The diagnosis and treatment of subependymal giant cell astrocytoma combined with tuberous sclerosis

Jiang, Tao; Jia, Ge; Ma, Zhenyu; Luo, Sulan; Zhang, Yuqi

doi:10.1007/s00381-010-1159-1

Cited by 41 publications

(24 citation statements)

References 33 publications

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“…In our study, the mean initial age was 16.4 years (range: 5-32years), and 15 patients were less than 18 years upon initial diagnosis, indicating that SEGA tended to occur in young patients. This was similar to the previously reported cases where the age distribution was in the first two decades of life, with the highest frequency in those 5-13 years old [9][10][11]. The male to female ratio in our series was 11: 9, which did not show obvious sex bias.…”

Section: Discussionsupporting

confidence: 92%

Clinical and imaging features of subependymal giant cell astrocytoma: report of 20 cases

et al. 2017

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Background: Subependymal giant cell astrocytoma (SEGA) is a clinically benign brain tumor associated with tuberous sclerosis complex (TSC). There are still controversies on early diagnosis of the tumor. Methods: CT and MR imaging of 20 patients with pathologically confirmed SEGA were retrospectively reviewed. Two radiologists evaluated the location, shape, size, number, edge, cerebral edema, homogeneous or heterogeneous appearance, attenuation and signal intensity, degree of enhancement and calcification of lesions. Their prognoses were based on clinical observations.

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Section: Discussionsupporting

confidence: 92%

Clinical and imaging features of subependymal giant cell astrocytoma: report of 20 cases

et al. 2017

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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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“…The initial primary resected tumors (22) were on the right side in 9 cases, on the left in 9 cases, and bilateral in 3 cases. The presence of bilateral tumors did not alter our decision to proceed with surgery.…”

Section: Resultsmentioning

confidence: 99%

“…Our series is among the few that have reported the resection of SEGAs ( Table 3). The latest, and second largest series in the past 10 years, by Jiang et al (2011), 22 reported on 17 SEGAs. Of these 17, 11 (65%) were resected via a transcallosal approach and 6 via a transcortical approach.…”

Section: 43mentioning

confidence: 99%

“…Clinical and radiographic monitoring are the mainstays of assessment. 22,36,54 Although resection was the only treatment for tumor growth or hydrocephalus, mammalian target of rapamycin (mTOR) inhibitors including everolimus can prevent or reverse SEGA growth. 6,15,17,[26][27][28][29] Subependymal giant cell astrocytomas may also hemorrhage, leading to acute neurological deterioration dictating neurosurgical intervention.…”

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confidence: 99%

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A management strategy for intraventricular subependymal giant cell astrocytomas in tuberous sclerosis complex

Harter¹,

Bassani²,

Rodgers³

et al. 2014

PED

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Object Subependymal giant cell astrocytomas (SEGAs) are benign tumors, most commonly associated with tuberous sclerosis complex (TSC). The vast majority of these tumors arise from the lateral ependymal surface adjacent to the foramen of Monro, therefore potentially encroaching on one or both foramina, and resulting in obstructive hydrocephalus that necessitates surgical decompression. The indications for surgery, intraoperative considerations, and evolution of the authors' management paradigm are presented. Methods Patients with TSC who underwent craniotomy for SEGA resection at New York University Langone Medical Center between January 1997 and March 2011 were identified. Preoperative imaging, clinical characteristics, management decisions, operative procedures, and outcomes were reviewed. Results Eighteen patients with TSC underwent 22 primary tumor resections for SEGAs. The indication for surgery was meaningful radiographic tumor progression in 16 of 21 cases. The average age at the time of operation was 10.3 years. Average follow-up duration was 52 months (range 12–124 months). The operative approach was intrahemispheric-transcallosal in 16 cases, transcortical-transventricular in 5, and neuroendoscopic in 1. Nine tumors were on the right, 9 on the left, and 3 were bilateral. Gross-total resection was documented in 16 of 22 cases in our series, with radical subtotal resection achieved in 4 cases, and subtotal resection (STR) in 2 cases. Two patients had undergone ventriculoperitoneal shunt placement preoperatively and 7 patients required shunt placement after surgery for moderate to severe ventriculomegaly. Two patients experienced tumor progression requiring reoperation; both of these patients had initially undergone STR. Conclusions The authors present their management strategy for TSC patients with SEGAs. Select patients underwent microsurgical resection of SEGAs with acceptable morbidity. Gross-total resection or radical STR was achieved in 90.9% of our series (20 of 22 primary tumor resections), with no recurrences in this group. Approximately half of our patient series required CSF diversionary procedures. There were no instances of permanent neurological morbidity associated with surgery.

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The diagnosis and treatment of subependymal giant cell astrocytoma combined with tuberous sclerosis

Cited by 41 publications

References 33 publications

Clinical and imaging features of subependymal giant cell astrocytoma: report of 20 cases

Clinical and imaging features of subependymal giant cell astrocytoma: report of 20 cases

Tuberous sclerosis complex: the past and the future

A management strategy for intraventricular subependymal giant cell astrocytomas in tuberous sclerosis complex

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