What are Pineal Region Tumors?
A pineal region tumor affects the area around the pineal gland, a hormone-producing organ deep inside the brain. The pineal gland produces melatonin, a substance that helps your body regulate its sleep-wake cycle. Tumors of the pineal gland are primary central nervous system (CNS) tumors, meaning they begin in the CNS and do not migrate from somewhere else in the body.
Some of these tumors grow quickly, and others grow very slowly. However, even slow-growing tumors can cause significant problems by putting pressure on surrounding brain tissue or blocking the flow of cerebrospinal fluid (CSF) within the brain.
Types and Grades of Pineal Tumors
Pineal tumors are assigned grades describing their growth pattern and other characteristics. The tumor grade will influence the disease’s prognosis and the appropriate course of treatment.
- Grade I. This grade grows slowly.
- Grades II and III. These tumor types grow more quickly than a Grade I tumor and are more likely to recur after treatment.
- Grade IV. This type of tumor grows quickly and is likely to spread into the surrounding brain tissue.
Pineal region tumors are also classified according to the type of cells they affect as well as other characteristics. For example, some pineal region tumors affect cells near the gland rather than the gland itself.
The types of tumors that affect the pineal gland include:
- Pineocytoma. These are Grade I or II tumors that are most common in adults. They are slow-growing and often respond well to treatment.
- Pineal parenchymal tumor. This is a moderately fast-growing tumor, typically Grade II or III.
- Pineoblastoma. These Grade IV tumors grow quickly and usually invade surrounding tissue. They are most common in children and adolescents, but they are very rare overall.
Pineal tumors can also be a mixed type that includes characteristics of more than one of the other types.
Symptoms of Pineal Tumors
Pineal region tumors cause symptoms when the growing tumor puts pressure on surrounding brain tissue. The tumors can also block the flow of cerebrospinal fluid (CSF) within the brain, causing the CSF to build up and exert pressure on brain tissue. This condition is called hydrocephalus.
Common symptoms of pineal tumors include:
- Poor balance
- Trouble walking
- Vision difficulties
- Memory problems
- Early puberty in children
What Causes Pineal Region Tumors?
The root cause of a brain tumor is a mutation or damage in the genes that control the growth of affected cells. The specific cause of the gene damage that triggers a tumor’s formation is usually not identifiable. In a healthy cell, these genes prevent the cell from growing or reproducing too rapidly, and the genes can also determine the cell’s normal lifespan. In a tumor’s cells, the damage to the genes causes the cells to grow and reproduce rapidly, and the cells may live longer than usual. As this rapid growth and reproduction continue, the cells grow into an abnormal mass.
Are Pineal Region Tumors Hereditary?
Most pineal region tumors do not appear to be linked to inherited traits. Instead, researchers believe most gene changes that cause tumors come from external environmental factors or changes within cells that occur randomly and with no external trigger. However, some pineoblastomas have been associated with the inherited disorder bilateral retinoblastoma.
How Are Pineal Region Tumors Detected?
Slow-growing pineal region tumors may develop for an extended time without producing symptoms, and some tumors may produce only mild symptoms. Fast-growing tumors may cause more severe symptoms that come on more quickly.
Some warning signs include:
- Nausea or vomiting
- Balance problems
How Are Pineal Region Tumors Diagnosed?
Doctors may take several different diagnostic steps when they suspect a patient may have a pineal region tumor.
- Neurological exam. A basic neurological exam will test a patient’s reflexes, balance, coordination, strength, vision, and hearing. This exam may prompt a doctor to look further for a tumor’s presence, giving a clue about the affected part of the brain.
- Imaging. Imaging technologies are non-invasive ways to look at brain tissue and possibly detect a tumor’s presence. They may also be used to judge the tumor’s size, location, and growth. Magnetic resonance imaging (MRI) uses a strong magnetic field to produce images of the brain and central nervous system. Computerized tomography (CT) scan may also be used to look for tumors.
- Lumbar puncture (spinal tap). This test examines the cerebrospinal fluid for tumor cells and other indicators of a tumor’s presence.
- Blood tests. These tests will look for tumor indicators such as the level of melatonin in the blood.
- Biopsy. Doctors may require a biopsy, in which a sample of the tumor is removed and analyzed by a pathologist. The biopsy might be conducted with surgery or, if the tumor is in a particularly hard-to-reach area, using a needle guided by imaging technology. A pathologist’s examination of the tissue sample can help suggest the best treatment course.
How Are Pineal Region Tumors Treated?
Treatment of a pineal region tumor can vary depending on the tumor’s growth rate, location, and size.
The most direct way to treat a brain tumor is to remove as much of it as possible with surgical intervention. Typically, the surgery involves opening the skull and removing the tumor while not damaging the surrounding healthy tissue.
However, when a tumor is located in an especially sensitive area or has infiltrated surrounding brain tissue, the surgeon may not be able to remove all of the tumor, and other subsequent treatment options may be necessary.
Radiation therapies involve using high-energy x-rays to target and kill tumor cells directly. The radiation is typically focused on the tumor so that they do not damage healthy cells. Radiation therapy is often used when the tumor can’t be entirely removed with surgery or when the tumor is in a location that is not safely accessible.
Side effects of radiation therapy may include headaches, memory loss, fatigue, and scalp reactions.
Chemotherapy uses chemicals that intentionally damage the body’s cells with the expectation that healthy cells can more easily recover from the damage than tumor cells can. Treating a pineal region tumor or any brain tumor with chemotherapy is challenging because some of the chemicals can’t cross the blood-brain barrier, a border that protects the brain from potentially harmful foreign substances. Ongoing research is being conducted to find new drugs that can effectively treat brain tumors.
How Do Pineal Region Tumors Progress?
The prognosis for a person with a pineal region tumor depends on several factors. On average, the five-year survival rate for all types of pineal region tumors is about 70%. However, aggressive tumors or those that do not respond well to treatment can lower survival rates.
Pineal tumors usually don’t spread to other parts of the body, but they can spread to other parts of the central nervous system via the cerebrospinal fluid (CSF).
How Are Pineal Region Tumors Prevented?
There is no clear way to prevent pineal region tumors from occurring.
Pineal Region Tumors Caregiver Tips
Caring for someone with a brain tumor can be even more challenging than the already high demands of caring for someone with any other type of severe and progressive illness. Along with the physical changes that make other cancers and serious illnesses so physically and emotionally exhausting, brain tumors also often produce psychological and cognitive changes in the patient that can threaten the caregiver’s well-being.
As you care for your loved one through the progressive stages of their illness, keep these tips in mind:
- Learn as much as possible about the potential effects of your loved one’s specific type of brain tumor. This will allow you to understand how the illness affects the sufferer’s behavior.
- Get help from your friends and family. Caring for a brain tumor patient is a huge task, and you shouldn’t try to do it alone.
- Take time whenever possible to step away from the patient and the illness and find time for yourself. Acknowledge that it is normal and acceptable to need occasional relief from caregiving burdens.
- Find a support group. It can be beneficial to learn that you are not alone and that other people understand what you are going through.
Some people with pineal region tumors also suffer from other brain and mental health-related issues, a condition called co-morbidity. Here are a few of the disorders commonly associated with these tumors:
Pineal Region Tumors Brain Science
The most common type of pineal region tumor arises from a kind of cell called a germ cell. These cells are part of the reproductive system and usually reside in the ovaries and testes. However, sometimes during embryonic development, some germ cells migrate to other parts of the body, including the brain. A tumor called a germinoma results when these mislocated cells grow out of control.
Not all germinomas are cancerous, but some are fast-growing and invasive. Fortunately, they also usually respond well to treatment, and about 90% of pineal region germinomas can be cured.
Pineal Region Tumors Research
Title: SJDAWN: St. Jude Children’s Research Hospital Phase 1 Study Evaluating Molecularly-Driven Doublet Therapies for Children and Young Adults With Recurrent Brain Tumors
Principal investigator: Giles W. Robinson, MD
St. Jude Children’s Research Hospital
Approximately 90% of children with malignant brain tumors that have recurred or relapsed after receiving conventional therapy will die of the disease. Despite this terrible and frustrating outcome, continued treatment of this population remains fundamental to improving cure rates. Studying this relapsed population will help unearth clues to why conventional therapy fails and how cancers continue to resist modern advances. Moreover, improvements in the treatment of this relapsed population will lead to improvements in upfront therapy and reduce the chance of relapse for all. Novel therapy and, more importantly, novel approaches are sorely needed. This trial proposes a new approach that evaluates rational combination therapies of novel agents based on tumor type and molecular characteristics of these diseases. The investigators hypothesize that using two predictably active drugs (a doublet) will increase the chance of clinical efficacy. The purpose of this trial is to perform a limited dose-escalation study of multiple doublets to evaluate the safety and tolerability of these combinations, followed by a small expansion cohort to detect preliminary efficacy. In addition, a more extensive and robust molecular analysis of all the participant samples will be performed as part of the trial so researchers can refine the molecular classification and better inform on potential response to therapy. In this manner, the tolerability of combinations can be evaluated on a small but relevant population, and the chance of detecting antitumor activity is potentially increased. Furthermore, the goal of the complementary molecular characterization will be to eventually match the therapy with better predictive biomarkers.
- To determine the safety and tolerability and estimate the maximum tolerated dose/recommended phase 2 dose (MTD/RP2D) of combination treatment by stratum.
- To characterize the pharmacokinetics of combination treatment by stratum.
- To estimate the rate and duration of objective response and progression-free survival (PFS) by stratum.
Title: A Study of a New Way to Treat Children and Young Adults With a Brain Tumor Called NGGCT
Principal investigator: Shannon M. MacDonald
Children’s Oncology Group
This phase II trial studies the best approach to combine chemotherapy and radiation therapy (RT) based on the patient’s response to induction chemotherapy in patients with non-germinomatous germ cell tumors (NGGCT) that have not spread to other parts of the brain or body (localized). This study has two goals: 1) optimizing radiation for patients who respond well to induction chemotherapy to diminish spinal cord relapses, and 2) utilizing higher dose chemotherapy followed by conventional RT in patients who did not respond to induction chemotherapy. Chemotherapy drugs, such as carboplatin, etoposide, ifosfamide, and thiotepa, work in different ways to stop the growth of tumor cells, either by killing the cells, preventing them from dividing, or stopping them from spreading. Radiation therapy uses high-energy x-rays or high-energy protons to kill tumor cells and shrink tumors. Studies have shown that patients with newly-diagnosed localized NGGCT, whose disease responds well to chemotherapy before receiving radiation therapy, are more likely to be free of the disease for a longer time than patients for whom the chemotherapy does not efficiently eliminate or reduce the size of the tumor. This study aims to see how well the tumors respond to induction chemotherapy to decide what treatment to give next. Some patients will be given RT to the spine and a portion of the brain. Others will be given high-dose chemotherapy and a stem cell transplant before RT to the whole brain and spine. Giving treatment based on the response to induction chemotherapy may lower the side effects of radiation in some patients and adjust the therapy to a more efficient one for other patients with localized NGGCT.
Title: Fluorescence Detection of Adult Primary Central Nervous System Tumors With Tozuleristide and the Canvas System
Principal investigator: John Yu, MD
Cedars-Sinai Medical Center
Los Angeles, CA
The purpose of this study is to examine the use of tozuleristide and the Canvas imaging system during surgical resection of primary central nervous system tumors, to identify which tumor types demonstrate detectable fluorescence following administration of 24 mg tozuleristide and imaged with the Canvas imaging system during surgical resection, and, among tumors that demonstrate fluorescence, to estimate the true positive rate and true negative rate of fluorescence in tissue biopsies, as well as sensitivity and specificity of tozuleristide fluorescence for distinguishing tumor from non-tumoral tissue. The secondary objectives of the study include evaluating the safety of tozuleristide and the Canvas imaging system and determining if the presence of residual fluorescence at the time of surgery corresponds to residual tumor evident on post-operative MRI images or if the absence of fluorescence corresponds to evidence of no gross residual tumor on post-operative magnetic resonance imaging (MRI).