controversial entities with regard to approach selection, especially when deciding between an open versus endonasal route. When choosing an approach to the intradural portion of the petroclival region, the location of critical neurovascular structures relative to the lesion must be anticipated, including the abducens and trigeminal nerves. The role of the expanded endoscopic endonasal approach (EEA) in the management of ventral posterior fossa meningiomas is still restricted due to limited surgical indications in selected cases. It is a safe alternative for the rare cases of meningiomas with most part of its dural base at the midline clival region, and it may be used as solely or combination of other approaches. Thus, appropriate case selection may optimize the advantages of the approach and reduce morbidity of this complex pathology. We discuss in this review article the main advantages and limitations of the EEA for clival and petroclival meningiomas.

Endoscopic Endonasal Approach for Removal of Tuberculum Sellae Meningiomas.

Tuberculum sellae meningiomas are challenging lesions; their critical location and often insidious growth rate enables significant distortion of the superjacent optic apparatus before the patient notices any visual impairment. This article describes the technical nuances, selection criteria and complication avoidance strategies for the endonasal resection of tuberculum sellae meningiomas. A stepwise description of the surgical technique is presented; indications, adjuvant technologies, pitfalls and the relevant anatomy are also reviewed. Tuberculum sellae meningiomas may be safely and effectively resected through the endonasal route; invasion of the optic canals does not represent a limitation.

The Endoscopic Endonasal Approach for Removal of Petroclival Chondrosarcomas.

Chondrosarcomas of the skull base are rare, locally invasive tumors that typically arise in the petroclival region, from degenerated chondroid cells located within the synchondrosis. Given their usually slow growth rate, they are capable of reaching sizable dimensions, promoting bone erosion and significant displacement of neurovascular structures before causing symptomatology that will eventually lead to diagnosis; cranial neuropathies and headaches are common complaints. This article discusses the pertinent surgical anatomy, patient selection criteria, technical nuances and complication management of the endonasal resection of skull base chondrosarcomas.

Nuances in the treatment of malignant tumors of the clival and petroclival region.

Introduction Malignancies of the clivus and petroclival region are mainly chordomas and chondrosarcomas. Although a spectrum of malignancies may present in this area, a finite group of commonly encountered malignant pathologies will be the focus of this review, as they are recognized to be formidable pathologies due to adjacent critical neurovascular structures and challenging surgical approaches. Objectives The objective is to review the literature regarding medical and surgical management of malignant tumors of the clival and petroclival region with a focus on clinical presentation, diagnostic identification, and associated adjuvant therapies. We will also discuss our current treatment paradigm using endoscopic, open, and combined approaches to the skull base. Data Synthesis A literature review was conducted, searching for basic science and clinical evidence from PubMed, Medline, and the Cochrane Database. The selection criteria encompassed original articles including data from both basic science and clinical literature, case series, case reports, and review articles on the etiology, diagnosis, treatment, and management of skull base malignancies in the clival and petroclival region. Conclusions The management of petroclival malignancies requires a multidisciplinary team to deliver the most complete surgical resection, with minimal morbidity, followed by appropriate adjuvant therapy. We advocate the combination of endoscopic and open approaches (traditional or minimally invasive) as required by the particular tumor followed by radiation therapy to optimize oncologic outcomes.

Nuances in the Treatment of Malignant Tumors of the Clival and Petroclival Region

Introduction: Malignancies of the clivus and petroclival region are mainly chordomas and chondrosarcomas. Although a spectrum of malignancies may present in this area, a finite group of commonly encountered malignant pathologies will be the focus of this review, as they are recognized to be formidable pathologies due to adjacent critical neurovascular structures and challenging surgical approaches. Objectives: The objective is to review the literature regarding medical and surgical management of malignant tumors of the clival and petroclival region with a focus on clinical presentation, diagnostic identification, and associated adjuvant therapies. We will also discuss our current treatment paradigm using endoscopic, open, and combined approaches to the skull base. Data Synthesis A literature review was conducted, searching for basic science and clinical evidence from PubMed, Medline, and the Cochrane Database. The selection criteria encompassed original articles including data from both basic science and clinical literature, case series, case reports, and review articles on the etiology, diagnosis, treatment, and management of skull base malignancies in the clival and petroclival region. Conclusions: The management of petroclival malignancies requires a multidisciplinary team to deliver the most complete surgical resection, with minimal morbidity, followed by appropriate adjuvant therapy. We advocate the combination of endoscopic and open approaches (traditional or minimally invasive) as required by the particular tumor followed by radiation therapy to optimize oncologic outcomes...