Astrocytoma (patient information)
Template:Subependymal Giant Cell Astrocytoma (patient information)
Editor-in-Chief: C. Michael Gibson, M.S.,M.D. [1] Associate Editor-In-Chief: Jinhui Wu, M.D.; Ammu Susheela, M.D. [2]
Overview
Subependymal giant cell astrocytoma is a rare, benign brain tumor commonly associated with tuberous sclerosis, a genetic disorder. It is seen in about 10-20% of patients with tuberous sclerosis. It is predominantly seen in the pediatric population but can also occur in adults. It arises in the ventricles of the brain and can cause symptoms such as headache, change in vision, nausea, vomiting, though some patients remain asymptomatic. An MRI or CT scan can be used in the diagnosis of subependymal giant cell astrocytoma. Surgery is the main treatment strategy but pharmacological therapy may be used in cases where surgery is contraindicated.
What are the Symptoms of Subependymal Giant Cell Astrocytoma?
Some common symptoms of subependymal giant cell astrocytoma include:
What Causes Subependymal Giant Cell Astrocytoma?
The exact cause of subependymal giant cell astrocytoma is unknown but it is predominantly associated with tuberous sclerosis which is a genetic disorder caused by a mutation in the TSC1 and TSC2 genes.
Who is at Highest Risk?
People with tuberous sclerosis have the highest risk of developing subependymal giant cell astrocytoma
Diagnosis
When patients present with signs and symptoms of subependymal giant cell astrocytoma, along with a thorough history and physical examination, a magnetic resonance imaging (MRI) and computed tomography (CT) scan of the head are used in the diagnosis of subependymal giant cell astrocytoma.
When to Seek Urgent Medical Care?
If acute symptoms such as headache, vomiting, confusion, and blurred vision should develop, or new onset of symptoms, seek urgent care immediately.
Treatment Options
The treatment options for subependymal giant cell astrocytoma include:
- Surgery: Surgery is the recommended therapy. It is usually done when patients present with a new set of symptoms, tumor growth is seen on imaging studies, or presents with acute symptoms. In cases that cannot be treated surgically, medical therapy can be used.
- Pharmacological therapy: This is used when surgery can't be done, which is when a patient presents with multiple tumors or the tumor has invaded neighboring structures. The preferred medical therapy is mammalian target of rapamycin (mTOR) inhibitors which includes everolimus and rapamycin.
Where to find Medical Care for Subependymal Giant Cell Astrocytoma
Prevention
There are no methods of preventing subependymal giant cell astrocytoma.
What to Expect (Outlook/Prognosis)?
The prognosis is generally poor. A few positive prognostic factors include:
- Surgical treatment
- Age under 18 years
Possible Complications
Possible complications of subependymal giant cell astrocytoma include:
- Bleeding within the tumor
- Obstructive hydrocephalus
- Stroke
- Sudden death