Pineal Tumors

Pineal Tumors Overview

Pineal tumors are rare brain tumors that begin in or around the pineal gland, a small almond-shaped structure at the brain’s center.

As part of the endocrine system, the pineal gland is essential in producing melatonin, the hormone that regulates sleep. By releasing melatonin, the pineal gland helps signal to the body when it’s time to rest and wake up, influencing sleep patterns, mood, and seasonal biological rhythms.

Pineal tumors can disrupt numerous neurological functions and present unique challenges because of their location deep within the brain. These tumors can also swiftly spread to other parts of the central nervous system via cerebrospinal fluid (CSF), particularly the spinal cord.

There are multiple types of pineal tumors, each with distinct characteristics and approaches for treatment:

• Germ cell tumors: These tumors arise from germ cells or cells that develop into reproductive cells. They’re the most common pineal region tumor type. Benign germ cell tumors are called germinomas, while malignant tumors, such as teratomas, are known as non-germinomatous germ cell tumors (NGGCTs).
• Pineocytomas: These pineal tumors are typically slow-growing, benign tumors that affect young adults. Pineocytomas are less aggressive and tend to have a favorable prognosis, especially if they can be entirely removed.
• Pineoblastomas: Highly aggressive and malignant, pineoblastomas most often occur in children under 10. Pineoblastomas are challenging to treat because they can spread quickly and usually require intensive treatment.
• Gliomas: These are tumors that arise from the glial cells around the pineal gland. They vary widely in behavior, ranging from low-grade gliomas like astrocytomas, which grow slowly and tend to be less aggressive, to high-grade glioblastomas, the most aggressive and fast-growing gliomas that can be difficult to treat.

Medical professionals describe pineal tumors using four grades based on their characteristics and behavior. They are:

• Grade 1: Pineocytomas are generally considered grade 1 tumors because they grow slowly, tend not to invade nearby tissues, and typically have a good prognosis.
• Grade 2: While some pineal tumors may earn a grade 2 classification—meaning they have a more aggressive growth rate than grade 1 tumors but are still slow-growing—diagnosed grade 2 pineal tumors are rare.
• Grade 3: These tumors are malignant and exhibit aggressive growth patterns. They infiltrate surrounding brain tissue but are less likely to spread outside the brain.Grade 3 tumors, like papillary tumors or pineal parenchymal tumors (PPTs), have a higher chance of returning after removal.
• Grade 4: Pineoblastomas are Grade 4 pineal tumors because they grow rapidly, invade surrounding brain tissue, and can swiftly spread to other areas like the spinal cord. Grade 4 tumors tend to affect children and often require intensive multimodal treatment, like surgery, radiation, and chemotherapy.

What causes pineal tumors?

Most pineal tumors develop sporadically, meaning they happen without a known cause.

That said, pineal tumors can result from genetic mutations that cause abnormal or misplaced cells to grow uncontrollably. They can also stem from anomalies in embryonic development. For example, germ cells typically migrate to form reproductive organs. Still, if some of these cells remain in the brain, they may develop into tumors later.

Although rare, some pineal tumors are associated with genetic conditions like retinoblastoma, a rare and fast-growing hereditary eye cancer, or Li-Fraumeni Syndrome, which is caused by mutations in the TP53 gene. Both of these inherited conditions can increase your risk of brain tumors. Unlike other cancers, pineal tumors are not strongly associated with lifestyle factors like smoking or diet, or environmental factors like exposure to carcinogens.

If you have a pineal tumor, you may experience one or more of the following symptoms:

• Headaches: Increased intracranial pressure from a pineal tumor can cause persistent headaches that often worsen over time and can be more intense in the morning or when lying down.
• Blurred or double vision: As intracranial pressure increases, your vision may blur or become impaired, such as issues with eye coordination or double vision.
• Parinaud’s syndrome: Often seen with pineal tumors, this condition involves difficulty moving the eyes up or down—known as upward gaze palsy—light sensitivities, and unusual eyelid positioning.
• Hydrocephalus: Since a pineal tumor can block the flow of cerebrospinal fluid, hydrocephalus or fluid buildup in the brain can occur. Symptoms include headaches, nausea, vomiting, and lethargy.
• Nausea and vomiting: Due to increased intracranial pressure, nausea and vomiting can occur and are more acute in the mornings.
• Memory and cognitive changes: The same pressure on nearby brain structures can cause concentration, memory, and information processing and recall issues.
• Sleep disturbances: A pineal tumor often disrupts sleep-wake cycles, leading to insomnia or irregular sleep patterns.
• Balance and coordination issues: If a pineal tumor compresses parts of your brain that control motor skills, it can lead to difficulties with balance and coordination and show up as unusual clumsiness or unsteadiness.
• Hormonal changes: Although rare, some pineal tumors, like germ cell tumors, can produce hormones that lead to early puberty in children. This is known as precocious puberty.

If you or a loved one is experiencing any of these symptoms, it’s essential to consult a trusted healthcare professional. While they can overlap with other neurological conditions, an early diagnosis can lead to better outcomes.

Pineal Tumors Diagnosis

Pineal tumor diagnosis often involves a combination of neurological exams, imaging tests, and lab work.

The following tools are often used in a pineal tumor diagnosis:

• Physical and neurological exam: First, your healthcare provider will ask about your symptoms, overall health, and any risk factors you might have. Next, they’ll complete an examination to assess your neurological function, including your reflexes, coordination, strength, sensation, and eye movements, as certain eye movement issues can point to pineal region abnormalities.
• Ophthalmologic exam: Since pineal tumors are known to produce vision-related symptoms, an eye specialist may be enlisted to evaluate your visual acuity and look for signs of increased intracranial pressure.
• Magnetic Resonance Imaging (MRI): An MRI is the primary imaging test to detect and diagnose pineal tumors, providing detailed images of your brain to help identify a tumor’s location, size, and characteristics. An MRI with contrast may be used to differentiate the tumor from surrounding tissues and determine whether or not it’s spread.
• Computed Tomography (CT): Although an MRI is often the first choice in neuroimaging, a CT scan relies on X-rays to create detailed cross-sectional brain images to help evaluate a tumor’s size and location. CT scans are also helpful in identifying calcifications, common to pineal tumors, and hydrocephalus.
• Lumbar puncture: Also known as a spinal tap, a lumbar puncture involves a needle inserted into the lower back to collect cerebrospinal fluid (CSF) to test for the presence of cancerous cells. This test can detect whether your tumor has spread within the central nervous system (CNS).
• Blood tests: Some pineal tumors—particularly germ cell tumors—release high markers of Alpha-fetoprotein (AFP) or Beta-human chorionic gonadotropin (hCG) in the blood or CSF that help diagnose certain pineal tumors.
• Biopsy: If your imaging results suggest a tumor, a biopsy is needed. During a biopsy, a small sample is removed from the tumor and sent to a pathology laboratory for analysis. There, pathologists examine the tissue under a microscope to determine the type of cells present and other important characteristics that guide treatment decisions. For tumors in the brain, a biopsy is typically done surgically or through stereotactic biopsy.

Additional tests may be needed, depending on the type and extent of your pineal tumor.

Neurosurgical treatments for pineal tumors include:

• Craniotomy: Craniotomy is the most common surgery for pineal tumors. During a craniotomy, a neurosurgeon makes an incision in the scalp, removes a portion of the skull, and accesses the brain to remove the tumor with intraoperative imaging and highly specialized tools. The goal of a craniotomy is to remove the bulk of the pineal tumor, except in cases where the tumor is in a highly sensitive area of the brain.
• Awake craniotomy: Awake brain surgery, or awake craniotomy, is a specialized surgery for brain tumors in areas that control critical functions, like speech or movement. During awake brain surgery, the patient is awake and alert while the neurosurgeon removes the tumor, allowing the surgical team to monitor brain function in real time and avoid damaging critical anatomy.
• Minimally invasive techniques: In some cases, neurosurgeons can use minimally invasive techniques to remove brain tumors. These techniques involve smaller incisions and specialized instruments to access and remove the tumor, like endoscopic surgery and laser interstitial thermal therapy (LITT). These techniques can offer shorter recovery times, reduced risk of complications, and more minor scars.
• Ventriculoperitoneal (VP) shunt: Sometimes, pineal tumors can block the flow of cerebrospinal fluid (CSF), leading to hydrocephalus, or fluid buildup in the brain. A VP shunt can drain excess fluid from the brain to the abdomen to relieve pressure and symptoms like headaches, nausea, and vision problems.
• Endoscopic third ventriculostomy (ETV): This minimally invasive procedure creates a small hole in the brain’s third ventricle, which allows CSF to bypass the blockage caused by the tumor and drain normally. An ETV is generally preferred over a VP shunt because it avoids the need for a permanent shunt.

Surgical treatment is often just one part of a comprehensive treatment approach, especially when it comes to high-grade pineal tumors. Nonsurgical approaches like radiation, chemotherapy, and targeted therapies are often part of the treatment plan.

• Stereotactic radiosurgery: This treatment method uses an exact form of radiation that delivers a focused dose to the tumor while sparing surrounding healthy tissue. The two most common forms are:
  • Gamma Knife radiosurgery: This option destroys abnormal tissue through radiation beams focused on a precise area and is only lethal to cells within the immediate vicinity. The Gamma Knife can only be used to treat lesions in the head, as it involves attaching a metal frame to the skull for accuracy. 
  • CyberKnife radiosurgery: This technique uses targeted energy beams to destroy tumor tissue while sparing healthy tissue. It uses image-guided robotics to deliver surgically precise radiation to help destroy tumors.
• Proton therapy: A specialized form of radiation that uses proton particles rather than X-rays, proton therapy allows for more targeted radiation with reduced exposure to the surrounding tissue. It’s especially beneficial for treating brain tumors in highly sensitive regions like the pineal gland.
• Chemotherapy: Chemotherapy is a standard treatment for certain pineal tumors, especially malignant germ cell tumors, aggressive pineal parenchymal tumors, or pineoblastomas. Chemotherapy can be used as a primary treatment or in combination with surgery and radiation to reduce tumor size, stop tumor recurrence, or treat cancer that’s spread. Oncologists usually give chemotherapy drugs like cisplatin, etoposide, carboplatin, and methotrexate intravenously rather than orally to circulate through the bloodstream and target pineal tumor cells.
• Hormone therapy: Some pineal tumors, like germ cell tumors, can produce hormones that cause symptoms like precocious puberty in children. Doctors can prescribe hormone therapy to balance or block hormone production and manage these symptoms.
• Targeted therapies: Targeted therapies are occasionally used to treat certain pineal tumors, especially in cases where traditional treatments are less effective. These therapies focus on the tumor’s specific molecular or genetic characteristics, inhibiting its growth while minimizing the harm to healthy cells.
• Corticosteroids: This class of medication can be used in the short term to reduce inflammation and brain swelling caused by the pineal tumor.
• Symptom management: To support overall quality of life, pain, sleep disturbances, and nausea can be managed via medications, in addition to physical or cognitive therapy.

Some pineal tumors can recur, even after successful treatment. Regular monitoring and imaging studies are often part of your long-term care plan.

One Central Location with Multiple Treatment Options

At Barrow Neurological Institute’s world-class Brain and Spine Tumor Program, we treat people with complex tumors like pineal tumors in one robust, full-service location. Our sophisticated multidisciplinary team—neurosurgeons, head and neck surgeons, neuro-oncologists, medical oncologists, and radiation oncologists, to name a few—can offer you the latest treatments for head and neck cancers, including metastatic cancers.

We also give our patients access to various neuro-rehabilitation specialists to maximize independence. Neuro-rehabilitation can include physical therapy to help you regain strength and balance, speech therapy to support speaking, expressing thoughts, or swallowing, and occupational therapy to aid you in managing daily activities like bathing, dressing, and using the bathroom. Treating a brain or spinal cord tumor is about more than extending your life—it’s also focused on enhancing your quality of life.

Clinical Trials

In partnership with the Ivy Brain Tumor Center, Barrow Neurological Institute is proud to be one of the country’s largest sites for brain tumor clinical trials. 

Clinical trials are part of the cancer research process to determine if new treatments are safe, effective, or even better than the current standard treatment. Although not yet FDA-approved, clinical trials can be the best option for those with difficult-to-treat tumors to improve the odds of finding an effective treatment for them and for future patients. To search for clinical trials that are now enrolling, visit here.