Subependymal giant cell tumors in tuberous sclerosis complex

Abstract

Subependymal giant cell tumors in patients with TSC appear to be of mixed glioneuronal lineage, and, therefore, the current practice of classifying these tumors as astrocytomas merits revision. The clinical diagnosis of SGCT should be made for subependymal lesions in TSC that are associated with symptoms, papilledema, or radiologic evidence of hydrocephalus or interval growth. A diagnosis of probable SGCT should be made when a lesion has the potential to cause obstruction based on size or location. Annual screening by MRI with or without contrast is indicated until at least 21 years of age even if subependymal nodules are absent on initial imaging. A diagnosis of SGCT or probable SGCT warrants more frequent monitoring or surgical intervention.

Obsessive compulsive disorder in a patient with tuberous sclerosis and subependymal giant cell astrocytoma

“…In TSC the most important neurologic findings are infantile spasms, hypsarrhythmia, seizures, autism, attention deficit disorders, psychoses, multiple calcified subependymal nodules along the ventricular surface, subependymal giant cell astrocytomas, multiple cortical tubers. [10][11][12][13] Our patient presented with seizures and a brain tumor compatible with subependymal giant cell astrocytoma. Also, TSC may be associated with retinal hamartomas, cardiac rhabdomyomas, arrhythmias, multiple bilateral renal angiomyolipomas, pulmonary lymphangioleiomyomatosis, and gastrointestinal hamartomatous polyps.…”

Tuberous sclerosis complex; a single center experience

“…Subependymal giant cell tumor is observed in 10-15% of the patients and frequently in the first 20 years. Only 6-9% of the patients are symptomatic (1,19,23). At presentation, brain MRI findings were found to be normal in three patients, but cortical tubers and subependymal nodules were found in the other patients.…”

“…Central nervous system lesions observed in tuberous sclerosis include glioneuronal hamartomas (cortical 55 (18,19). Cortical tubers are considered hamartomatous lesions which are differentiated with abnormal glial, neuronal cells and astrocytosis.…”

Computed Tomography Findings of a Tuberous Sclerosis Complex with Coexistant Multiple Myeloma: Case Report

“…3 In the central nervous system; cortical tubers, white matter heterotypes (dysplastic or demyelinizating white matter lesions), subependymal nodules and subependymal giant cell astrocytoma (SEGA) are regarded as characteristic lesions. 4,5 Cortical tubers are usually located in the frontal and temporal lobes and thought to be related to the seizures and behavioral problems seen in these patients. They do not show malignant transformation, but may calcify in time.…”

“…7 SEGA has been reported to occur in 5% to 14% of TSC patients and also account for 90% of all intracranial tumors associated with TSC. 5 These tumors usually develop around the foramen of Monro, appear hypointense on T1 and T2 weighted images and show intense contrast enhancement on brain magnetic resonance imaging (MRI). 7 SEGAs should be removed by surgical intervention, otherwise they can lead to neurological deficits and development of hydocephalus.…”