OTHER PRIMARY BRAIN TUMORS

CHORDOMAS

Chordoma is a rare type of cancer that occurs in the bones of the skull and spine. Chordomas are usually slow growing, and it is rare for the tumor to have metastasized (spread to other areas of the body) at the time the tumor is initially diagnosed; however, metastasis can occur when the tumor is more advanced.

Some of the early symptoms may be very nonspecific and include any of the following:

PINEAL-REGION TUMORS

Tumors in the pineal gland region are rare, constituting less than one percent of all primary brain tumors. Most tumors occur in children, so the incidence in adults is very low. Metastasis is found in about 10 percent to 20 percent of the cases, usually late in the disease.

The location of the pineal gland and other tumors in this region often causes a buildup of cerebrospinal fluid in one of the fluid-filled ventricles of the brain. This condition is referred to as hydrocephalus or hydrocephaly (also referred to as “water on the brain”). This can lead to symptoms such as headache, nausea, vomiting and neurological symptoms. 

Tumors that arise from the cells of the pineal gland are characterized based on their potential for rapid growth and metastasis (spread to other regions of the body).

• Pineocytomas are slow-growing tumors with a low potential to spread.
• Pineoblastomas are more aggressive, with a high potential to metastasize, although the spread is usually limited to the cerebrospinal fluid (CSF).
• Tumors containing both cell types also occur.

Diagnosing Pineal-Region Tumors:
Diagnostic imaging, such as magnetic resonance imaging (MRI), is used in the diagnosis of pineal gland tumors and developing a treatment plan. Analysis of the cerebrospinal fluid may be used to distinguish the particular type of cancer involved, and a biopsy may also be used to determine the tumor type.  

Treating Pineal-Region Tumors:
Treatment of the tumor may involve surgery, radiation, chemotherapy or a combination of any of all of these. Surgery to correct the hydrocephalus is also necessary. Tumors of the pineal gland (pineoblastomas), are very similar to embryonal tumors. Although they are not embryonal tumors, the treatment of pineoblastomas is often similar.

CENTRAL NERVOUS SYSTEM EMBRYONAL TUMORS 

Diagnosing CNS Embryonal Tumors:
Magnetic Resonance Imaging (MRI) is often used in the diagnosis of CNS embryonal tumors. Because these tumors often spread to the spinal cord, an MRI of the spine may be performed in addition to the MRI of the brain. For the same reason, doctors may want to collect a sample of the cerebrospinal fluid by lumbar puncture (also referred to as a spinal tap) a procedure in which a needle is inserted between the spinal vertebrae into the spinal cord.

Treating CNS Embryonal Tumors:
Surgery to remove as much of the tumor as possible is the first approach to treating CNS embryonal tumors. Challenges to complete removal of the tumor include the size of the tumor, extensive blood supply, and the likelihood that the tumor has already metastasized (spread) at the time of the initial surgery. In addition to removing the tumor, surgeons may need to perform procedures to correct hydrocephalus (also referred to as hydrocephaly, or water on the brain) caused by excess cerebrospinal fluid. In many cases radiation therapy and/or chemotherapy will be recommended after surgery, although radiation may not be appropriate for young children.
Because CNS embryonal tumors are extremely rare in adults, you want to be treated by a team with experience in treating rare cancers, such as the specialists at Houston Methodist. 

GERMINOMAS, TERATOMS & NONGERMINOMAS - GERM CELL TUMORS

• Tumors within the skull, or cranium
  
  • Pineal region – The pineal gland is located deep within the brain. Tumors located in the pineal region are often associated with headaches and vision problems.
  • Above the sella turcica – The pituitary gland is located deep in the brain, above a structure called the sella turcica. This location is referred to as the “suprasellar” region. Germ cell tumors in this location may press against the pituitary gland and cause hormone-related symptoms.
• Within the mouth – A particular kind of germ cell tumor, a teratoma, is often located here. 

• Tumor markers are molecules that are present on the surface of tumor cells, but absent or present in smaller amounts on normal cells. These molecules can be released into the blood or CSF so that their levels  will be elevated when a tumor is present.  Alpha-fetoprotein is a tumor marker for germ cell tumors.
• Hormones are normal substances, but tumors may be an abnormal source. Beta-human chorionic gonadotropin is prodiced in excess by some but not all germ cell tumors.