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Diseases

Genetic and Rare Diseases Information Center (GARD)

Other Names for this Disease
  • Pinealocytoma
  • Pinealoma
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Your Question

An MRI identified an unusual, one centimeter nodule on the edge of a pineal cyst.  How could this nodule be differentiated as a complicated pineal cyst or a pineocytoma?  What are the best treatment options for either a complicated pineal cyst or a pineocytoma if the patient is experiencing symptoms?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What are pineal cysts?

Pineal cysts are cysts of the pineal gland, a small organ in the brain shaped like the seed of a pine cone that is responsible for making melatonin, a hormone that regulates sleep.  Pineal cysts may be found by chance in up to 10% of people undergoing CT or MRI brain imaging, and they occur in three times more women than men.  Most pineal cysts do not cause symptoms and stay the same size over time (remain stable).[1]  In the rare circumstance where a pineal cyst does cause symptoms, it may cause headaches, hydrocephalus, eye movement abnormalities, and Parinaud syndrome.[2]  Because most pineal cysts do not grow or cause symptoms, there are no established guidelines for routine follow-up for individuals with pineal cysts.  Treatment is usually only considered when a cyst is causing symptoms.[3]  In the past, treatment for symptomatic pineal cysts included surgery to remove the cyst (craniotomy); more recently, less invasive procedures such as stereotactic aspiration or endoscopic treatment have been recommended.[4]
Last updated: 10/16/2012

What is a pineocytoma?

A pineocytoma is a tumor of the pineal gland, a small organ in the brain that makes melatonin, a hormone which regulates sleep.  Pineocytomas most often occur in adults as a solid mass, though it may appear to have fluid-filled (cystic) spaces on images of the brain.  Pineocytomas are usually slow-growing and do not usually spread to other parts of the body.[3]  Treatment includes surgery to remove the pineocytoma; most of these tumors do not regrow (recur) after surgery.[5]
Last updated: 12/20/2011

How might a complicated pineal cyst be distinguished from a pineocytoma?

It can be difficult to distinguish a complicated pineal cyst from a pineocytoma.  In some cases, imaging of the brain may help.  It is important that a chemical known as contrast material be given during a CT scan to help determine a pineal cyst from a pineocytoma.  During a CT scan, a pineal cyst will look like a white, smooth, thin (two millimeters or less) rim around a dark, empty space.  A pineocytoma will look like a large, round white spot, or as a thickening (greater than 2 millimeters) in the rim of a cyst.[3]  It is important to take note of the time that has passed between the giving of contrast material and when the CT images are taken.  If too much time passes, the contrast material can leak into the fluid-filled middle of a pineal cyst and cause the whole cyst to look like a large, round white spot, which would mimic the appearance of a pineocytoma.[1]  Because a complicated pineal cyst could look quite similar to a pineocytoma on brain imaging tests, it may not be possible to distinguish between the two by imaging only.[3][1]
Last updated: 12/20/2011

How might symptomatic pineal cysts be treated?

Whether patients with symptomatic pineal cysts should be monitored and/or treated is still unclear.  However, surgery is usually recommended for those people with pineal cysts who have hydrocephalus, worsening neurological symptoms, or enlargement of the cyst over time.[6]  Treatment may include surgery to remove the cyst, sometimes followed by the placement of a ventriculoperitoneal shunt. As of 1993, an alternative treatment option has been aspiration of the contents of the cyst using ultrasound guidance.  More recently, endoscopic procedures have been used.[4]
Last updated: 12/29/2011

How might a pineocytoma be treated?

Because pineocytomas are quite rare, there are no consensus guidelines on the best treatment for these tumors.[7]  However, surgery to remove the entire tumor is considered the standard treatment.  Because these tumors are located deep in the brain, it is important that the risks of surgery in this part of the body be carefully considered in each individual patient.[5]  Radiation therapy has sometimes been used following surgery to destroy any cancer cells that may remain, but the benefit of this additional treatment is questionable.[7]
Last updated: 12/20/2011

References
  • Fakhran S, Escott EJ. Pineocytoma mimicking a pineal cyst on imaging: true diagnostic dilemma or a case of incomplete imaging?. American Journal of Neuroradiology. 2008; 29:159-163. http://www.ncbi.nlm.nih.gov/pubmed/17925371. Accessed 12/13/2011.
  • Al-Holou WN, Maher CO, Muraszko KM, Garton HJL. The natural history of pineal cysts in children and young adults. J. Neurosurg. Pediatrics. 2010;
  • Gaillard F, Jones J. Masses of the pineal region: clinical presentation and radiographic features. Postgraduate Medical Journal. 2010; 86:597-607. http://www.ncbi.nlm.nih.gov/pubmed/20971711. Accessed 12/13/2011.
  • Costa F, Fornari M, Valla P, Servello D. Symptomatic Pineal Cyst: Case Report and Review of the Literature. Minim. Invas. Neurosurg.. 2008; 51:231-233.
  • Dahiya S, Perry A. Pineal Tumors. Advances in Anatomic Pathology. 2010; 17:419-427. http://www.ncbi.nlm.nih.gov/pubmed/20966647. Accessed 12/13/2011.
  • Taraszewska A, Matyja E, Koszewki W, Zaczynski A, Bardadin K, Czernicki Z. Asymptomatic and symptomatic glial cysts of the pineal gland. Folia Neuropathol. 2008;
  • Clark AJ, Ivan ME, Sughrue ME, Yang I, Aranda D, Han SJ, Kane AJ, Parsa AT. Tumor control after surgery and radiotherapy for pineocytoma. Journal of Neurosurgery. 2010; 113:319-324. http://www.ncbi.nlm.nih.gov/pubmed/20136388. Accessed 12/13/2011.