Surgical resection of subependymal giant cell astrocytomas (SEGAs) and changes in SEGA-related conditions: a US national claims database study

Sun, Peter; Krueger, Darcy A.; Liu, Jamae; Guo, Amy; Rogerio, Jaqueline Willemann; Kohrman, Michael

doi:10.1185/03007995.2012.658908

Cited by 17 publications

(7 citation statements)

References 19 publications

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“…Data on the efficacy and safety of SEGA surgery, as well as on outcome, are very limited and variable [90][91][92][93]. SEGA surgery is associated with significant morbidity such as hemiparesis, hydrocephalus, intracranial bleeding, infection, precocious puberty, neuropathic headache and cognitive decline, and mortality (6.2 %).…”

Section: Extrarenal Manifestationsmentioning

confidence: 99%

Tuberous sclerosis complex: the past and the future

2014

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Renal lesions represent the second most significant cause of morbidity and mortality in patients with tuberous sclerosis complex (TSC). Recent advances in the understanding of the pathophysiology of TSC have led to the exploration of new potential therapeutic targets. Clinical trials with mammalian target of rapamycin (mTOR) inhibitors have demonstrated promising results for several indications, such as renal angiomyolipoma, subependymal giant cell astrocytoma, lymphangioleiomyomatosis and facial angiofibromas. Currently, there is a scarcity of natural history data and randomized, placebo-controlled clinical trials on TSC. Recently, however, recommendations for the diagnostic criteria, surveillance, and management of TSC patients have been updated. This review focuses on these novel recommendations and highlights the need for multidisciplinary follow-up of this multi-systemic disease.

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Section: Extrarenal Manifestationsmentioning

confidence: 99%

Tuberous sclerosis complex: the past and the future

2014

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“…Based on US Food and Drug Administration's approval letter for everolimus 19 , mTOR inhibitor and SEGA surgery have their own unique and complementary roles in SEGA treatment. In our recent real-world study with one year post-SEGA observation 20 , only about 27.8% of TSC patients with SEGA had a SEGA surgery and about 72.2% did not have SEGA surgery 20 . We believe that a significant portion of these patients can benefit from mTOR inhibitor treatment because of the following proven benefits 4 : (1) the treatment can effectively reduce SEGA volume after 6 months of therapy without evidence of new SEGA, worsening hydrocephalus, or increased intracranial pressure; (2) patients with the treatment also experienced a reduction in seizure frequency; (3).…”

mentioning

confidence: 86%

Letter to the editor

Berhouma¹,

Dubourg²,

Messerer³

2012

Current Medical Research and Opinion

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“…Surgical resection has been the standard treatment option for symptomatic SEGAs; however, scientific evidence for the effectiveness of SEGA surgery in reducing SEGA-related conditions is limited 30,32–35. Postoperative morbidity is variable and may occur in ~50% of patients 36.…”

Section: Management and Current Therapeutic Strategiesmentioning

confidence: 99%

“…Postoperative morbidity is variable and may occur in ~50% of patients 36. Complications include increased prevalence of seizures, hydrocephalus, vision disorders, headaches, stroke, hemiparesis, and autism 32,33,35,37…”

Section: Management and Current Therapeutic Strategiesmentioning

confidence: 99%

Everolimus in the treatment of subependymal giant cell astrocytomas, angiomyolipomas, and pulmonary and skin lesions associated with tuberous sclerosis complex

Franz

2013

BTT

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Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder caused by inactivating mutations in either the TSC1 or TSC2 genes. It is characterized by the development of multiple, benign tumors in several organs throughout the body. Lesions occur in the brain, kidneys, heart, liver, lungs, and skin and result in seizures and epilepsy, mental retardation, autism, and renal and pulmonary organ system dysfunction, as well as other complications. Elucidation of the molecular pathways and etiological factors responsible for causing TSC has led to a paradigm shift in the management and treatment of the disease. TSC1 or TSC2 mutations lead to constitutive upregulation of the mammalian target of rapamycin pathway, which affects many cellular processes involved in tumor growth. By targeting mammalian target of rapamycin with everolimus, an orally active rapamycin derivative, clinically meaningful and statistically significant reductions in tumor burden have been achieved for the main brain (subependymal giant cell astrocytoma) and renal manifestations (angiomyolipoma) associated with TSC. This review provides an overview of TSC, everolimus, and the clinical trials that led to its approval for the treatment of TSC-associated subependymal giant cell astrocytoma and renal angiomyolipoma.

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Surgical resection of subependymal giant cell astrocytomas (SEGAs) and changes in SEGA-related conditions: a US national claims database study

Abstract: The study results have limitations in data source representativeness, coding accuracy, and study design.

Cited by 17 publications

References 19 publications

Tuberous sclerosis complex: the past and the future

Tuberous sclerosis complex: the past and the future

Letter to the editor

Everolimus in the treatment of subependymal giant cell astrocytomas, angiomyolipomas, and pulmonary and skin lesions associated with tuberous sclerosis complex

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