Pineal Region Tumor
Make an Appointment
Our team of dedicated access representatives is here to help you make an appointment with the specialists that you need.
A pineal region tumor is an abnormal growth in or near the pineal gland. Depending on the type of tumor, surgery may involve a craniotomy to remove it.
The pineal gland, a tiny, pinecone-shaped area located in the midline of the brain behind the third ventricle, synthesizes and secretes the neurotransmitters melatonin, which plays the critical role of regulating the body’s circadian rhythm, and serotonin, a precursor to melatonin. Other functions of the pineal gland remain incompletely understood. This gland is composed of a variety of cells, such as glial cells, endothelial cells, sympathetic nerve cells, pineal parenchymal cells. and germ cells. Given the diversity of cells, a wide variety of tumor types can arise in this region.
Symptoms
Regardless of the type, tumors in the pineal region usually cause symptoms through one of three mechanisms.
- Increasing pressure in the skull by blocking the flow of cerebrospinal fluid, leading to hydrocephalus
- Compressing part of the brain and surrounding structures
- Disrupting the endocrine system, which controls hormone production and secretion, including those hormones produced by the pineal gland
Hydrocephalus is a common symptom of tumors in this region and leads to headaches and possibly nausea, vomiting and altered mental status. The brainstem and the cerebellum are the most common sites of compression from a pineal region tumor and can cause abnormal eye movements (such as Parinaud syndrome), diplopia, ataxia or unsteady gait. Endocrine dysfunction is rare and usually is caused by hydrocephalus or the tumor spreading to the nearby hypothalamus.
Diagnosis
A neurological examination is usually performed to identify symptoms and any problems. This exam consists of evaluating eye movements, hearing, sensation, motor function, swallowing, sense of smell, balance, and coordination.
As with other brain tumors, imaging studies are the key component in the diagnosis of pineal region tumors. Magnetic resonance imaging (MRI) scans or computed tomography (CT) scans can be used to visualize the tumor and nearby structures in high detail. MRI scan, which can indicate the presence of hydrocephalus, is usually the preferred imaging test. MRI or CT scans may be performed with or without contrast enhancement.
A blood draw is performed to measure the biochemical markers alpha-fetoprotein and beta-human chorionic gonadotropin present in the blood, and a sample of cerebrospinal fluid may also be taken. Germ cell tumors can be differentiated by these biochemical markers.
Risk Factors
The cause of pineal region tumors, like most brain tumors, is currently unknown.
Pineal region tumors are rare among adults, making up less than 1 percent of adult brain tumors, and are less rare among children, making up 3 to 8 percent of childhood brain tumors. For children, these tumors tend to occur between the ages of 10 and 20, and for adults, these tumors tend to occur after age 30.
Dermoid and epidermoid cysts can be present at birth and yet remain asymptomatic for years. Dermoid cysts tend to show symptoms in children younger than 10, and epidermoid cysts tend to show symptoms in middle-aged adults.
Treatments
At Columbia, our neurosurgeons use the latest surgical techniques to successfully treat pineal region tumors, providing patients with the best possible outcome.
For the majority of pineal region tumors, the standard first step of treatment is surgery. Historically, surgically removing a tumor from the pineal region of the brain has been a challenge because this is a hard-to-reach location. Advances have made surgery a successful treatment option.
Surgery involves a neurosurgeon performing a craniotomy, a procedure in which part of the skull is removed to gain access to the tumor, allowing the tumor to be excised. If hydrocephalus is present, a shunt may be placed before surgery.