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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Pineal cyst

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* Not a rare disease
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Overview



What are pineal cysts?

What are the signs and symptoms of pineal cysts?

What causes pineal cysts?

How might symptomatic pineal cysts be treated?


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 are the signs and symptoms of pineal cysts?

Pineal cysts are usually asymptomatic. However, there are rare cases of symptomatic pineal cysts which cause headaches, hydrocephalus, gaze palsy, Parinaud syndrome, and vertigo.[5] Less frequently, patients with symptomatic pineal cysts may present with ataxia, motor and sensory impairment, mental and emotional disturbances, seizures, circadian rhythm disturbances, hypothalamic dysfunction of precocious puberty, and secondary parkinsonism.[6]
Last updated: 12/29/2011

What causes pineal cysts?

Although the exact cause of pineal cysts is unknown, several theories have been proposed based on autopsy findings. One theory is that pineal cysts arise from the pineal recess of part of the brain called the third ventricle. Others have proposed that pineal cysts form as a result of restriction of blood flow and degeneration of glial plaques (deposits that buildup). According to another theory, pineal cysts form in place of a cluster of degenerated group of cells called pinealocytes.[7]
Last updated: 4/8/2010

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.[8]  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

References
  1. 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.
  2. Al-Holou WN, Maher CO, Muraszko KM, Garton HJL. The natural history of pineal cysts in children and young adults. J. Neurosurg. Pediatrics. 2010;
  3. 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.
  4. 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.
  5. Wajd NA, Maher CO, Muraszko KM, Garton HJL. The natural history of pineal cysts in children and young adults. J. Neurosurg. Pediatrics. 2010;
  6. Bosnjak J, Budisic M, Azman D, Strineka M, Crnjakovic M, Demarin V. Pineal gland cysts--an overview. Acta Clin Croat. 2009;
  7. Wajd NA, Garton HJL, Muraszko KM, Ibrahim M, Maher CO. Prevalence of pineal cysts in children and young adults. J. Neurosurg. Pediatrics. 2009;
  8. Taraszewska A, Matyja E, Koszewki W, Zaczynski A, Bardadin K, Czernicki Z. Asymptomatic and symptomatic glial cysts of the pineal gland. Folia Neuropathol. 2008;