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Background In patients with symptomatic Rathke’s cleft cyst, transsphenoidal surgery is highly effective at preventing further visual loss and usually allows for some recovery of vision. However, cyst recurrence and the need for re-operation are well recognized. To this end, the aim of this study was to investigate patterns of recurrence and long-term outcomes and to use this information to develop an optimal follow-up strategy. Method A prospectively maintained database was searched over a 10-year period between 1 January 2008 and the 1 January 2018 to identify all adults that underwent transsphenoidal surgery with a new diagnosis of Rathke’s cleft cyst. A retrospective case note review was performed for each patient to extract data on their presentation, investigation, treatment, and outcome. Results In all, 61 eligible patients were identified. The median follow-up was 34 months (range 2–112 months). In the 22 patients with pre-operative visual loss, the outcomes at 6 months were as follows: normal vision (2/22; 9.1%), improved but not normal (7/22; 31.8%), stable (12/22; 54.5%), worse but not blind (1/22; 4.5%), and blind (0/22; 0%). The overall rate of regrowth and re-operation in our study was 19.7 and 11.5%, respectively. The only factor that was significantly associated with recurrence was the presence of residual cystic disease on the post-operative MRI (p Conclusions We propose a follow-up strategy that stratifies patients at “low risk” if there is no residual cyst, with increasing interval scans, or “high risk” if there is residual cyst, with annual visual assessment and scans.

Symptomatic Rathke’s cleft cyst is usually accompanied by a long history of headache, visual disturbance, and hypopituitarism; however, rare cases present with acute onset and the clinical features in such cases remain uncertain. We report herein the clinical features of Rathke’s cleft cyst with acute onset and discuss the clinical significance. In this study, we defined acute onset as the clinical course with clinical symptoms within a 7-day history. From among 35 cases of symptomatic Rathke’s cleft cyst that were pathologically diagnosed at Fukuoka University Hospital between 1990 and 2009, five cases presented with acute onset. The symptoms, endocrinological findings, MR image findings, and pathological findings of these cases were analyzed retrospectively. Mean age was 56.8 years. Initial symptoms included headache (n = 3), general malaise (n = 2), polyuria (n = 2), and fever (n = 1). MR imaging revealed an intrasellar cystic lesion with suprasellar extension in all cases and showed rim enhancement in three cases. All cases were treated by transsphenoidal surgery. Pathological findings included hemorrhage (n = 2), hypophysitis (n = 2), and abscess formation in the cyst (n = 1). Postoperatively, all symptoms, except for hypopituitarism, improved in all cases. Rathke’s cleft cysts sometimes present with acute onset, and the presentation is consistent with the features of pituitary apoplexy caused by pituitary adenoma. Although pituitary apoplexy due to hemorrhage, inflammation, or infection due to an underlying Rathke’s cleft cyst is difficult to diagnose pre-operatively, Rathke’s cleft cyst should be included in the differential diagnosis, and early surgical treatment is needed, as for pituitary apoplexy caused by pituitary adenoma.

Although pituitary adenomas and Rathke's cleft cysts have a shared ancestry, they rarely occur simultaneously. Only 32 reports involving a pituitary adenoma and a concomitant Rathke's cleft cyst were identified upon review of the literature. Most initial presenting complaints include hormonal symptoms, visual disturbances, and headache. Next to growth hormone, Prolactin was the most commonly hypersecreted pituitary hormone. Rathke's cleft cysts show variable position, size, and signal intensity on magnetic resonance imaging (MRI). Here, we report a patient with a growth hormone- secreting pituitary adenoma associated with a Rathke's cleft cyst. The mass contained two different signal intensities on MRI. The lesion was successfully removed assisted by intraoperative MRI, when the presence of both lesions was confirmed. When a non-enhancing cyst-like structure is demonstrated on imaging in a patient with a pituitary adenoma, the possibility of a coexisting Rathke's cleft cyst should be considered.

Rathke's cleft cysts are often difficult to differentiate from other sellar cystic lesions using conventional MR imaging. The purpose of this study was to investigate the usefulness of single-shot fast spin-echo (SSFSE) diffusion-weighted MR imaging (DWI) in the diagnosis of a Rathke's cleft cyst.We examined retrospectively 29 patients with a histologically verified sellar or suprasellar lesion containing a fluid component; 12 patients had a Rathke's cleft cyst, 6 patients a craniopharyngioma, 5 patients a haemorrhagic pituitary adenoma, and 6 patients a cystic pituitary adenoma). Several regions of interest (ROIs) for apparent diffusion coefficient (ADC) measurements were identified in the fluid components of the lesions. For comparison, ADC values were normalized using a relative ADC (rADC), which was determined by dividing the ADC values of lesions by those of normal white matter and expressing the quotient as a ratio.SSFSE provides diffusion-weighted images without significant susceptibility artifacts. DWI-SSFSE revealed Rathke's cleft cysts as hypointense relative to the normal brain parenchyma in all cases. The mean value of ADC for Rathke's cleft cysts was 2.12 x 10(-3) mm(2)/sec. Both the ADC and relative ADC of the Rathke's cleft cysts were significantly increased compared to those of the cystic components of craniopharyngiomas and haemorrhagic components of pituitary adenomas in the subacute phase (P0.05). There was not a statistically significant difference between Rathke's cleft cysts and cystic components of pituitary adenomas (P0.05).DWI-SSFSE with ADC values provides objective information in the differential diagnosis of Rathke's cleft cysts from other sellar cystic lesions. In addition, DWI-SSFSE with ADC values is useful for differentiating Rathke's cleft cysts from craniopharyngiomas and haemorrhagic pituitary adenomas.

We retrospectively analysed patients with histologically proven Rathke's cleft cyst (RCC) in relation to the clinical manifestations and MRI findings, in particular, of cyst size and intensity in order to obtain an insight into their growing mechanisms, clinical presentations and their management. Eleven patients with RCC were divided into two groups based on T1 weighted images(WI). The A group consisted of 4 patients with cyst of low intensity in T1 WI. The age averaged 64.5 years. Their initial complaints were visual field defects(VFD). Their complaints were rather insidious. The maximum cyst size averaged 27.8+/-2.4 mm. The B group consisted of 7 patients with cyst of iso- or high-intensity in T1 WI. Two patients in the B group showed mixture of low and high and iso- and high-intensity, suggesting the presence of bleeding at the onset of symptoms or growing mechanism of the cysts. In the B group the age averaged 39.9 years, being lower than that in the A group. (P=0.0140 with Mann-Whitney's U test) The 5 patients out of 7 showed headache of insidious type or acute onset and the 3 showed a fluctuation of the VFD. The average size was 21.7+/-3.5 mm and smaller than that of the A group. (P=0.0298 with Mann-Whitney's U test) Our study has shown that the cyst with iso-to high intensity on T1 WI may cause clinical symptoms with a smaller size than cysts of the low intensity. In the former cyst pattern the onset and growing mechanism may be related to bleeding. The patients with this pattern are more likely to have acute and/or fluctuation of clinical presentations. Knowing these various clinical manifestations based on MRI pattern will be of help in following and managing patients with RCC.

Rathke’s cleft cyst (RCC) with significant squamous and/or stratified epithelium including smooth transition from single cuboidal to squamous epithelium (tRCC) is rare and possibly represents an intermediate form to craniopharyngioma. Twelve patients with histologically confirmed tRCC were retrospectively investigated from a series of 167 cases of RCC and 96 cases of craniopharyngiomas. Clinical data were reviewed, and immunohistochemistry findings for cytokeratins and β-catenin were examined. All lesions were located in the sella turcica with marked extension to suprasellar cistern. Six of the 12 patients had suffered postoperative re-enlargement, and three of these six patients required more than two additional operations and irradiation. CAM5.2 was positive in the glandular epithelium in all tRCCs and focally positive in the squamous epithelium of all these tRCCs. 34βE12 was positive in the squamous epithelium in all tRCCs and focally positive in the glandular epithelium in all but one tRCC. The findings of cytokeratin expression of tRCCs were very similar to those of craniopharyngioma. β-Catenin showed nuclear translocation in five cases. All patients with nuclear translocation of β-catenin suffered postoperative re-enlargement. tRCC carries an extremely high risk of re-enlargement. Cytokeratin expression resembles that in craniopharyngioma, which might indicate a very close origin of these pathologies. Nuclear translocation of β-catenin may be related to the aggressive clinical course.

Rathke’s cleft cyst is known as an indolent disease, but has become intractable in a few cases. In this clinical investigation, the initial operative outcomes of Rathke’s cleft cyst and the mechanism of re-accumulation were investigated to identify the optimum surgical strategy for the second operation. We conducted a retrospective review of 155 patients with Rathke’s cleft cyst (58 males and 97 females, aged from 13 to 84 years) surgically treated between April 1996 and March 2010. The same initial operative strategy was adopted in all patients. Operative outcomes and prognostic factors were investigated. Re-accumulation occurred in 27 patients (17.4%), and re-operation was required in eight patients because of neurological deficits (5.2%). Three types of re-accumulating mechanism were identified. First, cysts with cerebrospinal fluid (CSF)-like intensity on magnetic resonance imaging had a higher risk of re-accumulation (logrank test, p

Three patients with histologically proved Rathke's cleft cysts (RCCs) were evaluated with magnetic resonance imaging (MRI) and computed tomographic (CT) scan. Thirteen cases reported in the previous literature were also reviewed to evaluate the MRI features of RCCs and to compare them with CT features. The RCCs had various patterns of signal intensities on T 1-weighted and T 2-weighted MRI. The cysts were well-circumscribed and mainly in the sella turcica extending to the suprasellar cistern with minimal mass effect. Ten of sixteen cases had homogeneous cysts, and six had heterogeneous cysts. The CT scans showed the cysts as low or isodensity, well-demarcated lesions in the sella, that did not enhance with a few exceptions in which a thin ring enhancement was seen. MRI is superior to CT in the evaluation of the RCC, and is particularly useful in surgical planning, although MRI has a limitation on the specific, analytic description of the cyst contents.

Dear Editor, Juvenile xanthogranuloma (JXG) is a histiocytic nonproliferative disease of the non-Langerhans cell group. It is usually characterized by solitary or multiple skin lesions. Extracutaneous manifestations of JXG without systemic involvement are uncommon, and isolated JXG involving the skull base is extremely rare [7, 8]. We report a 3-month old patient who presented with a large mass eroding the left temporoccipital bone. A three-month old boy was referred to our hospital for a left retroauricular tumor. MRI showed an intracranial extradural retromastoid lytic lesion that was isointense on T1 and T2, and showed a slight enhancement with contrast injection (Fig. 1a). The lesion destroyed the temporal bone and displaced the left cerebellar hemisphere, producing supratentorial ventricular dilatation. Neurological examination showed no other abnormalities. Given the solitary character of the tumor and its size and location, and destructive characteristics it was decided to perform resective surgery. A retrosigmoid approach was carried out and a subperiosteal tumour was found. The lesion was completely extradural. A complete macroscopic resection was made. Histologic examination of the surgical specimen showed evidence of bone and soft tissue. The tumour was composed of histiocytic mononuclear cells accompanied by multinucleated giant cells of osteoclast and Touton types. Multinuclear and mononuclear cells were positive for CD68 and negative for CD1a and S-100 protein. Histological and immunohistochemical findings confirmed the diagnosis of JXG. A control MRI performed within 24-h post-surgery showed no residual tumour or any contrast uptake (Fig. 1b). Based on the benign nature of the tumor and after complete macroscopic resection no further treatment was given and the patient was discharged. At most recent follow-up at 3 years after surgery, the patient remains asymptomatic and free of pathology to MRI. The first report documenting single, and occasionally multiple, cutaneous lesions occurring in infants and showing spontaneous involution was written by Adamson in 1905 [1]. In 1954 Helwig and Hackney proposed the name “juvenile xanthogranuloma” to define cutaneous lesions that arise from fibrohistiocytes [8]. Juvenile xanthogranulomas have been described as solitary or multiple swellings that are nodular or plaquiform and yellow to yellowish-brown in colour. Their size varies frommillimeters to a few centimeters in diameter. As the name implies, these lesions generally occurs in the first 4 years of life [7]. The most frequently involved organ is the skin, and these tumors aremore likely to present in the head and neck. However, there are reports that document juvenile xanthogranuloma cases involving extracutaneous sites like the eyes, heart, pericardium, liver, lungs, spleen, testes and oral cavity [7]. Also documented in the literature are skeletal JXG manifestations in the context of systemic cases, but solitary osseous lesions are rare [4, 5, 13]. Involvement of the brain and its coverings are also reported [9, 12]. More often they arise in the sellar region [2, 10, 11]. Xanthogranuloma of the sella should be included in the differential diagnosis of a pediatric tumor of the sellar region. Often, xanthogranuloma is difficult to differentiate from craniopharyngioma and Rathke s cleft cyst based only on the findings of MR imaging [11]. Histopathological examination of JXG shows wellcircumscribed nodules with dense infiltrates of histiocytes. Variable numbers of multinucleated giant cells and, in 85 % of cases, Touton giant cells are present [3]. Although the appearance of histiocytic disorders may be quite similar, their P. Puerta (*) : S. Candela :G. Garcia Fructuoso Department of Neurosurgery, Sant Joan de Deu Hospital, Paseo Sant Joan de Deu, 2, 08950, Esplugues de Llobregat, Barcelona, Spain e-mail: puertaroldan82@gmail.com

Two cases of entirely suprasellar cysts are reported. Total surgical removal was performed in both cases. Pathological and immunohistochemical profiles were consistent with neurenteric cysts, Rathke's cleft cysts or colloid cysts and was also in keeping with an endodermal origin. It is now admitted that these three kinds of cysts share similar histological and immunohistological features. We propose an hypothesis of common embryological origin from endodermal remnants.

A 71-year-old woman with a symptomatic Rathke's cleft cyst is reported. Our patient is unique in that both metrizamide computed tomographic cisternography and a transsphenoidal surgery were safe and successful procedures in spite of the advanced age of the patient.

A case of Empty Sella secondary to Rathke's cleft cyst, successfully operated on, is reported. Asymptomatic cysts of Rathke's pouch are fairly frequent autopsy observations. They can cause optic-chiasmatic and endocrine symptomatology or, very rarely, an Empty Sella syndrome if the cyst is very large.