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|LETTER TO EDITOR
|Year : 2014 | Volume
| Issue : 1 | Page : 94-95
An interesting lesion: Solitary extra ventricular sub-ependymal giant cell astrocytomas
Jasmit Singh, Hrushikesh Kharosekar, Vernon Velho
Department of Neurosurgery, Sir JJ Group of Hospitals and Grant Medical College, Mumbai, Maharashtra, India
|Date of Submission||06-Jan-2014|
|Date of Decision||07-Jan-2014|
|Date of Acceptance||02-Feb-2014|
|Date of Web Publication||7-Mar-2014|
Department of Neurosurgery, Sir JJ Group of Hospitals and Grant Medical College, Mumbai, Maharashtra
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Singh J, Kharosekar H, Velho V. An interesting lesion: Solitary extra ventricular sub-ependymal giant cell astrocytomas. Neurol India 2014;62:94-5
Sub-ependymal giant cell astrocytomas (SEGA) occur in association with tuberous sclerosis complex. We report a case of extraventricular solitary SEGA who did not have any other clinical manifestations of tuberous sclerosis.
A 20-year-old patient was brought with the complaints of holocranial headache, and occasional vomiting for 3 months duration. Neurologic examination revealed right congruous homonymous hemianopia. Computed tomography (CT) brain showed heterogeneously enhancing mass in left parieto-occipital lobe with areas of necrosis suggestive of high grade glioma. The medial wall of the tumor was abutting the occipital horn. Contrast magnetic resonance imaging (MRI) Brain confirmed the CT scan findings [Figure 1]a and b. Patient was operated by a left temporo-parietal craniotomy and gross total resection was carried out through transcortical approach as the tumor was seen on the surface of the brain. Intra-operatively the tumor was vascular and yellowish red in color, suckable, with well-defined brain-tumor interface. Total excision of the lesion was carried out [Figure 1]c. Intra-operative frozen section report was suggestive of SEGA. Patient was re-evaluated keeping tuberous sclerosis in mind; but the patient did not have any stigmata or radiological finding suggestive of SEGA. The final histopathology report confirmed the diagnosis of SEGA [Figure 1]d. Patient recovered well and his field defects improved. He was subsequently discharged and is attending our follow-up.
|Figure 1: (a) Pre-operative magnetic resonance images axial sections showing the heterogeneous lesion, (b) coronal sections showing no intraventricular extensions but attached to ventricular wall, (c) Postoperative computed tomography scan, (d) histopathology slide|
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Tuberous sclerosis complex (TSC) is characterized by the formation of histologically benign hamartomas and low-grade neoplasms in multiple organ systems it has prevalence of 1 in 6,000 newborns and affects approximately 1.5 million people world-wide, occurring in all races and both genders equally. ,, TSC is an autosomal-dominant disorder caused by a mutation in either the TSC1 or TSC2 gene with high penetrance and variance. Almost all patients with TSC have numerous cutaneous stigmata, some of which can be subtle. For a patient to express TSC, two mutational events occur: The first is a germ line mutation (spontaneous or familial), resulting in a loss of heterozygosity at the TSC1 or TSC2 allele; subsequent somatic mutations of the remaining allele result in the phenotype of TSC. Somatic mosaicism can result in a less severe phenotype or a so-called forme fruste variation of the disease.  Neurologic manifestations of TSC are the most common cause of morbidity and mortality in TSC patients. They include seizures, developmental delay, behavioral problems and autism. Brain imaging findings of TSC include cortical and subcortical tubers, white matter abnormalities, subependymal nodules and SEGA1.
SEGAs are low-grade tumors that arise near the foramen of Monro in 10-15% of patients with TSC. They have a mixed glioneuronal lineage. SEGAs development is a gradual process that generally occurs within the first two decades of life. , These are slow-growing tumors, usually developing over 1-3 years, although their development can occasionally be more aggressive and involve parenchymal invasion or extensive peri-tumoral edema. Typically, serial neuroimaging is performed every 1-3 years in pediatric TSC patients until the mid-20s, even in the absence of SEGA symptoms. In patients with identified SEGA, scans may be performed more frequently to monitor the growth. Surgery is indicated in the presence of documented tumor growth and in symptomatic SEGA.  To the best of our knowledge, this is probably the first report of solitary SEGA in extraventricular location, though there are few case reports on extraventricular SEGA as a part of TCS. Oikawa et al.,  in their study have reported a case of full term neonate with sporadic TSC in whom they diagnosed an intraventricular SEGA with extraventricular extension. Similar cases of intraventricular SEGA with extraventricular extension have also been reported by others. , Of interest, most of the extraventricular SEGA have some intraventricular extension or attachment. In our case, intra-operatively the tumor margin extended into the left occipital horn; it is presumed that this tumor must have originated from ventricle and invaded the surrounding deep white matter. Though MRI findings were atypical, but histopathology was diagnostic of classical SEGA.
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