These tumors originate from normal cells in the pineal gland. The pineal gland is located in the center of the brain and is involved in the secretion of specific hormones.
Tumor types occurring in the pineal region may or may not involve the pineal gland. True pineal cell tumors—pineocytoma, pineoblastoma, and mixed pineal tumors—are covered on this page.
Tumors that may occur in this region but are not necessarily pineal tumors include: germinoma, non-germinoma (eg, teratoma, endodermal sinus tumor, embryonal cell tumor, choriocarcinoma, and mixed tumors), meningioma, astrocytoma, ganglioglioma, and dermoid cysts. Information on these particular tumors can be found elsewhere on this site.
The pineal gland is located at the rear of the third ventricle, which is one of the fluid-filled cavities of the brain. Pineal tumors come from the normal cells of the pineal gland.
There are three types of pineal tumors:
- Pineocytoma: Slow-growing, grade II tumor.
- Pineoblastoma: More aggressive, grade IV, malignant tumor. A grade III intermediate form has also been described.
- Mixed Pineal Tumor: Contains a combination of cell types.
Symptoms are most often caused by blockage of the cerebrospinal fluid flow and problems with the eye movement pathways. Headache, nausea and vomiting, and double vision are common.
Pineal region tumors represent less than 1% of all primary brain tumors; however, 3% to 8% of childhood brain tumors occur in this area. These tumors tend to occur in young adults between 20 and 40 years old. About 10% to 20% of the tumors, particularly pineoblastoma, have the potential to spread through the cerebrospinal fluid. This usually occurs late in the disease. The tumors, however, rarely spread elsewhere in the body.
Like many tumor types, the exact cause of pineal cell tumors is not known. However, scientists have identified chromosomal abnormalities which may play a role in the development of these tumors.
Standard treatment for these kinds of tumors is radiation therapy. Radiation of the entire brain and spinal cord is recommended in patients with pineoblastoma. Chemotherapy may also be considered, particularly if the tumor has spread or if it regrows.
Surgery may be possible in some individuals to determine the tumor type and remove part of the tumor. In some cases, placement of a shunt (similar to a drain) is used to relieve pressure caused by buildup of cerebrospinal fluid.
Learn more about different treatment options for brain tumors on our Treatment page.