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Learn MoreOther names: Pineal gland tumor, Pinealoblastoma
Pineoblastoma is a very rare brain tumor that develops in the pineal gland. The pineal gland is a tiny structure found deep within the brain. The main pineal gland function is to release melatonin, a hormone that controls sleep.
Pineoblastoma is a rare tumor that develops in the pineal gland in the brain. The pineal gland releases melatonin, a hormone that controls sleep.
Pineoblastoma is most common in children and young adults. However, it is a rare tumor. Pineoblastomas account for less than 1% of childhood brain tumors. About half of tumors that occur in the pineal gland are pineoblastomas. Other tumors that can occur in the pineal gland include germ cell tumors, gliomas, and benign pineal parenchymal tumors.
Pineoblastomas are usually treated with surgery, radiation therapy, and chemotherapy. Because of the location of the tumor, pineoblastoma can be hard to treat. The 5-year survival rate for childhood pineoblastoma is about 60-65%.
Signs and symptoms of pineoblastoma depend on the tumor size and spread to other parts of the brain. Often, the tumor causes cerebrospinal fluid to build up (hydrocephalus) and puts pressure on the brain.
Pineoblastoma symptoms may include:
The brain has a connected set of fluid-filled openings, called ventricles. As a pineal gland tumor grows, it can press on one of these chambers and block the normal flow of cerebrospinal fluid. This causes a buildup of fluid within the brain known as hydrocephalus. The fluid increases pressure on the brain (intracranial pressure). Many of the symptoms of pineal tumors are due to the increased pressure against brain tissue.
Doctors test for pineoblastoma in several ways.
MRI scan with markings showing pineoblastoma. About half of pineal gland tumors are pineoblastomas.
There is no standard staging for pineoblastoma. However, doctors may classify the tumor by spread of disease. Pineoblastoma may spread through the cerebrospinal fluid in 10-20% of cases. It rarely spreads to other parts of the body outside the central nervous system.
Pineal gland tumors are classified by how they look under the microscope. The more abnormal tumor cells look, the higher the grade. Pineoblastomas are high-grade (grade IV) tumors.
Pineoblastomas are very similar in histology to medulloblastoma.
Successful removal of the tumor and treatment with radiation therapy are associated with better outcomes for children with pineoblastoma.
Factors that affect chance of cure include:
Survival rates for newly diagnosed pineoblastoma are about 60-65%.
Treatment for pineoblastoma depends on several factors including the size and location of the tumor and the child’s age. The main treatments for pineoblastoma are surgery, radiation therapy, and chemotherapy. Treatment plans are often similar to those used for medulloblastoma. Patients may be eligible for treatment through a clinical trial.
Ongoing follow-up care, laboratory tests, and routine MRI scans are used to monitor patients after completion of treatment. Monitoring should focus on early detection of recurrence and treatment for potential neurological, cognitive, and endocrine complications.
Patients who receive radiation therapy to the pineal gland are at risk for long-term endocrine problems including pituitary gland dysfunction (hypopituitarism). Low pituitary function can lead to problems such as delayed growth, fatigue, and fertility problems. Ongoing monitoring of hormone levels is important, and patients may need medicines including hormone replacement.
Patients who receive radiation therapy to the pineal gland are at risk for long-term endocrine system changes that may lead lead to problems such as delayed growth, fatigue, and fertility problems.
Regular checkups and screenings by a primary care physician are important to watch for health problems or late effects of treatment that can develop years after therapy.
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Reviewed: January 2020