Diagnosis Of Rathke Cleft Cysts Research Articles

Vertical triband flag sign for differential diagnosis of Rathke's cleft cyst

BackgroundThe Rathke cleft cyst (RCC) is a type of cystic growth that is benign, circular, and well-defined with an incidence rate of 4 %. This study aims to identify a useful diagnostic imaging sign that can aid in the differentiation of RCC from other cystic lesions. MethodsWe retrospectively analyzed the records of 42 symptomatic RCC patients who were referred to our facility between 2016 and 2023. The data for the study were obtained from our electronic database. All magnetic resonance imaging (MRI) studies were performed using a 1.5-T superconducting magnetic scanner. All patients underwent endonasal transsphenoidal surgical resection. All MRIs were reviewed and evaluated by a neurosurgeon and a neuroradiologist. ResultsThere were 8 (19 %) males and 34 (81 %) females with a mean age of 37.2-years. Our study identified a distinct imaging characteristic in 38 of the cases, which we have named the “vertical triband flag sign”, due to the growth of the cyst developing a specific appearance. The flag sign was mostly observed only in the T1-images (71.5 %), while in four cases the sign was spotted only in T2-images, and in four cases it appeared in both T1 and T2. In 4 cases, the flag sign was not observed in which further investigations revealed that these cases were suprasellar or small sellar RCCs. The dot sign, which is a characteristic finding in RCCs was only observed in one of our cases. ConclusionEarly diagnosis of RCCs may be facilitated by utilizing the vertical triband flag sign.

347 Management of Rathke Cleft Cysts in Children: The Rady Children’s Hospital Approach

INTRODUCTION: Rathke cleft cysts (RCCs) are benign, epithelial-lined sellar lesions that arise from remnants of the craniopharyngeal duct. Due to their rarity in the pediatric population, data are limited regarding the natural history and management of growing or symptomatic RCCs. METHODS: We performed a retrospective study of RCC patients age ≤18 years treated between 2006-2022 at our institution. RESULTS: 567 patients with a diagnosis of pituitary mass or cyst were identified. Of these, 31 underwent surgery and had a histopathological diagnosis of RCC, 58% female and 42% male. Mean age was 13.2 +/- 4.2 years. Presenting symptoms included headache (58%), visual changes (35%), and endocrinopathies or growth delay (26%); 13% were identified incidentally and grew significantly on serial imaging. Six percent presented with intralesional hemorrhage. Surgical approach was transsphenoidal for 90% of patients and orbitozygomatic for 10%. Preoperative headaches resolved in 61% of patients after surgery. New pituitary axis deficits were seen in 9.7%. Only two complications occurred from a first-time surgery: one cerebrospinal fluid leak requiring lumbar drain placement, and one case of epistaxis requiring cauterization. No patients experienced new visual or neurological deficits. Patients were followed postoperatively with serial imaging. Mean follow-up was 62.9 +/- 58.4 months. Recurrence requiring reoperation occurred in 32% of patients (transsphenoidal n = 8, pterional n = 2). Five-year progression-free survival was 47.9%. Except for one patient with multiple neurological deficits from a concurrent tectal glioma, all patients had a modified Rankin Scale score of 0 or 1 (good outcome) at last follow-up. CONCLUSIONS: Due to their secretory epithelium, RCCs may demonstrate rapid growth and recurrence. Incidental RCCs can be managed conservatively, whereas symptomatic or growing lesions may be fenestrated or resected. Due to their dynamic and variable nature, careful patient selection and close postoperative surveillance are critical to optimizing patient outcomes.

Cyst Type Differentiates Rathke Cleft Cysts From Cystic Pituitary Adenomas.

PurposeA classification system for cystic sellar lesions does not exist. We propose a novel classification scheme for these lesions based on the heterogeneity of the cyst wall/contents and the presence of a solid component on imaging.MethodsWe retrospectively reviewed 205 patients’ medical records (2008–2020) who underwent primary surgery for a cystic sellar lesion. Cysts were classified a priori into 1 of 4 cyst types based on the heterogeneity of the cyst wall/contents and the presence of a solid component imaging. There was high interrater reliability. Univariable and multivariable models were used to estimate the ability of cyst type to predict the two most common diagnoses: Rathke cleft cyst (RCC) and cystic pituitary adenoma.ResultsThe frequencies of RCC and cystic pituitary adenoma in our cohort were 45.4% and 36.4%, respectively. Non-neoplastic lesions (e.g., arachnoid cysts and RCC) were more likely to be Type 1 or 2, whereas cystic neoplasms (e.g., pituitary adenomas and craniopharyngiomas) were more likely to be Type 3 or 4 (p<0.0001). Higher cyst types, compared to Type 1, had higher odds of being cystic pituitary adenomas compared to RCCs (OR: 23.7, p=0.033, and 342.6, p <0.0001, for Types 2 and 4, respectively). Lesions with a fluid-fluid level on preoperative MRI also had higher odds of being pituitary adenomas (OR: 12.7; p=0.023). Cystic pituitary adenomas were more common in patients with obesity (OR: 5.0, p=0.003) or symptomatic hyperprolactinemia (OR: 11.5; p<0.001, respectively). The multivariable model had a positive predictive value of 82.2% and negative predictive value of 86.4%.ConclusionWhen applied to the diagnosis of RCC versus cystic pituitary adenoma, higher cystic lesion types (Type 2 & 4), presence of fluid-fluid level, symptomatic hyperprolactinemia, and obesity were predictors of cystic pituitary adenoma. Further validation is needed, but this classification scheme may prove to be a useful tool for the management of patients with common sellar pathology.

A Surgical Perspective on the Association between Cystic Lesions of the Pineal Gland (Descartes' Seat of the Soul) and the Pituitary (the Master Gland).

Introduction Rene Descartes (1596-1650), the famous philosopher and scientist, identified the pineal gland as the only cerebral structure not represented bilaterally, the "seat of the soul"; and the source of rational thought. Pineal cysts (PCs) are often incidentally identified in MRI studies, with a reported prevalence of 1 to 4.3%. Rathke cleft cysts (RCCs) are pituitary lesions accounting for <1% of intracranial masses. There are scant data in the literature addressing any association between these two midline cystic lesions. Methods We reviewed the medical records of patients presenting at our institution from April 2008 through February 2020, whose records indicated a diagnosis of RCC, and those whose records included pineal lesions. Our objective was to evaluate the association between these two midline lesions. Brain MRI studies were reviewed for the presence of PCs; only patients with PCs that measured ≥5 mm in diameter were included. Results We identified 116 patients with RCCs, and 34 patients with PCs, treated from April 2008 through February 2020. Among the RCC group, 14/116 patients (12%) had PCs. Among the PC group, 3/34 patients (8.8%) had RCCs. Overall, 17 patients (11.3%) had concomitant RCCs and PCs. The mean maximal diameter of the PCs was 7.5 mm (range = 5-17 mm), whereas the mean maximal diameter of RCCs was 13 mm (range = 5-40 mm). Conclusion The incidental diagnosis of cystic lesions of the pineal and pituitary gland is increasingly reported, primarily because of advances in current diagnostic modalities. Our data demonstrated no clear consensual association between pineal and pituitary cysts.

Rathke's cleft cysts: a 6-year experience of surgery vs. observation with comparative volumetric analysis.

Rathke's cleft cysts (RCCs) are common sellar lesions. Their management remains controversial, particularly when small or asymptomatic. Herein we review a consecutive series of RCC patients managed with surgery or observation. All patients with a new diagnosis of presumed RCC, based on MRI, from February 2012-March 2018 were retrospectively divided into observational and surgical cohorts based on an intent-to-treat model. The cohorts were compared for clinical presentation, and cyst volume. The observational cohort was followed for change in cyst size. The surgical cohort was followed for changes in endocrinopathy, visual symptoms, headache and recurrence. Of 90 patients (mean age 36.7 ± 19.4years; 68% female), 60% (n = 54) were in the observational cohort and 40% (n = 36) in the surgical cohort. Average follow-up was 13 ± 23months in the observational cohort and 24 ± 19months in the surgical group. In comparing the cohorts, mean ages were similar with more women in the surgical group (81% vs. 56%, p = 0.04). Most patients in the observational cohort had incidentally-discovered RCCs (n = 50, 88%) as opposed to the surgical cohort (n = 6, 17%). The surgical cohort had higher rates of headache (89% vs 26%, p < 0.001), endocrinopathy (36% vs 0%, p < 0.001), and visual dysfunction (19% vs 0%, p = 0.001). Mean cyst volume and maximal cyst dimensions were greater in the surgical cohort (0.94 ± 0.77cm3 and 14.2 ± 4.1mm), compared to the observational cohort (0.1 ± 0.14cm3 and 6.4 ± 3mm), (p < 0.001). Among the 53% (n = 30/54) of patients in the observational group with follow-up, 3 (10%) had spontaneous RCC shrinkage, 1 (3%) had modest asymptomatic growth (at 10months from initial MRI), and 87% had stable cyst size. Of the 36 patients recommended to have surgery, 89% (n = 32) did so. Post-operatively, complete or partial resolution of headache, endocrinopathy and visual dysfunction were documented in 90% (n = 28/30), 75% (n = 10/12), and 100% (n = 7/7), respectively. On follow-up MRI, 8 (22%) patients had some cyst reaccumulation, of whom 3 (8%) were symptomatic and underwent uneventful reoperation. No major complications such as hematoma, CSF leak, new endocrinopathy or visual deficits occurred. From this consecutive series, a majority (60%) of RCCs do not appear to warrant surgical intervention and have a low risk of cyst progression. However, surgical cyst removal appears to be indicated and safe for patients with larger, symptomatic RCCs. Simple cyst drainage has a high rate of improvement in pituitary gland function, visual function and headache resolution with low complication rates and symptomatic recurrence risk. These findings stress the importance of careful case selection and potential utility of volumetric assessment for patients with RCCs.

Entirely Suprasellar Rathke Cleft Cysts: Clinical Features and Surgical Efficacy of Endoscopic Endonasal Transtuberculum Sellae Approach

Rathke cleft cysts (RCCs) arise from the remnant of Rathke pouch and are usually found in the intermediate lobe. In most cases, RCCs remain intrasellar and extend into the suprasellar region. Rarely, they emerge entirely into the suprasellar cistern-entirely suprasellar Rathke cleft cyst (ESSRCC). We retrospectively reviewed medical records of 7 patients (5.5%) with ESSRCC among 128 patients with a diagnosis of RCC from January 1994 to September 2018. Of RCC cases, 70 were treated surgically. Patient age and sex, symptoms, magnetic resonance imaging findings, cyst diameter, presence of preoperative or postoperative hypopituitarism, operative procedures and complications, and functional outcomes at the final follow-up were investigated. All patients with ESSRCC were women with mean age 45.7 years (range, 29-69 years) at diagnosis. All patients experienced headache, mainly in the retro-orbital region. Mean ESSRCC diameter was 9.9 mm (range, 6-14 mm). Most cases exhibited hyperintensity on both T1-weighted and T2-weighted imaging. The endoscopic endonasal transtuberculum sellae approach relieved headache effectively and safely in 5 patients. Postoperative endocrinologic functions were almost entirely preserved. No patient experienced recurrence during the follow-up period. RCCs should be considered in the differential diagnosis of suprasellar cysts. The endoscopic endonasal transtuberculum sellae approach has an advantage in visualizing these lesions owing to the direction of light from the endoscope. Headache could be treated effectively with the endoscopic endonasal transtuberculum sellae approach without recurrence of cysts or pituitary dysfunction.

Symptomatic Rathke cleft cyst in paediatric patients - clinical presentations, surgical treatment and postoperative outcomes - an analysis of 38 cases.

Rathke cleft cysts (RCC) are benign, epithelium-lined intrasellar and/or suprasellar cysts believed to originate from the remnants of the Rathke pouch. The aim of this study was to analyse the symptoms and surgical outcome of patients with the diagnosis of RCC verified in a histopathological examination of the postoperative material. The study is a retrospective analysis of 38 cases of children who underwent a neurosurgical treatment due to RCC at the Children's Memorial Health Institute in Warsaw, Poland, between 1994 and 2015. At diagnosis, the mean age was 13 years and 8 months (6 years and 11 months-17 years and 10 months, sex ratio was 1:0.9 with a female prevalence). The most common symptoms were the following: headache (50%), hypothyroidism (50%), short stature and/or decreased growth velocity (47%), delayed puberty and menstrual abnormalities (37%), diabetes insipidus or polydipsia and polyuria (26%), adrenal dysfunction (26%), sleepiness and general weakness (13%) and visual disturbances (11%). Due to the gravity of symptoms and size of the lesion, all the patients underwent a surgical treatment. All but one were successful (one patient died due to postoperative neurosurgical complications). The most common postoperative complications were the following: adenohypopituitarism (67%) and diabetes insipidus (45%). RCC can present with serious symptoms that significantly deteriorate patients' quality of life. Despite a successful neurosurgical treatment in most of the analysed cases, patients required long-term pharmacological treatment.

Surgical Management of Rathke Cleft Cysts.

The diagnosis of Rathke cleft cysts (RCC) has increased in recent times as a result of improvements in imaging techniques; however, symptomatic patients are uncommon and accurate preoperative diagnosis may sometimes be difficult. The indications of surgical management protocol are evolving. We aim to provide a comprehensive review of clinical, imaging, and histopathologic features with operative management strategies along with outcome and prognosis in RCC. A retrospective analysis (2003-2015) was performed of 58 consecutive cases of RCC seen in a surgical unit. Twenty-seven surgically treated symptomatic RCCs were further evaluated for their clinical presentation, imaging characteristics, surgical approaches, and intraoperative findings. Headache was the most common presenting complaint followed by visual deficit. Hormonal abnormality was observed in 13 patients. On magnetic resonance imaging, the characteristic intracystic nodule was identified in 6 patients. Transsphenoidal surgery for cyst excision was performed in all 27 patients with an endoscopic route in 25 patients and radical excision was performed in 17 patients. The pituitary stalk and the normal gland were preserved in all patients. Headache improved in 96% of patients and visual field defect resolved in all. Around 46% had improvement of the anterior pituitary axis. New permanent hormone deficiency was not observed. The recurrence rate was 3.7% after a minimum of 18 months follow-up. RCCs are an uncommon disease with a wide spectrum of clinical and radiologic features. Endonasal endoscopic transsphenoidal surgery provides excellent clinical and endocrinologic improvement. We believe that radical excision does not necessarily result in endocrinologic impairment and may have a better impact on recurrence and cyst resolution.

Diagnosis of concomitant pituitary adenoma and Rathke's cleft cyst with magnetic resonance imaging

IntroductionThe diagnosis of concomitant pituitary adenoma (PA) and Rathke's cleft cyst (RCC) is difficult because PA and RCC cause similar symptoms. This study aimed to investigate magnetic resonance imaging (MRI) characteristics and surgical management of sellar lesions for concomitant PA and RCC. MethodsA retrospective study was performed in 18 patients with concomitant PA and RCC who visited our hospital between June 2008 and May 2014. MRI features, surgical management, histopathological diagnosis and prognosis of concomitant PA and RCC were summarized. ResultsOf the 18 patients, 3 cases were diagnosed with concomitant PA and RCC and 2 with concomitant PA and intermedia cyst by preoperative MRI. The remaining 8 cases were misdiagnosed as cystic formation of PA, 3 cases were bleeding of PA, and 2 cases were RCC. Surgery via the trans-sphenoidal route was performed in 17 cases and trans-frontal approach was chosen for one patient. All PAs resected were diagnosed by histological examination and the diagnosis of concomitant PA and RCC was histologically confirmed. ConclusionA non-enhancing cyst-like structure within the pars intermedia of PA usually located in the midline is a prominent MRI feature of coexisted PA and RCC. Total resection of coexisted RCC must be achieved and fat graft should be avoided during surgery because of high recurrence rate of RCC and complicated hypophysitis.

A Rathke's Cleft Cyst Presenting with Apoplexy

The occurrence of symptomatic pituitary hemorrhage into a Rathke's cleft cyst (RCC) is extremely rare. The author reports an interesting case of intra- and suprasellar RCC presented with features of pituitary apoplexy. This 62-year-old woman suffered acute headache, mental confusion, and partial hypopituitarism. The characteristics of the magnetic resonance imaging seemed most compatible with a hemorrhagic pituitary adenoma. Transsphenoidal drainage of the cyst contents confirmed the diagnosis of hemorrhagic RCC and resolved the symptoms. All published data on this rare clinical entity are extracted and reviewed.

Rathke cleft cyst presenting with hyponatremia: an unusual presentation

The authors report a case of Rathke cleft cyst presenting with severe hyponatremia. A 33-year-old man suffered sudden severe headaches, visual changes, dizziness, nausea, vomiting, and a metallic taste in his mouth. Initial laboratory values demonstrated severe hyponatremia. Magnetic resonance imaging revealed a cystic lesion with questionable intracystic hemorrhage, concerning for pituitary apoplexy. Transsphenoidal decompression and drainage of the cyst confirmed the diagnosis of Rathke cleft cyst and resolved the symptoms. Postoperative follow-up studies at 6 months demonstrated normal endocrine function and no evidence of a cyst.

Pathological laughter in a patient with Rathke cleft cyst

Rathke cleft cysts (RCCs) arise from embryonic remnants of Rathke’s pouch, and usually manifest as headaches, visual impairments and endocrine disturbances. We report a rare case of suprasellar RCC with the main symptom of pathological laughter. A 14-year-old girl presented with involuntary laughter, ataxia in the right arm and slightly impaired speech articulation. MRI revealed a huge suprasellar cyst causing severe compression of the midbrain and pons. The cyst was removed via a sphenoid ridge keyhole approach. Histologic examination resulted in a diagnosis of RCC. The spontaneous laughing spells disappeared immediately after surgery. In this case, compression of the upper brainstem might have been the cause of the pathological laughter.

Rathke cleft cyst of the sphenoid bone and nasopharynx

1095 Manuscript received May 31, 2004; accepted in final form August 30, 2005. Address reprint requests to: Karim Ben Hamouda, M.D., Service de Neurochirurgie, Institut National de Neurologie, La Rabta, 1007, Tunis, Tunisia. email: kbenhamouda@lycos.com. We report an exceptional case of a sphenoidal and nasopharyngeal Rathke cleft cyst (RCC). In a literature review, we found that this cyst had never been described in this region. This 41-year-old man presented in August 2002 with progressive headaches, ptosis, and diplopia. In November 2002, he experienced a spontaneous episode of rhinorrhea and a complete subsequent resolution of the symptoms. In February 2003, the frontal headaches relapsed, associated this time with a progressive decrease in left-sided visual acuity. On clinical examination in April 2003, the patient had an isolated loss of visual acuity (1/10) in the left eye. Endocrinological assessment demonstrated normal results. Computerized tomography scanning revealed that the sphenoid region had been destroyed by a large noncalcified low-density cystic lesion. Magnetic resonance (MR) imaging demonstrated a cystic sphenoidal extradural lesion that was isointense on T1-weighted sequences and peripheral enhancement after Gd enhancement (Fig. 1 left); hyperintensity was observed on T2-weighted images (Fig. 1 right). The maximal diameter of the lesion was 4 cm. There was also a smaller contiguous cyst on the posterior wall of the nasopharynx. A transrhinoseptal approach was used. The wall of the sphenoid sinus was paper thin; a motor oil–like fluid escaped. The cyst widely communicated with the nasal fossa, and its wall was partially extirpated. The dura covering the sellar floor was unperforated. Pathological examination showed a columnar ciliated epithelium (Fig. 2) consistent with an RCC. The postoperative course was uneventful; all symptoms resolved and no neurological symptoms developed. Imaging performed 1 week after surgery revealed partial evacuation of the cyst, with reappearance of the pituitary gland and a morphologically normal pituitary stalk. We also observed the persistence of a nasopharyngeal cyst under the sphenoidal lesion. One year later imaging revealed the disappearance of the sphenoidal cyst and the persistence of the lesion located on the posterior wall of the nasopharynx (Fig. 3). The sphenoidal, infrasellar origin of this RCC is supported by the absence of a suprasellar extension and by its entirely extradural location. Remnants of the craniopharyngeal canal can originate in a spectrum of cystic lesions, from simple RCCs to complex craniopharyngiomas.1 According to Erdheim’s theory,2 a craniopharyngioma can arise from every part of the craniopharyngeal canal. This may be also true for an RCC. In our case, it seems that the cyst derived from the canal enclosed in the cranial base bones, in contact with the walls of the pharynx. Computerized tomography and MR imaging features of RCC are not specific and are seen in more common lesions in this area such as craniopharyngiomas and mucoceles. The preoperative diagnosis of RCC is theoretically important because it may limit the operative management to biopsy sampling of the cyst wall and drainage of the contents via the transsphenoidal route.1 Voelker and colleagues3 concluded in a review of 155 cases that the transsphenoidal approach is optimal choice for sellar and suprasellar RCC and that partial excision and drainage of these cysts are the recommended treatments. In this case the sphenoid cyst disappeared after a partial excision of its walls.

Rathke cleft cyst intracystic nodule: a characteristic magnetic resonance imaging finding

The fluid content of Rathke cleft cysts (RCCs) displays variable appearances on magnetic resonance (MR) images and can appear indistinguishable from other intrasellar or suprasellar cystic lesions. Intracystic nodules associated with individual RCCs have been noted, but to date their significance has not been fully explored. The authors retrospectively reviewed MR imaging studies obtained in patients harboring intrasellar or suprasellar lesions that were consistent with RCCs to identify the presence and imaging characteristics of intracystic nodules. An intracystic nodule was present in nine (45%) of 20 patients with an RCC. All intracystic nodules were clearly visible and displayed a characteristic low signal intensity on T2-weighted MR images. The nodule was only visualized on T1-weighted images in four cases, in which it exhibited a consistent high signal intensity similar to that of the cyst fluid. The nodules did not enhance following the intravenous administration of a contrast agent. Although it is difficult to differentiate RCCs from other sellar cystic lesions because of the variable signal intensities displayed on MR images, the intensity of the intracystic nodule seems consistent on T1- and T2-weighted images, and the nodule is always clearly visible on T2-weighted images. With a nonenhancing cystic lesion that does not cause significant symptoms in the patient, the identification of an intracystic nodule with a characteristic signal intensity will aid in the diagnosis of RCC and the selection of conservative management.

Differential expression of cytokeratins 8 and 20 distinguishes craniopharyngioma from rathke cleft cyst.

Craniopharyngiomas are epithelial neoplasms usually located in the sellar and suprasellar regions. Distinguishing craniopharyngioma from Rathke cleft cyst is sometimes difficult, and the distinction is clinically significant because Rathke cleft cysts have a better prognosis than craniopharyngiomas. We retrieved 10 cases with a primary diagnosis of craniopharyngioma and 5 cases with a diagnosis of Rathke cleft cyst for analysis. Five cases of normal pars intermedia of pituitary glands from autopsy served as controls. We evaluated the expression patterns of a broad range of low- to intermediate-molecular weight cytokeratins (CK7, CK8, CK10, CK17, CK18, CK19, and CK20) and high-molecular weight cytokeratins (K903: a combination of CK1, CK5, CK10, and CK14; and CK5/6) in these cases. Craniopharyngiomas had a cytokeratin expression pattern distinct from that of Rathke cleft cysts and pituitary gland pars intermedia: craniopharyngiomas did not express cytokeratins 8 and 20, whereas Rathke cleft cysts and pars intermedia of pituitary glands both expressed cytokeratins 8 and 20. The differential expression of cytokeratins distinguishes between craniopharyngioma and Rathke cleft cyst, and this difference could be useful for identifying craniopharyngioma in difficult cases in which only a small biopsy is available. The different cytokeratin profiles of craniopharyngioma and Rathke cleft cyst suggest that these lesions do not come from the same origin, or that they come from a different developmental stage of the pouch epithelium.

Rathke Cleft Cyst

We report the radiologic findings in 23 cases of Rathke cleft cyst (RCC) and correlate them with the histopathology. We reviewed the radiology and pathology of 23 cases of surgically treated RCC operated upon at our institution or referred in consultation. There appears to be a correlation between the MR and CT appearance of the cyst, the gross appearance of the cyst contents, and the histopathologic characteristics of the cyst lining. Some of the lesions demonstrated peripheral enhancement, which in two cases was clearly due to a peripherally displaced rim of pituitary tissue. The appearance of RCC with CT and MRI is variable, and radiologic diagnosis can be difficult. Imaging features such as a sellar epicenter, smooth contour, absence of calcification, absence of internal enhancement, and homogeneous attenuation or signal intensity within the lesion suggest the diagnosis of RCC. Rim enhancement does not correlate with the presence of squamous metaplasia, hemosiderin, or cholesterol within the cyst wall and is not consistently seen in cases with changes of mild, chronic inflammation. In some cases, rim enhancement is due to a peripherally displaced rim of pituitary tissue.