Overview
Pineocytoma (also known as pinealocytoma) is a rare, slow-growing brain tumor that arises from the pineal gland, a small endocrine organ located deep in the center of the brain. It is classified as a World Health Organization (WHO) grade I pineal parenchymal tumor, meaning it is the most benign form of pineal parenchymal neoplasm. The pineal gland normally produces melatonin, a hormone involved in regulating sleep-wake cycles. Pineocytomas are composed of well-differentiated pinealocytes (the mature cells of the pineal gland) and tend to grow slowly with well-defined borders. The primary effects of pineocytoma on the body result from the tumor's location rather than its biological aggressiveness. As the tumor grows, it can obstruct the cerebral aqueduct, leading to obstructive hydrocephalus (a buildup of cerebrospinal fluid in the brain). This can cause symptoms such as headaches, nausea, vomiting, visual disturbances (particularly Parinaud syndrome, characterized by difficulty looking upward), and gait abnormalities. Some patients may also experience fatigue, sleep disturbances, or hormonal changes related to disruption of pineal gland function. In some cases, the tumor may be discovered incidentally on brain imaging performed for other reasons. The primary treatment for pineocytoma is surgical resection, which can be curative when complete removal is achieved. Due to the deep location of the pineal gland, surgery can be technically challenging and may carry risks related to surrounding critical brain structures. When hydrocephalus is present, cerebrospinal fluid diversion procedures (such as ventriculoperitoneal shunting or endoscopic third ventriculostomy) may be necessary. The role of radiation therapy is generally reserved for cases of incomplete resection or recurrence. The overall prognosis for pineocytoma is favorable, with high long-term survival rates following successful surgical treatment. Regular follow-up with neuroimaging is recommended to monitor for recurrence.
Clinical phenotype terms— hover any for plain English:
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
Treatments
1 availableAzedra Ultratrace
AZEDRA is indicated for the treatment of adult and pediatric patients 12 years and older with iobenguane scan positive, unresectable, locally advanced or metastatic pheochromocytoma or paraganglioma w…
AZEDRA is indicated for the treatment of adult and pediatric patients 12 years and older with iobenguane scan positive, unresectable, locally advanced or metastatic pheochromocytoma or paraganglioma who require systemic anticancer therapy.
Clinical Trials
View all trials with filters →No actively recruiting trials found for Pineocytoma at this time.
New trials open frequently. Follow this disease to get notified.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Pineocytoma.
Community
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Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Common questions about Pineocytoma
What is Pineocytoma?
Pineocytoma (also known as pinealocytoma) is a rare, slow-growing brain tumor that arises from the pineal gland, a small endocrine organ located deep in the center of the brain. It is classified as a World Health Organization (WHO) grade I pineal parenchymal tumor, meaning it is the most benign form of pineal parenchymal neoplasm. The pineal gland normally produces melatonin, a hormone involved in regulating sleep-wake cycles. Pineocytomas are composed of well-differentiated pinealocytes (the mature cells of the pineal gland) and tend to grow slowly with well-defined borders. The primary effe
How is Pineocytoma inherited?
Pineocytoma follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Pineocytoma typically begin?
Typical onset of Pineocytoma is adult. Age of onset can vary across affected individuals.
Which specialists treat Pineocytoma?
20 specialists and care centers treating Pineocytoma are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.