scholarly journals P.050 NICO-assisted neuroendoscopic management of enlarging subependymal giant cell astrocytoma in tuberous sclerosis complex: a case report

Author(s):  
A Dakson ◽  
P McNeely

Background: Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous syndrome classically associated with mental disability, seizure disorder and adenoma sebaceum, among other anomalies. One of the major causes of mortality and morbidity in adults is the exclusive occurrence of subependymal giant cell astrocytoma (SEGA) which responds in at least 35% of cases to everolimus, mTOR inhibitor. However, drug treatment is associated with 33% rate of adverse events and requires long-term treatment Methods: In this report, we present a case of 49-year old female with TSC and a left enlarging SEGA that was approached endoscopically in order to minimize morbidity associated with open surgical approaches. Results: The use of NICO Myriad system is described in this case to achieve successful tumor debulking without post-operative neurologic morbidity. Conclusions: This report reveals the value of minimally invasive neuroendoscopic techniques in the management of challenging intraventricular tumors while avoiding injury to crucial deep venous structures.

2019 ◽  
Vol 10 ◽  
Author(s):  
Anna C. Jansen ◽  
Elena Belousova ◽  
Mirjana P. Benedik ◽  
Tom Carter ◽  
Vincent Cottin ◽  
...  

2014 ◽  
Vol 30 (9) ◽  
pp. 1192-1195 ◽  
Author(s):  
Shinobu Fukumura ◽  
Toshihide Watanabe ◽  
Rumiko Takayama ◽  
Kimio Minagawa ◽  
Hiroyuki Tsutsumi

2015 ◽  
Vol 16 (2) ◽  
pp. 134-137 ◽  
Author(s):  
Thomas L. Beaumont ◽  
Jakub Godzik ◽  
Sonika Dahiya ◽  
Matthew D. Smyth

The authors report the case of a 14-year-old male with a subependymal giant cell astrocytoma (SEGA) that occurred in the absence of tuberous sclerosis complex (TSC). The patient presented with progressive headache and the sudden onset of nausea and vomiting. Neuroimaging revealed an enhancing left ventricular mass located in the region of the foramen of Monro with significant mass effect and midline shift. The lesion had radiographic characteristics of SEGA; however, the diagnosis remained unclear given the absence of clinical features of TSC. The patient underwent gross-total resection of the tumor with resolution of his symptoms. Although tumor histology was consistent with SEGA, genetic analysis of both germline and tumor DNA revealed no TSC1/2 mutations. Similarly, a comprehensive clinical evaluation failed to reveal any clinical features characteristic of TSC. Few cases of SEGA without clinical or genetic evidence of TSC have been reported. The histogenesis, genetics, and clinical approach to this rare lesion are briefly reviewed.


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