Rathke cleft cyst

Last revised by Joshua Yap on 19 May 2023

Rathke cleft cysts, also known as pars intermedia cysts, are non-neoplastic, sellar or suprasellar epithelium-lined cysts arising from the embryologic remnants of Rathke pouch in the pituitary gland. They are common lesions and usually incidentally identified. 

  • common, found in ~15% (range 11-22%) of autopsies 2,6

  • rare in childhood; mostly seen in adults

    • since the underlying substrate is congenital, lesions thought to enlarge throughout life 2

  • recognized female preponderance (F:M = ~2:1)

The vast majority of Rathke cleft cysts, particularly when <3 mm, are asymptomatic and incidentally found 3

If large, they may cause:

  • visual disturbances: due to compression of the optic chiasm 

  • pituitary dysfunction

    • due to compression of adjacent pituitary tissue and distortion of the pituitary stalk

    • present in up to 69% of symptomatic cases 10

  • headaches 3

Rathke pouch forms during the 4th week of embryologic development as a rostral outpouching from the roof of the primitive oral cavity. The anterior wall of the pouch gives rise to the anterior lobe of the pituitary (pars distalis). The posterior wall of the pouch does not proliferate and remains as the intermediate lobe of the pituitary (pars intermedia). The lumen of the pouch narrows to form a cleft (Rathke cleft) that normally regresses. Persistence of this cleft with the expansion is believed to be the origin of a Rathke cleft cyst.

The wall of the cyst is typically lined by a single columnar cell layer of epithelium, often containing goblet cells, and is often ciliated. An intraluminal nodule which macroscopically appears white and is often adherent to the cyst wall (although it may be free-floating) is composed of solid tissue that represents desquamated cellular debris 3,10.

On imaging, a Rathke cleft cyst is seen as a well defined non-enhancing midline cyst within the sella arising between the anterior and intermediate lobes of the pituitary. 40% are purely intrasellar and 60% have suprasellar extension. Purely suprasellar location, although reported, is rare.

On lateral skull x-ray, it can rarely cause sellar enlargement if reaches large size 12.

  • non-contrast

    • typically non-calcified and of homogeneous low attenuation

    • uncommonly it may be of mixed iso- and low-attenuation, or contain small curvilinear calcifications in the wall (seen in 10-15% of cases)

  • post-contrast

    • ​​typically non-enhancing, although the cyst wall may enhance in some cases 8

Rathke cleft cysts are usually in the midline, unicameral (without septations) and homogeneous in signal intensity 13. Fluid fluid levels are uncommon and are more suggestive of a pituitary adenoma 13.

The signal characteristics vary according to the cyst composition, which may be mucoid or serous.

  • T1

    • 50% are hyperintense (high protein content)

    • 50% are hypointense

  • T2

    • 70% are hyperintense

    • 30% are iso or hypointense

    • 20% have a hypointense rim (this is more common in adenomas) 13

  • T1 C+ (Gd)

    • no contrast enhancement of the cyst is seen; however, a thin enhancing rim of surrounding compressed pituitary tissue may be apparent 9,10

An intracystic nodule (sometimes referred to as intracystic dot) is present in 65-75% of cases 3,13. When present it is usually hyperintense on T1 and hypointense on T2 compared to surrounding fluid, although, depending on the signal of the surrounding fluid, it may be inapparent on one sequence or the other 3.

Surgical treatment is indicated for symptomatic cases.

A case series showed 18% recurrence rate after surgical resection, supporting the theory that a relationship exists between symptomatic Rathke cleft cyst and craniopharyngioma 11.

The main differential diagnoses are:

  • cystic pituitary adenoma

    • fluid-fluid level or hemorrhagic debris 11,13

    • off midline location 13

    • septations 13

  • craniopharyngioma

    • no gender difference

    • similar age group

    • usually suprasellar or have a suprasellar component

    • tend to calcify

  • arachnoid cyst

    • older patients

    • no gender difference

  • epidermoid cyst

    • usually suprasellar

    • restriction on DWI

  • teratoma

    • usually has solid components

    • often fatty signal

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Cases and figures

  • Figure 1: histology
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  • Case 1: with large T2 hypointense intracystic nodule
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  • Case 2: hyperdense on CT
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  • Case 3: large with suprasellar extension
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  • Case 4
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  • Case 5
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  • Case 6: with sellar enlargement
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  • Case 7
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  • Case 8: T2 hypointense cyst
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  • Case 9: with large intracystic nodule
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  • Case 10
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  • Case 11: large cyst with claw sign
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  • Case 12
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  • Case 13: with intracystic nodule
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  • Case 14: large with suprasellar component
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  • Case 15
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  • Case 16: growing Rathke cleft cyst
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  • Case 17: with intracystic nodule
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