Pineal Region Tumors
Pineal region tumors are primary central nervous system (CNS) tumors. These tumors begin in the brain (in the pineal gland) but can spread to the spinal cord.
What are the grades of pineal region tumors?
Pineal region tumors are grouped in four grades and subtypes based on their characteristics.
- Grade I pineocytoma are low grade tumors. This means the tumor cells grow slowly.
- Grade II or III pineal parenchymal or papillary tumors of the pineal region are both mid-grade tumors. This means the tumors have a higher chance of coming back after being removed.
- Grade IV pineoblastoma are malignant (cancerous). This means they are fast-growing tumors that tend to invade nearby tissue.
Who is diagnosed with pineal region tumors?
Pineal region tumors occur in children and young to middle age adults. Pineoblastomas are more common during the first 20 years of life. All tumors, except papillary tumors of the pineal region, occur slightly more often in females than males. They are most common in black people.
What causes pineal region tumors?
The cause of most pineal region tumors is not known. Pineoblastomas can occur in people with the inherited genetic disorder bilateral retinoblastoma.
Where do pineal region tumors form?
Pineal region tumors form in the pineal region of the brain. This region is located deep in the middle of the brain. Pineal region tumors arise from stem cells near the pineal gland.
Do pineal region tumors spread?
Pineal region tumors can spread to other areas in the CNS through cerebrospinal fluid (CSF).
What are symptoms of a pineal region tumor?
Symptoms related to pineal region tumors depend on the tumor’s location. People with pineal region tumors may have increased pressure inside the skull due to a build-up of CSF, known as hydrocephalus. Signs and symptoms of hydrocephalus may include headaches, nausea, vomiting, difficulty with eye movements, difficulty with balance, and walking.
What are the treatment options for pineal region tumors?
The first treatment for pineal region tumors is surgery, if possible. The goal of surgery is to obtain tissue to determine the tumor type and to remove as much tumor as possible without causing more symptoms for the person.
Treatments after surgery may include radiation, chemotherapy, or clinical trials. Clinical trials, with new chemotherapy, targeted therapy, or immunotherapy drugs, may also be available and can be a possible treatment option. Treatments are decided by the patient’s healthcare team based on the patient’s age, remaining tumor after surgery, tumor type, and tumor location.
Open clinical studies for pineal region tumors
- Outcomes and Risk Project for Patients with Rare CNS Cancers
- Evaluation of the Natural History and Specimen Banking for Patients with CNS Cancers
- Immune Checkpoint Inhibitor Nivolumab for Patients with Rare CNS Cancers
What do pineal region tumors look like on a CT scan or MRI?
Pineal region tumors usually appear as a solid mass that brightens with contrast. On a CT scan, calcium may be present. Pineoblastomas may extend into surrounding brain structures.
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