RATHKE CLEFT

CYSTS

H. ZAGHOUANI, N. EZZAIRI, A.BEN ABDALLAH,S. YAHYAOUI, S.

MAJDOUB,T. RZIGUA, L. BEN CHRIFA,H. AMARA, D. BEKIR, CH.
KRAIEM
Departement of radiology, Hospital of Farhat Hached, Sousse,
Tunisia

NR37
 INTRODUCTION:
◦ Rathke’s cleft cysts (RCCs) are benign congenital, non-
neoplastic sellar and/ or suprasellar lesions originating
from epithelial remnants of Rathke’s pouch .

◦ These cysts are extremely common, found during routine

autopsies in 13% to 22% of normal pituitaries.
◦ In 1913, Goldzieher described the first case of RCC as an incidental postmortem
finding.

◦ The description of RCC has expanded since the advent of computed tomography
(CT) scanning and magnetic resonance imaging (MRI), showing that the incidence of
this disease wich discovery was only by autopsy, was underestimated.
OBJECTIVES:
The aim of this work is to emphasize the value of MRI in
the positive and differential diagnosis of this disease
through a retrospective study of 5 cases of Rathke’s cleft
cyst with a literature review.
MATERIALS AND
METHODS
◦ We retrospectively analyzed the records of five patients
with Rathke’s cleft cysts collected in the service of
radiology in the hospital of Farhat Hached Sousse.

◦ A brain MRI was performed for all patients. All the cysts
were discovered incidentally.
◦ There were two female and three male patients ranging in
age from 14 to 48 years.

◦ MR examinations were performed using 1.5-T imagers.

◦ Axial and coronal T1-weighted images were obtained

without administration of gadolinium.

◦ With axial and coronal FSE T2-weighted images.

◦ Section thickness was 4 mm, with intersection spacing of

0%.
RESULTS:
 The signal, shape, size, seat and reports of the lesions were
analyzed.

 It showed in all cases a cystic formation of the sellar region

with variable signals and sizes.

 The localization of the cysts was strictly intrasellar in 4 cases

and extended to the suprasellar region in one case.

 Of the five MR scans reviewed, the lesions were of low signal

intensity in T1-weighted images, there was no enhancement in
post-gadolinium sequences .They were recorded to be of high
intensity T2-weighted images.
 Coronal
T2 MR
Sagittal T1 MR Coronal T1
image
image enhanced MR
image
 Coronal T1 MR image Coronal T2 MR image

Sagittal post contrast T1- Coronal postcontrast T1

weighted MR image
MR image
EPIDEMIOLOGY:
◦ Rathke’s cleft cyst (RCC) was first incidentally reported by
Lushka in 1860 as ‘an epithelial area in the capsule of the
human hypophysis resembling oral mucosa’.

◦ The first symptomatic RCC case was described by

Goldzieher in 1913.
◦ RCCs have been referred to by a variety of names
including pituitary cyst, mucoid epithelial cyst, intrasellar
epithelial cyst, Rathke’s pouch cyst, and colloid cyst of the
pituitary.

◦ Not until 1934 did Frazier and Alpers propose its

contemporary name of tumor of Rathke’s cleft.
PATHOPHYSIOLOGY:
◦ As Voelker and colleagues have stated, the most common
theory about the origin of RCCs is that the cysts are
derived from true remnants of the embryologic Rathke
pouch.

◦ On or about the 24th day of embryonic life, the Rathke

pouch arises as a dorsal diverticulum from the stomodeum;
it is lined with epithelial cells of ectodermal origin.
 At approximately the same time, the infundibulum forms
as a downgrowth of the neuroepithelium from the
diencephalon.

 By the fifth week, the Rathke pouch comes into contact

with the infundibulum, and the neck of the pouch
becomes occluded at the buccopharyngeal junction.

 During the sixth week, the Rathke pouch separates from

the oral epithelium. Subsequently, the pars distalis of the
pituitary gland develops from the anterior wall of the
pouch.
 The posterior wall does not proliferate and remains as the poorly defined pars
intermedia.

 The residual lumen of the pouch is reduced to a narrow Rathke cleft, which
generally regresses. The persistence and enlargement of this cleft is considered to
be the cause of the RCC.

 Other authors have different theories regarding the formation of RCCs, suggesting
instead that the cells of origin are derived from the neuroepithelium or the
endoderm, or that they come from metaplastic anterior pituitary cells.
 ANATOMO- PATHOLOGIC
FINDINGS
◦ Rathke cleft cysts are smoothly marginated cysts that vary in
size from a few millimeters to 1–2 cm.

◦ The contents vary from clear CSF-like fluid to thick mucoid

material.

◦ Microscopically, they are similar to other endodermal cysts

(neurenteric and colloid). They are lined by pseudostratified
or single-layered columnar or cuboidal epithelium.
◦ Cilia and scattered mucin-secreting goblet cells are
common. Many cysts have squamous differentiation, and
cornified squamous pearls are occasionally identified.

◦ The intracystic nodule consists of mucinous material at

histologic examination. Biochemical analysis of this
material is consistent with cholesterol and protein.
◦ Forty percent are completely intrasellar, while 60% have
some suprasellar extension through the cleft of the
diaphragma sella .

◦ Completely suprasellar cysts are rare .

 PRESENTATION:
 RCCs often produce no symptoms and so are usually
discovered incidentally, when radiographic or necropsy
findings are reviewed.

 Symptomatic RCCs are uncommon, but cysts can enlarge

and cause symptoms secondary to compression of the
pituitary gland, pituitary stalk, optic chiasm, or
hypothalamus.

 Symptomatic RCCs vary in presentation: headache, visual

and/or endocrine disturbance.
PREFERRED EXAMINATION:

◦ MRI is the modality of choice in the detection of RCCs. It

is superior to CT scanning for evaluating RCC mass
extension.

◦ Sagittal and coronal MRI scans provide reliable

information concerning the relationship of the mass to the
optic nerves, optic chiasm, and hypothalamus.
◦ Coronal MRI is also helpful in the evaluation of the lateral
extension of the sellar cyst and its relationship to the
internal carotid arteries and cavernous sinuses. MRI also
has superior multiplanar capabilities and contrast
resolution compared with those of CT scanning.

◦ The advantage of CT scanning is that it is superior to MRI

in depicting small amounts of calcium.
◦ This advantage can be important, because the presence of
calcification tends to indicate an alternative diagnosis, such
as craniopharyngioma, although small calcifications are
observed in some cases of RCC. CT scanning is also
superior to MRI in the evaluation of associated bony
remodeling.
COMPUTED TOMOGRAPHY:

◦ Rathke cleft cysts (RCCs) frequently appear as well-

circumscribed, hypo-attenuating, cystic sellar masses that
may have suprasellar extension.As a result of the different
cystic contents, RCCs may appear iso-attenuating or
hyperattenuating relative to the brain parenchyma.

◦ RCCs usually have a thin wall that may enhance.

 Variability in CT scan contrast enhancement among
individual cysts may reflect squamous metaplasia in the
wall or a peripherally displaced rim of pituitary tissue.

 Extravasation of cystic contents may inflame nearby

structures, resulting in enhancement.

 Calcification characteristically is not depicted on CT

scans, although Shin and colleagues have described this
finding in a number of cases.Complex cysts may have
septations.

 Large cysts may cause bony remodeling.

 MR IMAGING:
◦ The best imaging clue is a non enhancing non calcified
intra- and/or suprasellar cyst with an intracystic nodule .
While this is the typical picture, the imaging characteristics
vary widely.

◦ Approximately half are hyperintense on T1- weighted

images, while half are hypointense. On T2-weighted
images, 70% are hyperintense and 30% are iso- or
hypointense.
 Although no characteristic MRI features have been
identified, many RCCs are in 1 of the following 2
groups:

• Rathke cleft cysts (RCCs) with low signal intensity on

T1-weighted images and high signal intensity on T2-
weighted images.

• RCCs with high signal intensity on T1-weighted images

and variable signal intensity on T2-weighted images.

The cystic contents of the first group resemble those of

cerebrospinal fluid (CSF). In the second group, an
increase in the signal on T1-weighted images has been
related to the high content of mucopolysaccharides, which is
believed to result from an increase in the number of mucin-
secreting cells in the cyst wall, as well as from an increase in
the activity of these cells.

Uncommon cases with high signal intensity on T1-weighted

images and low signal intensity on T2-weighted images have
been suggested to result from a combination of factors,
including the presence of mucopolysaccharides, chronic
hemorrhage, a high cholesterol content, and cellular debris
from the cyst wall.
◦ A small nonenhancing intracystic nodule is considered a
virtually pathognomonic sign of a Rathke cleft cyst. These
nodules show high signal intensity on T1-weighted images
and low signal intensity on T2-weighted images, and they
do not enhance.

◦ Rathke cleft cysts do not enhance after contrast material

administration, although an enhancing rim of displaced
compressed pituitary gland is present in approximately half
of the cases.
◦ The report's authors went on to conclude that, with regard
to RCCs, DWI-SSFSE with apparent diffusion co-efficient
(ADC) values provides objective information for
differentiation from other sellar cysts. DWI-SSFSE with
ADC values can also be employed in the differentiation of
RCCs from craniopharyngiomas and hemorrhagic pituitary
adenomas. All the RCCs are hypo-intense relative to the
normal brain parenchyma (restricted diffusion) .
DIFFERENTIAL DIAGNOSIS:
◦ The differential diagnosis for Rathke cleft cysts includes
craniopharyngioma, cystic pituitary adenoma, or other non
neoplastic cysts (arachnoid cysts or epidermoids) .

◦ Unlike Rathke cleft cysts, craniopharyngiomas typically

demonstrate calcification and approximately 90% have
nodular, globular, or rim enhancement.
◦ The presence of solid enhancing nodules in the cyst wall
also favors the diagnosis of craniopharyngioma.

◦ The rare noncalcified cystic nonenhancing

craniopharyngioma, a finding more common in adults than
in children, may be impossible to distinguish from Rathke
cleft cyst with imaging findings alone.
TREATMENT:
 The most common approach in the treatment of RCCs is transsphenoidal surgery,
in which the cyst is partially excised and drained.

 This method is effective and helps to preserve pituitary function.

 Radical excision can cause additional and unnecessary pituitary damage;

therefore, it is not the treatment of choice.

 In transsphenoidal surgery, the cyst is opened, a biopsy specimen is obtained

from the wall, and the cyst is drained into the sphenoid sinus.
◦ An interesting aspect of treatment is the decrease in the
size of the cyst after high-dose steroid therapy. Although
the pathophysiologic mechanism is not clear, the steroids
are assumed to have an effect on the secretion or
absorption of cystic fluid. This finding suggests that steroid
therapy may be useful in some patients with an RCC and
inflammatory changes. Further study in this area is needed
to gauge its effectiveness is the treatment of RCCs.
 CONCLUSION:
 The MRI is effecticient in the positive and especially
the differential diagnosis of these cysts, and to guide the
therapeutic decision.Once the diagnosis
is considered, a spaced clinical and MRI monitoring is
adopted in cases of asymptomatic cyst, while a surgical
treatment is proposed for the rare symptomatic cysts.