World Neurosurg
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Review Case Reports
Bilateral Recurrent Dysplastic Cerebellar Gangliocytoma (Lhermitte Duclos Disease) in Cowden Syndrome: A Case Report and Literature Review.
Dysplastic gangliocytoma (Lhermitte-Duclos disease [LDD]) typically presents as a PTEN (phosphatase and tensin homolog)-positive, insidious unilateral mass of the cerebellar cortex. Patients can present with symptoms of increased intracranial pressure. Magnetic resonance imaging (MRI) will reveal a characteristic laminar/tigroid appearance. Surgical management has been superior to conservative measures for symptomatic lesions. The outcomes for bilateral craniotomy have not yet been described. ⋯ To the best of our knowledge, the present case is the first reported case of bilateral cerebellar hemisphere LDD ultimately managed by temporally dissociated bilateral surgical resections. Our patient was unique in that he had undergone surgery for resection of LDD, followed by a second surgery for contralateral progression 8 years later. The therapeutic options to prevent recurrence are limited, although temozolomide did seem to impede progression. Symptomatic patients will typically benefit most from surgical intervention. Given the strong association between adult LDD and Cowden syndrome, maintaining close follow-up care and possible surveillance imaging will be essential.
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Review Case Reports
Dysembryoplastic Neuroepithelial Tumors: What You Need to Know.
An updated and comprehensive review on dysembryoplastic neuroepithelial tumor (DNET) focusing on differential diagnosis, atypical presentation, seizure outcome, and risk of malignant transformation. ⋯ Advanced MRI techniques are fundamental in the differential diagnosis for DNET versus other low-grade gliomas. Immuno-phenotype assessment and search for fibroblast growth factor receptor 1 and BRAF V600E mutations limit the risk of misdiagnoses. A gross total tumor removal is generally associated with a seizure-free outcome. Recurrences and malignant transformations may rarely follow, legitimizing MRI surveillance in cases of subtotal tumor resection.
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Evidence continues to emerge regarding the inverse relationship between high neurointerventional case volume and complication rates, leading several medical/surgical societies to recommend minimum volumes for specific procedures. Recent data suggest few centers are meeting these requirements. We report a single center's neurointerventional complication rates with associated case volumes, along with a review of the literature. ⋯ We provided a single-center experience of the relationship between neurointerventional procedural case volume and complication rates in the growth phase of our center's establishment. We demonstrated that as our center was being developed, specific procedural staffing measures allowed proficiency maintenance, acquisition of new techniques, and complication avoidance, whereas specific case volumes crossed the suggested thresholds as defined in the literature.
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Review Case Reports
Skull Base Chondrosarcoma Caused by Ollier Disease: A Case Report and Literature Review.
Ollier disease (OD) is a rare, nonhereditary bone disease that is characterized by the presence of multiple enchondromatosis (3 or more) with a typical asymmetric distribution which is mainly confined to the appendicular skeleton. OD's most serious complication is the transformation of an enchondroma into chondrosarcoma. The most common sites for chondrosarcoma are in the pelvic and shoulder bones, the superior metaphyseal and diaphyseal regions of the extremities. However, the cranium is an extremely rare site for chondrosarcoma because of OD. ⋯ Intracranial chondrosarcoma caused by OD is extremely rare but should be considered in the differential diagnosis when primary chondrosarcoma is diagnosed. Preoperative diagnosis is challenging, and definitive diagnosis requires immunohistochemical examination and systematic examination of the body. Surgical resection is the most effective therapy for rapid relief of symptoms. For patients with OD with normal intracranial MRI, long-term follow-up is necessary.
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Diagnosis of cerebrospinal fluid (CSF) leaks traditionally involves laboratory testing of markers and appropriate imaging. Surgical localization can be difficult, and the inability to accurately localize skull base defects leads to increased rates of repair failure and complications. Many imaging techniques localizing and identifying CSF leaks have been proposed. Comparisons of current and investigational imaging techniques used to localize CSF leaks are reviewed. ⋯ Although radionuclide cisternography has been the historical standard, recent imaging techniques have emerged considering the low sensitivity. Computed tomography cisternography with contrast also has low sensitivity, even in active leaks. Although high-resolution computed tomography is commonly the initial study of choice, magnetic resonance imaging methods, particularly 3-dimensional imaging, may prove to be a more sensitive study of choice. Computed tomography/magnetic resonance imaging combination methods may show promise in localizing CSF leaks. Stratifying by status and etiology may be an important determinant. Further studies investigating various imaging techniques for localizing CSF leaks are needed.