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Genetic and Rare Diseases Information Center (GARD)

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Pineal cyst

*

* Not a rare disease

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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 which is a small organ in the brain that produces melatonin (a sleep-regulating hormone). Pineal cysts are relatively common and may be found by chance in up to 10% of people undergoing CT or MRI brain imaging. The exact cause of pineal cysts is unknown. Most people with pineal cysts do not have any signs or symptoms.[1] Rarely, a pineal cyst may cause headaches, hydrocephalus, eye movement abnormalities, and Parinaud syndrome.[2] Treatment is usually only considered when a cyst is causing symptoms, in which case surgery, stereotactic aspiration or endoscopic treatment may be recommended.[3][4]
Last updated: 11/13/2014

What is a pineocytoma?

A pineocytoma is a tumor of the pineal gland, a small organ in the brain that makes melatonin (a sleep-regulating hormone). Pineocytomas most often occur in adults as a solid mass, although they may appear to have fluid-filled (cystic) spaces on images of the brain. Signs and symptoms of pineocytomas include headaches, nausea, hydrocephalus, vision abnormalities, and Parinaud syndrome.[5] Pineocytomas are usually slow-growing and rarely spread to other parts of the body.[4] Treatment includes surgery to remove the pineocytoma; most of these tumors do not regrow (recur) after surgery.[6]
Last updated: 11/13/2014

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. For example, a chemical known as contrast material can be given during a CT scan to help distinguish 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.[4] However, a complicated pineal cyst could look quite similar to a pineocytoma on brain imaging tests so it may not be possible to distinguish between the two by imaging alone.[4][1]
Last updated: 11/13/2014

How might pineal cysts be treated?

The best treatment options for pineal cysts depend on many factors, including the size of the cyst and whether or not it is associated with symptoms. For example, people with pineal cysts that do not cause symptoms may not require any form of treatment.[5] However, they may need to have regular check-ups with a physician and follow up imaging if they have a large cyst (greater than 10-14 mm) or develop symptoms that could be related to the cyst.[5]

Treatment is often recommended for those individuals with pineal cysts that cause hydrocephalus; neurological symptoms such as headache or disturbance of vision; or enlargement of the cyst over time.[7][2] Treatment may include surgery to remove the cyst, sometimes followed by the placement of a ventriculoperitoneal shunt. Aspiration of the contents of the cyst using ultrasound guidance has been explored as an alternative approach to surgery, and more recently, endoscopic procedures have been used.[3] Radiation therapy may be recommended for cysts that recur after treatment.[5]

Last updated: 11/13/2014

How might a pineocytoma be treated?

Because pineocytomas are quite rare, there are no consensus guidelines on the best treatment for these tumors.[8] 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 be carefully considered in each person.[6]  Radiation therapy is sometimes used following surgery to destroy any tumor cells that may remain, but the benefit of this additional treatment is questionable.[8]
Last updated: 11/13/2014

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. February 2010; 5(2):162-166.
  • 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.
  • 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.
  • Maria Moschovi, MD; George P Chrousos, MD. Pineal Gland Masses. UpToDate. October 2013;
  • 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; 46(3):186-195.
  • 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.
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.