Pineoblastoma

What causes pineoblastoma?

Because pineoblastomas are quite rare, the exact cause of these tumors is not yet known. Most are thought to occur by chance (sporadic cancer). However, there is some evidence that a small proportion of pineoblastomas may be caused by an inherited predisposition, meaning that changes (mutations) in a gene inherited from a parent may increase the chance that a pineoblastoma could develop. Mutations in the RB1 gene are known to cause an increased risk for pineoblastoma.

Last updated on 05-01-20

Can pineoblastoma be inherited?

Only a small proportion of pineoblastomas are thought to be inherited. Of the inherited pineoblastomas, most are associated with changes (mutations) in the RB1 gene, which are usually associated with an increased chance of developing eye tumors known as retinoblastomas. A few articles have suggested that inherited changes in the APC gene, which are usually associated with an increased chance to develop colon cancer and brain tumors, might contribute to the development of a small number of pineoblastomas. There has been a report of a mother and daughter both developing pineoblastoma, which suggests that there may be other inherited causes of this tumor.

Last updated on 05-01-20

What is pineoblastoma?

Pineoblastoma is a cancerous (malignant) tumor that develops in the pineal gland. The pineal gland is located in the brain and makes the hormone melatonin to regulate the body's sleep patterns. Pineoblastoma is a type of primitive neuroectodermal tumor (PNET). Pineoblastoma mainly affects children, but can also occur in adults. Treatment of pineoblastoma includes surgery to remove as much of the tumor as possible, followed by radiation therapy and chemotherapy.

Last updated on 05-01-20

What are possible risk factors for pineoblastoma?

Pineoblastoma is one type of primitive neuroectodermal tumor (PNET), a group of tumors of the brain that occur in young children. PNETs also include medulloblastomas, neuroblastomas, and ependymoblastomas. One article identified two risk factors for PNETs: exposure to chemicals called N-nitroso before birth (which could occur if the mother drank beer during her pregnancy), and living on a farm for at least one year.

Last updated on 05-01-20

What is the chance that a pineoblastoma will be cured?

Because each diagnosis is unique, it is difficult to determine the chance of curing a pineoblastoma. Instead, research studies look at how many people are alive and for how long following their diagnosis; these measurements are used to estimate the survival rate. Individuals with a pineoblastoma live an average of 20 months after their diagnosis, and 10% of affected individuals are alive 5 years after their diagnosis. Survival is better when individuals are older than age 3 when diagnosed, when treatment includes complete removal of the tumor during surgery, and when cancer cells have not spread to other parts of the body (metastasized).

Last updated on 05-01-20

How might pineoblastoma be treated?

Standard treatment for pineoblastoma begins with surgery to remove as much of the tumor as possible. Surgery is followed by radiation therapy, which aims to destroy any cancer cells that may remain near the original location of the tumor, and chemotherapy, which is designed to kill any cancer cells that may have traveled to other parts of the body.

Last updated on 05-01-20


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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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