Subependymal Giant-Cell Astrocytomasin Pediatric Tuberous Sclerosis Disease

Ribaupierre, Sandrine de; Dorfmüller, Georg; Bulteau, Christine; Fohlen, Martine; Pinard, Jean-Marc; Chiron, Catherine; Delalande, Olivier

doi:10.1227/01.neu.0000249216.19591.5d

Cited by 123 publications

(78 citation statements)

References 24 publications

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“…Reported in up to 20% of patients with TSC, SEGAs are generally slow‐growing glioneuronal tumors 1, 5, 6. Growth of these lesions can eventually cause ventricular obstruction with the potential for sudden death secondary to acute hydrocephalus 5, 6, 7…”

mentioning

confidence: 99%

Everolimus for subependymal giant cell astrocytoma: 5‐year final analysis

Franz

Agricola

Mays

et al. 2015

Annals of Neurology

146

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ObjectiveTo analyze the cumulative efficacy and safety of everolimus in treating subependymal giant cell astrocytomas (SEGA) associated with tuberous sclerosis complex (TSC) from an open‐label phase II study (NCT00411619). Updated data became available from the conclusion of the extension phase and are presented in this ≥5‐year analysis.MethodsPatients aged ≥ 3 years with a definite diagnosis of TSC and increasing SEGA lesion size (≥2 magnetic resonance imaging scans) received everolimus starting at 3mg/m2/day (titrated to target blood trough levels of 5–15ng/ml). The primary efficacy endpoint was reduction from baseline in primary SEGA volume.ResultsAs of the study completion date (January 28, 2014), 22 of 28 (78.6%) initially enrolled patients finished the study per protocol. Median (range) duration of exposure to everolimus was 67.8 (4.7–83.2) months; 12 (52.2%) and 14 (60.9%) of 23 patients experienced SEGA volume reductions of ≥50% and ≥30% relative to baseline, respectively, after 60 months of treatment. The proportion of patients experiencing daily seizures was reduced from 7 of 26 (26.9%) patients at baseline to 2 of 18 (11.1%) patients at month 60. Most commonly reported adverse events (AEs) were upper respiratory tract infection and stomatitis of mostly grade 1 or 2 severity. No patient discontinued treatment due to AEs. The frequency of emergence of most AEs decreased over the course of the study.InterpretationEverolimus continues to demonstrate a sustained effect on SEGA tumor reduction over ≥5 years of treatment. Everolimus remained well‐tolerated, and no new safety concerns were noted. Ann Neurol 2015;78:929–938

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mentioning

confidence: 99%

Everolimus for subependymal giant cell astrocytoma: 5‐year final analysis

Franz

Agricola

Mays

et al. 2015

Annals of Neurology

146

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“…[10][11][12]19,24,35,37 The most common clinical presentation is elevated ICP due to CSF obstruction at one or both foramina of Monro. Cases of sudden death have been reported.…”

Section: Discussionmentioning

confidence: 99%

“…3,5,[11][12][13]23,24,30,33,36,44 Multiple patient series have reported on the role of surgery in treating patients with SEGAs ( Table 3). The historical incidence of significant, and in some cases, permanent neurological morbidity has been documented (ranging from 5% to 50%).…”

Section: 43mentioning

confidence: 99%

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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“…Ribaupierre et al (8) reported that if the radiological characteristics of the SEGA have an incomplete or noncalcification, with a location near the foramen of monro and a size greater than 5mm, the lesions should be excised as early as possible. Their numbers were larger or symptomatic lesions tend to have a higher morbidity.…”

Section: A B C Ekici Ma Et Al: Surgical Timing Of the Subependymal Gmentioning

confidence: 99%

Surgical timing of the subependymal giant cell astrocytoma (sega) with the patients of tuberous sclerosis complex. .

Ekici

Kumandaş²,

Per³

et al. 2011

Turkish Neurosurgery

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AIm: Tuberous sclerosis complex has shown a wide variety of clinical, pathologic and radiologic manifestations. Many tumor types are found in tuberous sclerosis, which includes subependimal giant cell astrositoma. The aim of this study is to focus on surgical timing of the tumor. mAterIAl and methOds: This study included 37 children with tuberous sclerosis presenting to Erciyes Univercity Medical School, whose hospital record were retrospectively evaluated between 1995 and 2010. Of the 5 patients had diagnosed with the subependymal giant cell astrocytoma and three patients were opereted on.results: In the 27 of the patients had subependimal nodules (73%), cortical tubers were in the 19 patients (51,4%), giant cell astrositoma (SEGA) were in the 5 patients (13,5%). Mental retardation in different level was detected in the 18 patients (48.6%). The other clinical findings of the patients were angiomyolipomas (37.8%), hypomelanotic macules (91.9%), Convulsion (54.1%), adenoma sebaceum (32.4%) , West syndrome (16.22%), shagreeen patch (16.2%), intracardiac tumor (37.8%), subungual fibroma (2.7%), fibroadenom in the neck (2.7%).COnClusIOn: A multidisciplinary approach is essential for an early, accurate diagnosis and proper management of affected individuals. The early surgical menagement for subependimal giant cell astrocytoma are recommended, and also periodic monitoring even for asymptomatic patients with subependymal nodules. KeywOrds: Tuberous sclerosis complex, Subependymal giant cell astrositoma, Surgical timing ÖZAmAÇ: Tüberus sklerozis kompleks klinik, patolojik ve radyolojikolarak geniş bir çeşitlilik göstermektedir. Subependimal dev hücreli astrositomanın dahil olduğu birçok tümör tipi bulunur. Bu çalışmanın amacı tümörün cerrahi zamanlamasına dikkat çekmek. yÖntem ve GereÇ: Erciyes Üniversitesi Tıp Fakültesi Hastanesi'nde 1995-2010 yılları arasında hasta kayıtları geriye dönük olarak değerlen-dirildi ve 37 tüberus sklerozisli hasta çalışmaya dahil edildi. Hastaların 5'ine subependimal dev hücreli astrositom tanısı konuldu ve üç hasta ameliyat edildi.BulGulAr: 27 hastada (%73) subependimal nodul, 19 hastada (%51.4) kortikal tuber, 5 hastada (%13.5) dev hücreli astrositom vardı. 18 hastada (%48.6) farklı seviyelerde mental retardasyon tespit edildi. Hastaların diğer klinik bulguları; anjiyomyolipom (%37.8), hipomelanotik makul (%91.9), konvulzyon (%54.1), adenoma sebaseum (%32.4), west sendromu (%16.2), shagreen lekeleri (%16.2), intrakardiak tümör (%37.8), subungual fibroma (%2.7), boyunda fibro adenomdu (%2.7).sOnuÇ: Etkilenmiş bireylerin erken, doğru ve uygun tedavisi için multidisipliner yaklaşım esastır. Subependimal dev hücreli astrositomada erken cerrahi önerilir ve de subependimal nodullü hastalar için peryodik izleme önerilir.AnAhtAr sÖZCÜKler: Tüberus sklerozis kompleks, Subependimal dev hücreli astrositom, Cerrahi zamanlama

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Subependymal Giant-Cell Astrocytomasin Pediatric Tuberous Sclerosis Disease

Cited by 123 publications

References 24 publications

Everolimus for subependymal giant cell astrocytoma: 5‐year final analysis

Everolimus for subependymal giant cell astrocytoma: 5‐year final analysis

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

Surgical timing of the subependymal giant cell astrocytoma (sega) with the patients of tuberous sclerosis complex. .

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