Welcome to the Rare Classroom, a new series from Patient Worthy. Rare Classroom is designed for the curious reader who wants to get informed on some of the rarest, most mysterious diseases and conditions. There are thousands of rare diseases out there, but only a very small number of them have viable treatments and regularly make the news. This series is an opportunity to learn the basics about some of the diseases that almost no one hears much about or that we otherwise haven’t been able to report on very often.

Eyes front and ears open. Class is now in session.

The disease that we will be learning about today is:

Anaplastic Astrocytoma

What is Anaplastic Astrocytoma?

• A grade III (high grade) astrocytoma tumor also known as AA or grade III astrocytoma​
• Tend to have tentacle-like projections that grow into surrounding tissue, making them difficult to completely remove during surgery​
• Tumor frequently found in the frontal lobes​
• The first step in treatment of anaplastic astrocytoma is surgery. Radiation is then used to treat the remaining tumor. Chemotherapy may be recommended immediately after radiation or when and if the tumor recurs​
• Astrocytomas come in four grades based upon how fast the cells are reproducing and the likelihood that they will spread to nearby tissue​
• Grades III and IV astrocytomas are malignant and are called high-grade astrocytomas​
• Lower grade astrocytomas can change into higher grade astrocytomas over time​
• Astrocytomas can appear in various parts of the brain and nervous system, including the cerebellum, the cerebrum, the central areas of the brain, the brainstem, and the spinal cord​
• Arise from cells called astrocytes
• Common among those in their 30s-50s​
• More common in men than women​
• Accounts for two percent of all brain tumors​
• Comprise 10-15% of all glial cancers​
• Median Survival is 2-3 years​
• More than 25% survive for 5 years or more after diagnosis​
• 2% of all brain tumors are anaplastic astrocytomas​

How Do You Get It?

• Exact cause is unknown
• certain genetic syndromes (ex. neurofibromatosis) are risk factors
• Radiation exposure is also increases risk

What Are The Symptoms?

Symptoms of anaplastic astrocytoma and related brain cancers may include:

• Motor weakness​
• Headache​
• Nausea and vomiting​
• Cognitive impairment​
• Seizures​
• Personality changes​
• Weakness on one side of body (hemiparesis)​
• Sensory loss​
• Visual loss​
• Aphasia​
• Drowsiness​

How Is It Treated?

• Current therapies are only modestly effective​. There is limited consensus on therapy for either initial treatment or recurrent disease​
• Typically treated with surgery, radiotherapy, and adjuvant temozolomide (chemo)
• Treatment for newly diagnosed patients includes surgery, chemotherapy, radioactive implants, stereotactic radiotherapy, and targeted therapy​
  • Anaplastic astrocytomas are usually more responsive to chemotherapy than glioblastomas​
  • First and second line agents typically used are: nitrosoureas (e.g. carmustine (BCNU) and Lomustine (CCNU)), temozolomide (TMZ), procarbazine, cis-retinoic acid, irinotecan (CPT-11) and cyclophosphamide​
• For recurrent AA, chemotherapy and re-operation is of modest benefit as response durations are short​
  • For recurrent anaplastic astrocytomas previously treated with nitrosoureas, temozolomide showed a 35% response rate​
    • Compared to therapies with lower response rates, temozolomide provided an increased 6-month survival rate (46% vs 31%). Some smaller survival benefit has been shown with adjuvant carmustine.​
• Bevacizumab (Avastin) may be tried in some cases.

Where Can I Learn More???

• Check out our cornerstone on this disease here.
• Learn more from the National Brain Tumor Society.