What are the other Names for this Condition? (Also known as/Synonyms)

• Pars Intermedia Cyst
• Rathke’s Cleft Cyst
• RCC (Rathke Cleft Cyst)

What is Rathke Cleft Cyst? (Definition/Background Information)

• Rathke Cleft Cyst (RCC) is a type of fluid-filled lesion that forms in the Rathke’s pouch, near the pituitary glands in the brain. RCC is a benign formation and is not considered a true tumor; it is usually an incidental finding during autopsies
• The pituitary gland is made up of two glands, namely the anterior pituitary (adenohypophysis) and the posterior pituitary (neurohypophysis). The space between the anterior and posterior pituitary gland is called the Rathke's pouch
• Rathke Cleft Cysts are common lesions and about two-thirds of these cysts are diagnosed in women. The risk factors and cause of RCCs are unknown
• Small-sized Rathke Cleft Cysts do not present any significant signs and symptoms; and these form the majority. However, large tumors may cause significant symptoms including severe hormonal disruptions and vision impairment
• Many individuals with Rathke Cleft Cyst may not require any treatment. However, a surgery may be considered in case of severe symptoms, as determined by the healthcare provider. The prognosis is typically good for most individuals with Rathke Cleft Cysts

Who gets Rathke Cleft Cyst? (Age and Sex Distribution)

• Rathke Cleft Cysts are common lesions found in individuals of all age groups; although, the cysts are much more common in adults than children
• Both males and females are affected, but a female preference is observed (2:1 female-male ratio)
• Individuals of all racial and ethnic background may be affected

What are the Risk Factors for Rathke Cleft Cyst? (Predisposing Factors)

• Currently, no risk factors have been identified for Rathke Cleft Cyst
• Gender: Over 65% of these cysts are noted in females

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Rathke Cleft Cyst? (Etiology)

The exact cause of development of Rathke Cleft Cyst (RCC) is unknown.

• RCC is a benign cyst that is known to arise from the Rathke pouch (embryological) remnants, which during normal fetal development closes by itself
• A persistence of the pouch occasionally leads to fluid accumulation within the cleft (divided space) resulting in the formation of the Rathke Cleft Cyst
• This space is narrow and small; however, it can become large following cyst formation

Some researchers believe that RCC and craniopharyngioma (a form of brain tumor) may be related to each other.

What are the Signs and Symptoms of Rathke Cleft Cyst?

The signs and symptoms of Rathke Cleft Cyst (RCC) can vary from one individual to another and depends on the size of the cystic tumor. In general, a vast majority of them are small, and no evident symptoms are noted. Significant signs and symptoms usually occur in adults from large-sized cysts causing compression due to mass effect, frequently resulting in vision impairment and pituitary dysfunction causing endocrinological abnormalities.

The signs and symptoms may include:

• Headaches
• Nausea and vomiting
• Involvement of the optic chiasm can cause loss of visual acuity (keenness), poor color rendering (colors appear faded), and loss of peripheral and central vision
• Compression of the pituitary gland can cause a host of associated problems due to hypopituitarism. This results in reduced hormone production and output causing abdomen pain, decrease in appetite, excess thirst and excess urination, fatigue, nausea and vomiting, anemia, and other issues including mental impairment
• Pituitary gland dysfunction causing reduced production of hormones, such as luteinizing hormone (LH), sex hormones (testosterone and estrogen), and follicle-stimulating hormone (FSH), may be observed in nearly 70% of the individuals showing symptoms
• Hyperprolactinemia or increased production of the prolactin hormone, causing menstrual irregularities, breast pain, milky nipple discharge in women (galactorrhea), and reduced breastmilk output
• Lack of sexual drive in men and women

How is Rathke Cleft Cyst Diagnosed?

A majority of cystic brain tumors may remain undiagnosed for prolonged periods, if they are asymptomatic and generally slow-growing. The slow development of symptoms may contribute to a delayed detection and diagnosis of these cysts. They may be detected when there is a sudden worsening of symptoms prompting the healthcare provider to perform radiological studies of the brain.

Rathke Cleft Cysts (RCCs) are often incidental findings on autopsies. According to a particular large study, RCCs were found in about 15-20% of the autopsies.

The diagnosis of Rathke Cleft Cyst may involve the following tests and examinations:

• Complete physical examination and a thorough medical history
• Assessment of the presenting signs and symptoms
• Blood tests to assess hormonal imbalances and serum electrolytes
• Visual field tests; tests to assess visual acuity
• Neurological, motor skills, and cognitive assessment:
  • Checking intellectual ability, memory, mental health and function, language skills, judgment and reasoning, coordination and balance, reflexes, and sensory perceptions (space, sight, hearing, touch, etc.)
  • The healthcare provider/neurologist may use the Karnofsky Performance Scale in order to assess the neurological functioning of the individual’s central nervous system (CNS)
• Electroencephalography (EEG)
• Electromyography with nerve conductivity tests
• Imaging studies that may be performed include:
  • X-ray of head and neck region
  • Computerized tomography (CT) scan of the head and neck region; CT with contrast
  • Magnetic resonance imaging (MRI) scan of the central nervous system (brain and spine); MRI with contrast agents such as gadolinium
  • Cerebral angiographic studies or MR angiography: An angiogram involves injecting dye into the bloodstream, which makes the blood vessels to appear visually on X-rays. The X-ray may show a tumor in the brain or the blood vessels leading into the tumor
  • Magnetic resonance spectroscopy: This radiological technique is used to study the chemical profile of the tumor. It is often performed with and compared to corresponding MRI scan images of the affected region
• Cerebrospinal fluid analysis, where a spinal tap or lumbar puncture procedure may be performed: This diagnostic test is used to remove a sample of cerebrospinal fluid (CSF) from the spaces in and around the brain and spinal cord. The sample is removed from the lower spinal cord using a thin needle, and it is then checked for the presence of cancer cells
• Molecular studies, if necessary

Tissue biopsy: A biopsy of the affected region (brain) is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy sample under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy sample under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis.

A differential diagnosis to eliminate other conditions or tumor types may be considered, before arriving at a definitive diagnosis.

The radiological differential diagnosis may include:

• Arachnoid cyst
• Craniopharyngioma
• Cystic pituitary adenoma

The pathological differential diagnosis may include:

• Intermediate lobe cysts
• Papillary craniopharyngioma

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Rathke Cleft Cyst?

The complications of Rathke Cleft Cyst may include the following:

• Stress and anxiety due to fear of a brain tumor
• Large cysts may cause total blindness, if undiagnosed and/or left untreated
• Central diabetes insipidus, from pituitary gland dysfunction
• Severe hormonal disturbances leading to reduced quality of life
• Infertility in women and impotence in men
• Large tumor masses may get secondarily infected with bacteria or fungus
• Recurrence of the cystic tumor following treatment; around 18% of the cysts are known to recur
• Damage to the muscles, vital nerves, and blood vessels, during surgery
• Post-surgical infection at the wound site is a potential complication

How is Rathke Cleft Cyst Treated?

The treatment of Rathke’s Cleft Cyst may involve the following:

• In a great majority of cases of small-sized and asymptomatic tumors, the healthcare provider may consider conservative ‘wait and watch’ measures. In individuals with no symptoms, no treatment may be necessary
• Hormonal disturbances caused by pituitary dysfunction are treated using hormonal supplements
• Generally, a complete surgical excision may be undertaken for large cystic tumors presenting severe symptoms
  • The surgeon may use certain microsurgical approaches, such as an endoscopic brain surgery, to drain cyst contents and/or remove the cyst 
  • Surgery to remove Rathke Cleft Cysts offer a lot of challenges to healthcare providers, due to the considerable complexity of the region
• Follow-up care with regular screening and check-ups are important and encouraged

How can Rathke Cleft Cyst be Prevented?

Currently, there are no known methods available to prevent the occurrence of Rathke Cleft Cysts.

What is the Prognosis of Rathke Cleft Cyst? (Outcomes/Resolutions)

• In a vast majority of individuals, the prognosis for Rathke Cleft Cyst is excellent for small-sized asymptomatic cysts, even in the absence of any treatment
• Large-sized tumors may require suitable treatment in the form of surgery. In such cases, the overall prognosis depends on the initial set of symptoms and post-operative complications noted, if any
• Following surgery, regular medical screening at periodic intervals with blood tests, scans, and physical examinations are recommended, due to higher incidence of recurrences

Additional and Relevant Useful Information for Rathke Cleft Cyst:

The following article link will help you understand other cancers and benign tumors:

http://www.dovemed.com/diseases-conditions/cancer/