Observing a brain scan

Pineal Region Tumors

What are Pineal Region Tumors?

Pineal region tumors are a group of tumors that develop in the area of the brain known as the pineal region, which is located near the pineal gland. The pineal gland is a small endocrine gland responsible for producing melatonin, a hormone involved in regulating sleep-wake cycles. Pineal region tumors can arise from various cell types within this region, leading to different tumor types and characteristics.

Pineal region tumors can occur at any age but are more commonly found in children and young adults. The clinical presentation of these tumors can vary depending on their size, location, and the structures they affect. Symptoms may include headaches, nausea, vomiting, visual disturbances, hormonal abnormalities, sleep disturbances, and behavioral changes. The specific symptoms depend on the tumor type and the compression of surrounding structures.

Types of Pineal Region Tumors

There are several types of pineal region tumors, including pineal parenchymal tumors, germ cell tumors, and gliomas. Pineal parenchymal tumors include pineocytomas and pineoblastomas, which arise from specialized cells in the pineal gland. Germ cell tumors originate from cells that would normally develop into sperm or eggs and can include germinomas, teratomas, embryonal carcinomas, yolk sac tumors, and choriocarcinomas. Gliomas in the pineal region are rare but can occur.

Treatment for Pineal Region Tumors

Minimally invasive keyhole operations are now possible for Pineal Region Tumors

The optimal treatment for patients with a pineal tumor depends upon the tumor subtype and may include surgical removal, radiation therapy and/or chemotherapy. Fortunately, for the majority of pineal tumors that warrant surgical biopsy or removal, they can be accessed via a minimally invasive keyhole endoscopic approach passing over the cerebellum. At the BTC, we have a large experience in these keyhole procedures.

By incorporating cutting edge technology and instrumentation with proven surgical experience, we make surgery safer, less invasive and more effective. Our neurosurgeons work closely with our radiation oncologists, neuro-oncologists and neuro-pathologists to design the optimal treatment plan that incorporates the latest in radiotherapy techniques and tumor biomarker studies.

Overview

The pineal gland lies deep within the brain and regulates circadian rhythm. Occasionally, tumors or cysts can arise from this gland which may require surgery, radiation or chemotherapy.

As these tumors grow, they can compress or invade surrounding brain structures, causing various neurological deficits. Due to their location to the fluid filled regions of the brain, the ventricles, they can also cause increased fluid build-up and intracranial pressure due to hydrocephalus. These lesions can range from benign cysts to malignant tumors.

Symptoms

Symptoms of these tumors/cysts can vary depending on their type and effect on the surrounding brain structures. Typical symptoms include headaches, particularly those that are worse lying down, coughing, or upon awakening.

Other findings include vision difficulties, double vision, concentration difficulties, memory impairment and altered states of consciousness.

Diagnosis

As the treatment(s) of these lesions vary greatly on the diagnosis, numerous tests may be necessary prior to surgery. Typical imaging studies include magnetic resonance imaging (MRI) or computer tomography (CT) scans of the brain. Specific high resolution MRI sequences can further characterize these tumors or cysts. The cerebrospinal fluid (CSF) may need to be analyzed via a lumbar puncture (spinal tap).

In specific situations, surgical biopsy may be necessary prior to definitive therapy. The most common pineal region tumors requiring treatment include pineocytomas, pineoblastomas, germinomas, teratomas, astrocytomas, ependymomas, papillary tumors and occasionally pineal cysts.

Treatment

In many cases, the pineal lesion may be a benign cyst that may be observed with serial MRIs. In certain scenarios with specific imaging and CSF findings, chemotherapy and/or stereotactic radiosurgery may be indicated. Most tumors, however, will require surgical debulking or resection. Given recent advances, this surgical approach is typically performed endoscopically using a high-definition surgical telescope through a small bony opening at the back of the head working above the cerebellum and below a dural structure called the tentorium.

Biopsies could also be performed endoscopically through the ventricles. In cases with hydrocephalus – fluid buildup – due to the tumor, endoscopic “bypass” of the fluid (endoscopic third ventriculostomy) or extracranial bypass (ventriculoperitoneal shunt) may be indicated. Occasionally, biopsies and “bypasses” can be performed in the same setting.  In many cases, chemotherapy and/or fractionated stereotactic radiotherapy may still be necessary following surgery.


Doctors and Specialists Who Treat Pineal Region Tumors

Written and reviewed by:
We are a highly specialized team of medical professionals with extensive neurological and cranial disorder knowledge, expertise and writing experience.
Last updated: June 30, 2023