World Neurosurg
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Intracranial myxoid mesenchymal tumors (IMMTs) carrying an EWSR1-CREB gene family fusion are extremely rare and have only been identified in 10 previous reports. All but one has been found in children or young adults. Although there appear to be similarities to a myxoid variant of angiomatoid fibrous histiocytoma (AFH), clear histologic differences exist that appear to distinguish IMMTs as a distinct and novel entity. Previous reports have lacked detailed long-term follow-up and recommendations regarding treatment approach. ⋯ Intracranial myxoid mesenchymal tumor is a novel and rare entity that has been previously identified in only 10 cases. This case is particularly remarkable because it is only the second IMMT case to occur in a middle-aged adult and shares striking similarities in clinical presentation to the previously reported case. Given the aggressive recurrence seen with the presented case, we recommend the treatment plan to be surgical resection followed by adjuvant radiation therapy.
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Case Reports
De novo cavernoma formation in a patient with Cowden syndrome and Lhermitte-Duclos disease.
Cowden syndrome (CS) is an autosomal dominant genodermatosis with a predisposition for the development of multiple cancers, benign hamartomas, and extracranial vascular malformations. Rarely, intracranial lesions like meningiomas and vascular malformations can also be present with CS. These vascular malformations include developmental venous anomalies, arteriovenous fistulae and cavernomas. Most cases of cavernomas are thought to be congenital, although in recent literature they have been shown to occur de novo with other conditions (e.g., other vascular malformations, trauma, postcranial surgery, viral infection, and genetic disorders). ⋯ We present a case of a patient with CS and LDD who had de novo cavernoma development several years after the initial diagnosis, as well as a review of the literature. We highlight the need of surveillance neuroimaging for patients with CS, as there is the risk of new development of vascular abnormalities (particularly cavernomas).
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Subarachnoid neurocysticercosis (NCC) is a form of NCC with cysticerci located in the subarachnoid space. This form of NCC can cause general and focal neurologic symptoms, and sometimes requires surgical intervention as a treatment. ⋯ At 8-month follow-up, the patient reported no recurrence of symptoms. To our knowledge, this is the first case reported to surgically manage hemifacial spasm secondary to NCC arachnoiditis.
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We sought to analyze the clinical data and imaging features from a rare case presenting an intravertebral mobile nerve sheath tumor of the lumbar spine, review the relevant literature, discuss the imaging features and possible causes of the tumor, and propose preventive measures and solutions. ⋯ Intravertebral mobile nerve sheath tumors are rare, and the marked distortion and elongation of the carrier nerve seen on MRI are important imaging features of this disease. The possible causes of tumor movement include tumor texture, location, positional changes, and altered cerebrospinal fluid dynamics. Acute changes in intraabdominal pressure caused by forceful defecation may be a high-risk factor for tumor migration. Multiple preoperative MRIs to localize the tumor are particularly important.
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Iatrogenic cerebrospinal fluid (CSF) leaks are associated with serious sequelae. We analyzed the causes, complications, treatments, and outcomes of iatrogenic CSF leaks. Furthermore, the comorbidities and outcomes between timely and delayed repairs of iatrogenic CSF leaks were also compared. ⋯ Delayed intervention of CSF leak after transsphenoidal surgery and craniotomy was observed, increasing the risks of meningitis. Timely intervention with grafts based on the defect sites and patient prior surgical history is crucial for avoiding life-threatening comorbidities.