Subependymal giant cell astrocytomas (SGCA's) are benign tumours (WHO grade I), seen almost exclusively in young patients with tuberous sclerosis (TS). They can be either asymptomatic or symptomatic due obstructive hydrocephalus, surgery treatment is often curative.
On imaging they classically appear as an intraventricular mass near the foramen of Monro, larger than 1 cm, showing calcifications, heterogenous MRI signal, and marked contrast enhancement.
Subependymal giant cell tumours are a well known manifestation of TS, affecting 5-15% of patients with the condition. They are principally diagnosed in patients under 20 years of age, but are occasionally found in older people.
Subependymal giant cell tumours are often asymptomatic. When symptoms occur, they are usually a result of obstructive hydrocephalus because of mass effect around the ventricular system. Common sites of obstruction include the interventricular foramen (of Monro), the cerebral aqueduct (of Sylvius) and the third or fourth ventricles.
Subependymal giant cell tumours are believed to arise from a subependymal nodule present in the ventricular wall in a patient with TS 4. Histologically, subependymal nodules and subependymal giant cell tumours are indistinguishable, and the distinction lies in the potential of a subependymal giant cell tumour for growth and mass effect 5.
The ependymal lining over SGCA remains intact making CSF seeding highly unlikely 7.
Current evidence suggests that SGCAs are of a mixed neuronal and glial lineage, although they continue to be classified as astrocytomas 5. They are considered WHO grade I lesions (see WHO classification of CNS tumours).
- typically appears as an intraventricular mass near the foramen of Monro
- they are usually larger than 1 cm
- lesions are iso- or slightly hypoattenuating to grey matter
- calcification is common and haemorrhage is possible
- accompanying hydrocephalus may be present
- often shows marked contrast enhancement (subependymal nodules also enhance)
- T1: heterogenous and hypo- to isointense to grey matter
- T2: heterogenous and hyperintense to grey matter; calcified components can be hypointense
- T1 C+ (Gd): can show marked enhancement
Treatment and prognosis
Young children who have tuberous sclerosis may be offered screening because of the increased risk of developing subependymal giant cell astrocytomas.
The main treatment is surgery, which is indicated if the tumour is symptomatic, or growth is demonstrated on MRI. Surgery is often curative. Oral Rapamycin™ (sirolimus) has also been trialled 3.
In the clinical context of known TS, the appearance is virtually pathognomonic, and the main differential is between a subependymal nodule and SGCA. Serial imaging is most helpful here, as growth implies SCGA.
Other general considerations include:
- WHO classification of CNS tumours
- WHO grading of CNS tumours
- VASARI MRI feature set
- diffuse astrocytoma grading
- grade I:
- grade II:
- grade III
- anaplastic astrocytoma
- anaplastic oligodendroglioma
- grade IV:
- glioblastoma vs cerebral metastasis
- radiation-induced gliomas
- gliomatosis cerebri (growth pattern)
- specific locations
- treatment response
- Stupp protocol
- glioma treatment response assessment in clinical trials
- multicentric glioblastoma
- multifocal glioblastoma
- prognostic genetic markers
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- 6. Smith A, Smirniotopoulos J, Horkanyne-Szakaly I. From the Radiologic Pathology Archives: Intraventricular Neoplasms: Radiologic-Pathologic Correlation. Radiographics. 2013;33 (1): 21-43. Radiographics (full text) - doi:10.1148/rg.331125192
- 7. Textbook of Radiology and Imaging. Churchill Livingstone. (2003) ISBN:0443071098. Read it at Google Books - Find it at Amazon