Brain Tumors in Tuberous Sclerosis

Tsuchida, Takahiro; Kamata, Kenichi; Kawamata, Masaharu; Okada, Kohei; Tanaka, Ryuichi; Oyake, Y

doi:10.1159/000119990

Cited by 15 publications

(11 citation statements)

References 4 publications

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“…We have chosen to follow these patients with serial scans until the tumor shows radiographic evidence of growth or becomes symptomatic. Most authorities agree that opera tive intervention is appropirate for symptomatic lesions [6,7,12,[17][18][19]23]. Two of our cases and others reported previously [17,19] suggest that documented growth of the tumor is a prelude to symptoms.…”

Section: Discussionmentioning

confidence: 53%

“…Diagnosis is often clouded by the presence of the other neurologic symptoms of tuberous sclerosis, namely seizures and mental retarda tion. SEGA may be initially diagnosed during the course of a work-up for a seizure disorder [7,19], and Frerebeau et al [21] described 11 patients with intraventricular tumors and tuberous sclerosis (8 had SEGA), 4 of whom presented only with worsening seizures. How this relates to the presence of a SEGA is not entirely clear.…”

Section: Discussionmentioning

confidence: 99%

“…We currently obtain an MRI preoperatively, immediately postoperatively, and then yearly for surveillance. The best operative approach is arguable, and there are proponents of both the transcor tical and the transcallosal approaches [13,15,16,18,19,23,27], We currently favor the transcallosal approach in most instances. Since these tumors are frequently large enough to cross the midline, the ease with which one can gain access to both lateral ventricles via this approach is helpful.…”

Section: Discussionmentioning

confidence: 99%

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Subependymal Giant Cell Astrocytomas in Children

et al. 1994

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Between 1977 and 1991, at the Children’s Hospital of Philadelphia, 10 patients, 5–16 years of age, were diagnosed as having subependymal giant cell astrocytomas. These patients accounted for 1.4% of all pediatric brain tumors seen during that time interval. One patient received a course of radiation therapy, which was ineffective in preventing tumor growth. All underwent surgical resections with the goal being maximal tumor debulking, if not complete resection. In 6 patients this was accomplished by the frontal transventricular route and, in the more recent patients, surgery was performed using a transcallosal approach. There were 2 perioperative deaths, and 2 other patients died later of causes unrelated to tumor progression. The remaining 6 patients remain alive and stable at a mean of 6.7 years of follow-up (range 1.8–12.4). None of these patients has received additional radiation therapy. Two patients have no other evidence of tuberous sclerosis. The use of modern radiographic and surgical techniques has made the treatment of this disease safer than in the past.

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Section: Discussionmentioning

confidence: 53%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Subependymal Giant Cell Astrocytomas in Children

et al. 1994

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“…The advantages consist of the possibility of assuring a nor mal CSF circulation, without utilizing an artificial CSF shunting system, and of obtaining the histological diagno sis [20,[28][29][30][31],…”

Section: Discussionmentioning

confidence: 99%

On the Treatment of Subependymal Giant Cell Astrocytomas and Associated Hydrocephalus in Tuberous Sclerosis

Rocco

Iannelli²,

Marchese³

1995

Pediatr Neurosurg

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Between 1980 and 1992, 10 children affected by tuberous sclerosis and intraventricular subependymal giant cell astrocytomas were surgically treated at the Institute of Neurosurgery, Section of Pediatric Neurosurgery, Catholic University of Rome. Nine patients presented with signs and/or symptoms of intracranial hypertension; in all of them the neuroradiological investigations demonstrated the presence of a space-occupying lesion in the region of the foramen of Monro with secondary ventricular dilation. In the remaining patient, a 5-month-old male infant, an intraventricular mass was discovered by means of an ultrasound examination performed after the first epileptic fit. Three patients underwent a ventriculoperitoneal CSF shunt as first surgical procedure; in 2 of them it was subsequently necessary to remove the intraventricular tumor due to the frequent occlusion of the CSF shunt device. Seven subjects underwent the direct surgical excision of the lesion. In all of them the procedure resulted in the control of the associated hydrocephalus. On the basis of such an experience, the authors conclude that the surgical removal of the intraventricular tumors in patients with tuberous sclerosis and hydrocephalus is the most appropriate treatment. In fact, in the series considered here, the removal of the tumor was not accompanied by significant morbidity, and was followed by improvement in clinical conditions. In particular, in cases in whom the occurrence of hydrocephalus was associated with a worsening in the seizure disorder, the tumor removal and the correction of intracranial hypertension was followed by a significant reduction in frequency or even by the disappearance of the seizures. However, in no case presenting with mental impairment was a significant improvement observed in mental performances as a consequence of the surgical treatment.

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“…9,10 Although rare, other tumors such as glioblastoma multiforme, astroblastoma and hemangioma have also been reported in these patients. 11 TSC has also been associated with gastric, testicular, breast, hepatocellular, somatostatinoma and sarcoma types of cancer. But TSC with accompanying MM disease, we think would be a rare association in the literature.…”

Section: Discussionmentioning

confidence: 99%