Solid tumors
Pineoblastoma
If you or someone you love has just heard this diagnosis, start here. This guide explains what the condition is, how it is usually treated and whether a transplant plays any part.
Pineoblastoma is a rare, fast-growing brain tumor that starts in the pineal gland, deep in the center of the brain. It mostly affects children. Treatment usually combines surgery, chemotherapy and, for children older than 3, radiation to the brain and spine. Some plans for very young children add high-dose chemotherapy followed by a return of the child’s own stem cells. No donor is involved, and experts say the long-term benefit of that step is not proven.
Other names and abbreviations
pinealoblastoma, pineal PNET, primitive neuroectodermal tumor of the pineal region, Pinealoblastoma, previously grouped with CNS primitive neuroectodermal tumours
In short
- Pineoblastoma is a rare, aggressive brain tumor in the pineal area that mainly affects children. It can block the flow of brain fluid or spread within the brain and spine.
- Treatment generally combines surgery, chemotherapy and radiation suited to the patient's age. Very young children may get chemotherapy plans meant to delay radiation.
- Some intensive chemotherapy plans include a return of the patient's own stem cells, so no donor is normally needed. These plans are used especially for young children or tumors that come back.
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Underlined words open a short explanation. See all terms
Where transplant fits
Autologous stem cell rescueComing from the patient's own body. In an autologous transplant, the patient's own stem cells are collected and stored, then given back after high-dose treatment. It does not use a donor. is used in selected intensive chemotherapy protocols, particularly in young children or recurrent disease. It supports marrowThe soft, spongy tissue in the center of most bones. Red bone marrow holds the blood-forming stem cells that make red blood cells, white blood cells and platelets. recovery using the patient’s own cells; a registry donor is not normally part of treatment.
Treatment depends on the exact diagnosis, disease stage, prior treatment and the person’s health.
Key facts
- Who it affects
- Pineoblastoma mainly affects children, with age patterns varying by molecular subtype.
- How common
- Less than 1% of childhood brain tumorsChildren, from St. Jude Children’s Research Hospital patient information reviewed August 2026 (region not specified); St. Jude adds that about 3 in 10 tumors found in the pineal gland are pineoblastomas Source: How common
- Cells used in a transplant
- The patient’s own collected blood-forming cells for autologous stem cell rescue, when that procedure is appropriate.
- Where a donor fits
- Limited transplant role
The condition
What it is
The pineal gland is a tiny organ near the center of the brain. It makes melatonin, a hormone that helps control sleep. Pineoblastoma is a cancer that forms in or around this gland. It is a grade 4 tumor, the highest grade, which means its cells grow fast. Like related brain tumors, it tends to spread through the fluid around the brain and spinal cord (cerebrospinal fluid).
Pineoblastoma used to be grouped with embryonal brain tumors, a group that once included tumors called primitive neuroectodermal tumors (PNET). The World Health Organization (WHO) now classes it as a tumor of the pineal gland itself. Because it is still treated much like embryonal tumors such as medulloblastoma, it often appears in the same treatment guides.
Tests of the tumor’s genes now sort pineoblastoma into four main groups: PB-miRNA1, PB-miRNA2, PB-MYC/FOXR2 and PB-RB1. The groups differ in the age at which they usually appear and in their outlook. A related tumor, called pineal parenchymal tumor of intermediate differentiation (PPTID), mostly affects adults and is a separate diagnosis.
What causes it
Pineoblastoma begins when cells in the pineal area gain gene changes that make them grow out of control. Most pineal tumors of this kind are sporadic, which means they are not linked to an inherited condition. Often, doctors do not know what causes them.
Some children are born with a gene change that raises their risk. A child with the inherited form of retinoblastoma, an eye cancer caused by changes in the RB1 gene, has a higher chance of pineoblastoma. When retinoblastoma and a pineal tumor happen together, it is called trilateral retinoblastoma. Inherited changes in the DICER1, DGCR8 (linked to DiGeorge syndrome) and APC genes are also linked to pineoblastoma, mainly in older children.
Because of these links, the NCI says children with pineoblastoma may have RB1 or DICER1 gene testing. An international expert group recommends genetic counseling for every patient with pineoblastoma, whatever the family history. MedlinePlus Genetics explains that DICER1 syndrome can pass from parent to child, but many people with the gene change never develop a tumor.
Symptoms and effects
The tumor sits where brain fluid normally drains, so it often blocks that flow. Fluid then builds up and raises pressure inside the head (hydrocephalus). This can cause headaches, nausea and vomiting, and trouble with balance and walking.
Pressure on the nearby brainstem can affect the eyes. A child may have trouble looking up, pupils that react poorly to light, or double vision. Doctors call this group of eye signs Parinaud syndrome. As the tumor grows, it may cause weakness on one side of the body or clumsiness. Infants and young children with tumors like this may be fussy, eat poorly, grow slowly, miss milestones or have a head that grows too fast.
Pineoblastoma can spread through the spinal fluid to other parts of the brain and spine. Spread outside the brain and spine, such as to bone or lungs, is rare.
About these numbers. Each one says which group of people it comes from, and the place and years where the source gives them. It describes what happened across that group, not what will happen to any one person. And a figure measured among people who had a transplant is not the same as the number of people who need one.
- 10% to 30%Had already spread at diagnosis
Patients with pineoblastoma, as summarized from published studies by the NCI PDQ pediatric treatment board (health professional summary updated April 2025); region and years not specified
Read the source: Had already spread at diagnosis
Diagnosis and treatment
How pineoblastoma is diagnosed
Children usually come to a doctor with signs of fluid pressure, such as headache, vomiting or trouble walking, or with problems moving their eyes. An international expert group says the first steps are to protect the child from the high pressure, get brain imaging, and avoid a spinal tap until it is safe. When possible, the child is moved to a specialist center.
MRI of the brain and the whole spine, with contrast dye, is the main test. It shows the tumor and whether it has spread. Blood, and spinal fluid when it can be collected safely, are tested for the germ cell tumor markers AFP and hCG. High levels point to a germ cell tumor, a different kind of pineal tumor that can sometimes be diagnosed without surgery. A spinal tap (lumbar puncture) checks the spinal fluid for tumor cells.
The diagnosis is confirmed on tumor tissue, taken by biopsy or during surgery. Lab doctors (pathologists) study it under a microscope and run gene and DNA methylation tests that place the tumor in its gene group. In the United States, the NCI’s Molecular Characterization Initiative offers free molecular testing to some children, teens and young adults with newly diagnosed cancer. For children who have retinoblastoma, the NCI notes that MRI may find pineoblastoma early, when it can be treated successfully.
Specialist review matters. In one Children’s Oncology Group trial, methylation testing agreed with the diagnosis in 26 of 29 pineoblastomas, but it changed the diagnosis for most tumors outside the pineal area that had been called CNS-PNET.
How it is treated
A team that treats children’s brain tumors plans the care. Surgery often comes first. It relieves fluid pressure, either with a shunt (a thin tube that drains fluid) or by making a small opening in a fluid space in the brain (endoscopic third ventriculostomy). It also gives tissue for diagnosis. Surgeons remove as much tumor as is safe, but because the tumor lies deep in the brain and near blood vessels, removing all of it is often not possible.
For children older than 3, treatment usually goes on to radiation to the whole brain and spine (craniospinal irradiation), with a higher dose to the tumor site. Chemotherapy is given much as it is for high-risk medulloblastoma, often with medicines such as vincristine, cyclophosphamide and cisplatin. For older children whose tumor is in a lower-risk gene group, has not spread and was fully or almost fully removed, an international expert group says a lower radiation dose can be used.
Children 3 and younger are treated differently, because radiation can harm the growing brain. They usually get chemotherapy first, in the hope of delaying or avoiding radiation. Some plans use very high doses of chemotherapy followed by a return of the child’s own stored stem cellsYoung cells that can grow into every type of blood cell: red cells that carry oxygen, white cells that fight infection and platelets that help blood clot. They are found in the bone marrow and the bloodstream. (autologous stem cell rescue). The NCI says there is no standard treatment for this age group. Because childhood cancer is rare, it says taking part in a clinical trialA research study that tests how well a new medical approach works in people. Trials can test new ways to screen for, prevent, diagnose or treat a disease. should be considered.
If pineoblastoma comes back, options may include more surgery, more radiation, chemotherapy or high-dose chemotherapyVery strong drug treatment given in large doses, often to kill cancer cells. It also destroys the bone marrow and can cause other serious side effects. A stem cell transplant usually follows to rebuild the marrow. with stem cell rescue. The NCI says it is not known whether high-dose chemotherapy with stem cell rescue improves survival at that stage.
The plan depends on the child’s age, whether the tumor has spread, how much was removed and the tumor’s gene group.
Kinds of treatment described for pineoblastoma: medicines, surgery or radiation, a transplant with the person’s own cells (for some people) and clinical trials.
After diagnosis, the options described here
Medicines
Chemotherapy is given much as for high-risk medulloblastoma, and young children usually get it first to delay or avoid radiation.
Surgery or radiation
Surgery relieves fluid pressure and removes as much tumor as is safe, and older children usually have radiation to the brain and spine.
Transplant with the person’s own cells, For some people
Some plans for very young children add high-dose chemotherapy and a return of the child’s own stem cells, but the long-term benefit is not proven.
Clinical trials
There is no standard treatment for the youngest children, and the NCI says taking part in a clinical trial should be considered.
These are the kinds of treatment this page describes, not a plan. Which ones fit, in what order and whether they are combined differs from person to person.
Daily life and the donor’s role
Living with the condition
Treatment is long and is planned by a team that treats children’s brain tumors. A family may face brain surgery, a course of radiation and months of chemotherapy. High-dose chemotherapy with stem cell rescue adds more steps: the child’s stem cells are collected and frozen first, and after the high-dose treatment they are given back through a vein. Blood counts are low for a time, which raises the risk of infection.
Effects of the tumor and its treatment can last for years. The NCI lists possible late effects such as problems with growth, the thyroid, heart or hearing, changes in thinking, learning and memory, and second cancers. St. Jude notes that radiation to the pineal area can cause hormone problems, such as slow growth, tiredness and fertility problems, so hormone levels are checked over time.
After treatment, children have regular MRI scans and exams to watch for the tumor coming back. For pineoblastoma, NCI experts suggest spine checks every 6 months until 5 years after diagnosis. Social workers and psychologists can help families cope. A written survivorship care plan helps other doctors know what treatment the child had.
The donor’s role
No donor is involved in treating pineoblastoma. When a transplantA treatment that gives a patient healthy blood-forming stem cells through a vein. The cells travel to the bone marrow and replace faulty marrow or marrow damaged by treatment. They can come from the patient or a donor. is used, it is an autologous stem cell rescue. The child’s own blood-forming stem cells are collected and frozen before very high-dose chemotherapy, then given back to rebuild the blood and immune system. The NCI explains that these returned cells do not usually work against the cancer directly. They let the body recover from chemotherapy strong enough to destroy the blood-forming cells.
This step is used in some plans, mainly for children 3 and younger or for tumors that come back, and the evidence is limited. In a pooled study of North American and Australian children under 3, survival was not clearly different between the 12 who had it and the 20 who did not. An international expert group wrote in 2024 that its benefit for long-term survival remains unproven. European transplant experts (EBMT) list own-cell transplant for children’s brain tumors as developmental, meaning more trials are needed, and generally do not recommend a donor transplantComing from another person. In an allogeneic, or donor, transplant, the stem cells come from a relative or an unrelated volunteer whose cells are a close enough match to the patient's. for them.
Joining a stem cell registry does not help a child with pineoblastoma directly. Registry volunteers help people with leukemia and other conditions that do need donated cells.
Looking ahead
Looking ahead
Outlook for pineoblastoma
Outlook depends most on age and on whether the tumor has spread. Children aged 3 and older do much better than younger children. In a pooled study of 178 children from North America and Australia, about 67% of those aged 3 or older were alive 5 years later. Among those older children whose tumor had not spread, it was about 83%. For children under 3, it was about 16%.
The gene group matters too. In an international study of 221 children and adults with pineal tumors, about 70% of people with PB-miRNA1 and 100% with PB-miRNA2 were alive at 5 years. The figures were about 20% for PB-MYC/FOXR2 and about 30% for PB-RB1. These two groups are found mostly in very young children and often have already spread.
Most of these numbers come from children treated over several decades, often before gene groups were known. Experts are calling for studies of gentler treatment for lower-risk groups and of new medicines for the highest-risk groups.
About these numbers. They describe groups of people, not what will happen to any one person.
- 67%5-year survival, age 3 or older
122 children aged 3 and older with pineoblastoma, pooled from Children’s Oncology Group trials and hospital series in North America and Australia over about 30 years (published 2022)
Read the source: 5-year survival, age 3 or older - 16%5-year survival, under age 3
49 children under age 3 with pineoblastoma in the same pooled North American and Australian study (published 2022)
Read the source: 5-year survival, under age 3 - About 50%5-year survival, age 3 or older with spread at diagnosis
Children aged 3 and older whose pineoblastoma had spread when found (36 of 129 in that age group), in the same pooled North American and Australian study (published 2022)
Read the source: 5-year survival, age 3 or older with spread at diagnosis
These figures describe groups of children. They cannot predict what will happen to any one child.
Common questions
Is pineoblastoma cancer?
Yes. Pineoblastoma is a malignant (cancerous) brain tumor. It is grade 4, the highest grade, which means it grows fast and can invade nearby tissue. It can also spread through the fluid around the brain and spinal cord. It is different from a pineal cyst and from pineocytoma, a slow-growing grade 1 tumor of the same gland.
What is the survival rate for pineoblastoma?
It depends a lot on age, spread and gene group. A pooled study followed 178 children from North America and Australia. About 67% of children aged 3 or older were alive 5 years later. Among those older children whose tumor had not spread, about 83% were. For children under 3, it was about 16%. In an international study, 5-year survival ranged from about 20% to 100% by gene group. These are group figures, not a forecast for any one child.
Is pineoblastoma hereditary?
Usually not, but sometimes. Most cases are not linked to an inherited condition. Some children carry an inherited change in the RB1 gene (linked to retinoblastoma, an eye cancer) or the DICER1 gene. That is why an international expert group recommends genetic counseling for every patient with pineoblastoma, and the NCI says RB1 or DICER1 testing may be done. Genetic counselors can also help families talk about the results with relatives.
Does pineoblastoma need a stem cell or bone marrow transplant?
Not usually, and donor transplants are generally not recommended. Some treatment plans, mostly for children 3 and younger or for tumors that come back, use very high-dose chemotherapy followed by a return of the child’s own stem cells. The NCI lists it as an option for young children, but a 2024 international expert review says its benefit for long-term survival remains unproven. European transplant experts (EBMT) call it developmental for children’s brain tumors, meaning more trials are needed.
What is the difference between a pineal cyst and pineoblastoma?
A pineal cyst is a fluid-filled sac that is not cancer. Cysts are common: an international expert group says they are found by chance in about 1% of children and up to 23% of adults. Most need no treatment. Pineoblastoma is a solid, fast-growing cancer. MRI and, when needed, tissue tests tell them apart.
Can adults get pineoblastoma?
Yes, but it is less common. NCI-CONNECT says pineoblastomas are more common during the first 20 years of life, and an international expert group gives a mean age at diagnosis of 13. Another pineal tumor, pineal parenchymal tumor of intermediate differentiation (PPTID), is seen mostly in adults, so tumor testing matters at any age.
For your next appointment
Pineoblastoma
From the Jada Bascom Foundation disease library, jadabascomfoundation.org. Printed .
Questions to bring to your care team
- Has the tumor had methylation or gene testing, and which pineoblastoma group is it in?
- Should our child have genetic counseling and RB1 or DICER1 testing, and does that matter for brothers and sisters?
- Does the plan include high-dose chemotherapy with our child’s own stem cells, and what do you expect it to add?
- Which late effects on hormones, growth, learning and hearing will you watch for, and who will follow our child after treatment?
- Is there a clinical trial for our child’s age and tumor group?
- What is the exact name of the diagnosis or subtype, and what does it mean for treatment?
- What is the goal of each treatment you are suggesting?
- What would make a transplant worth considering later on?
- Where can our family find support during treatment?
A one-page list to take to the next appointment, with room for notes.
Supporting someone with a diagnosisSupport for patients and families
These independent organizations offer information and support. JBF is not affiliated with them.
- The Brain Tumour Charity Support and info line, online groups, counseling and information packs for children, adults and families affected by a brain tumor.United Kingdom
- Children's Brain Tumor Foundation Support groups, retreats, an educational advocacy program and bereavement support for children with brain and spinal cord tumors and their families.United States
- American Brain Tumor Association Free CareLine, support groups, mentor programs and financial help for people with brain tumors and their caregivers.United States
Sources and further reading
- Childhood Medulloblastoma and Other CNS Embryonal Tumors Treatment (PDQ)
NCI, Accessed 2026-09-05 - Indications for haematopoietic cell transplantation and CAR-T: 2025 EBMT practice recommendations
EBMT / Bone Marrow Transplantation, Accessed 2026-09-05 - Stem Cell and Bone Marrow Transplants for Cancer
NCI, Accessed 2026-09-05 - Childhood Medulloblastoma and Other Central Nervous System Embryonal Tumors Treatment (PDQ): Patient Version
NCI (PDQ, patient version), Updated 2024-12-02; accessed 2026-09-26 - Pineal Region Tumors: Diagnosis and Treatment
NCI-CONNECT (National Cancer Institute), Updated 2024-08-20; accessed 2026-09-26 - SNO-EANO-EURACAN consensus on management of pineal parenchymal tumors
Neuro-Oncology (Liu et al., SNO-EANO-EURACAN consensus; via PMC), 2024-07-29 - Pediatric pineoblastoma: A pooled outcome study of North American and Australian therapeutic data
Neuro-Oncology Advances (Hansford et al.; via PMC), 2022 - Pineoblastoma in Children and Teens
St. Jude Children’s Research Hospital (Together by St. Jude), Reviewed August 2026; accessed 2026-09-26 - DICER1 syndrome
MedlinePlus Genetics (U.S. National Library of Medicine), Accessed 2026-09-26
This information explains a condition and its treatments. It cannot diagnose an illness or recommend treatment for an individual. Your care team can explain how the evidence applies to you. Written and source-checked by the Jada Bascom Foundation. Each page lists the published sources it draws on.
Ways to help
Help another family understand.
Most people with pineoblastoma are treated without a registry donor. A clear explanation can help the next family who hears this diagnosis, and many people with other blood cancers and blood disorders need a donor who is a stranger.
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