RESUMEN
Melanocytic tumors of the central nervous system (CNS) such as meningeal melanoma are exceedingly rare tumours derived from leptomeningeal melanocytes. We report an illustrative case of a previously healthy 47-year-old male who presented with tonic-clonic seizure. Magnetic resonance imaging (MRI) with contrast demonstrated a homogenously enhancing right temporal extra-axial lesion. The patient was stabilized on anti-epileptic medications and dexamethasone prior to proceeding with complete surgical resection of the lesion. Intraoperatively, the lesion was heavily pigmented with invasion of the surrounding dura and skull. Histopathology revealed a poorly differentiated neoplasm with nuclear atypia and melanin-containing cells with strong SOX10 and variable S100 positivity. Computed tomography (CT) of the chest, abdomen, and pelvis showed no metastatic disease, and molecular profiling was negative including absent BRAF mutation. He began checkpoint inhibitor therapy and subsequently developed cerebral venous sinus thrombosis managed with anticoagulation. Sixteen months post-operatively, he was neurologically intact, working full-time, and had resumed immunotherapy. We systematically reviewed the literature on primary intracranial malignant melanoma (PIMM) with the goal of understanding the prognosis and best treatment options for this disease. Our systematic review produced 82 articles (118 unique cases) of PIMM. The average age at diagnosis was 45.9 years (95% CI:42.9-48.9), and headache (54.2%) was the most common initial presentation. Eighty-nine percent of patients had primary surgical resection, and 41.0% of these individuals experienced a recurrence with a mean time to recurrence of 19.6 months (95% CI:6.95-32.23). Adjuvant therapy was administered in 65.7% of surgically resected patients; including radiotherapy, chemotherapy, immunotherapy, or a combination. In summary, PIMM is a rare tumour that can appear radiographically similar to meningioma. The results of our systematic review demonstrate that surgical resection remains the mainstay of therapy for best long-term prognosis.
RESUMEN
OBJECTIVE: Endoscopic third ventriculostomy and choroid plexus cauterization (ETV+CPC) is a novel procedure for infant hydrocephalus that was developed in sub-Saharan Africa to mitigate the risks associated with permanent implanted shunt hardware. This study summarizes the hydrocephalus literature surrounding the ETV+CPC intraoperative abandonment rate, perioperative mortality rate, cerebrospinal fluid infection rate, and failure rate. METHODS: This systematic review and meta-analysis followed a prespecified protocol and abides by Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A comprehensive search strategy using MEDLINE, EMBASE, PsychInfo, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, Scopus, and Web of Science was conducted from database inception to October 2019. Studies included controlled trials, cohort studies, and case-control studies of patients with hydrocephalus younger than 18 years of age treated with ETV+CPC. Pooled estimates were calculated using DerSimonian and Laird random-effects modeling, and the significance of subgroup analyses was tested using meta-regression. The quality of the pooled outcomes was assessed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. RESULTS: After screening and reviewing 12,321 citations, the authors found 16 articles that met the inclusion criteria. The pooled estimate for the ETV+CPC failure rate was 0.44 (95% CI 0.37-0.51). Subgroup analysis by geographic income level showed statistical significance (p < 0.01), with lower-middle-income countries having a lower failure rate (0.32, 95% CI 0.28-0.36) than high-income countries (0.53, 95% CI 0.47-0.60). No difference in failure rate was found between hydrocephalus etiology (p = 0.09) or definition of failure (p = 0.24). The pooled estimate for perioperative mortality rate (n = 7 studies) was 0.001 (95% CI 0.00-0.004), the intraoperative abandonment rate (n = 5 studies) was 0.04 (95% CI 0.01-0.08), and the postoperative CSF infection rate (n = 5 studies) was 0.0004 (95% CI 0.00-0.003). All pooled outcomes were found to be low-quality evidence. CONCLUSIONS: This systematic review and meta-analysis provides the most comprehensive pooled estimate for the ETV+CPC failure rate to date and demonstrates, for the first time, a statistically significant difference in failure rate by geographic income level. It also provides the first reported pooled estimates for the risk of ETV+CPC perioperative mortality, intraoperative abandonment, and CSF infection. The low quality of this evidence highlights the need for further research to improve the understanding of these critical clinical outcomes and their relevant explanatory variables and thus to appreciate which patients may benefit most from an ETV+CPC. Systematic review registration no.: CRD42020160149 (https://www.crd.york.ac.uk/prospero/).
RESUMEN
Intracranial Masson tumor (intravascular papillary endothelial hyperplasia, IPEH) is a benign lesion that is thought to originate from a reactionary process in response to compromised blood flow. IPEH may be classified into one of three subtypes based on etiology as it may result from the excessive proliferation of endothelial cells within a normal vessel (primary), vascular malformation (type II), or organized hematoma (type III). We report the case of a 79-year-old woman who presented with confusion, gait instability, and urinary incontinence. Neuroimaging revealed a hemorrhagic lesion within the right lateral ventricle, which was successfully resected. To our knowledge, this is the first reported case of an intraventricular IPEH and 33rd case of primary intracranial IPEH. We further performed a systematic review of the literature on all prior type I intracranial IPEH cases and discuss the importance of long-term follow-up in intracranial IPEH.