Rathke's Cleft Cyst Research Articles

Expanded Endonasal Endoscopic Approach for Suprasellar Rathke Cleft Cyst: Treatment Outcome and Surgical Nuances

Rathke cleft cysts (RCCs) are benign lesions originating from remnants of the Rathke pouch. RCCs have been classified into the intrasellar-based or purely suprasellar types. This research aims to study the optimal treatment nuances and evaluate the surgical outcomes of this distinct type of RCCs. This study retrospectively reviewed 36 patients with purely suprasellar RCCs who were treated by expanded endonasal endoscopic approach (EEA) from September 2018 to January 2021. The demographic data, presenting symptoms, endocrine status, imaging findings, operative details, and surgical outcomes were analyzed. According to the relationship with pituitary stalk, the suprasellar RCCs can be divided into 2 different growth patterns: intrastalk cyst (ISC, 52.8%) and extrastalk cyst (ESC, 47.2%). Preoperative endocrine dysfunctions occurred more in patients with ISC than ESC (P= 0.007), which are characterized by hypoadrenalism (P= 0.004) and hypogonadism (P= 0.008). Operation exerted different impacts on endocrine functions between ISC and ESC (P= 0.012). When identifying by single hypothalamic-pituitary axis, 4 patients with normal endocrine function developed a new hormonal deficit, 14 patients with preoperative endocrine dysfunctions benefited from our expanded EEA surgical treatment, and 3 of them completely recovered from the endocrinopathy after surgery. The postoperative endocrinopathy was positively associated with the numbers of preoperative abnormal endocrinal axes. Thirty-four (94.4%) patients had achieved gross total resection. Pituitary stalk was intactly preserved in 23 of 36 patients (63.9%), partly preserved in 10 patients, and not preserved in 3 patients. Purely suprasellar RCCs are a distinct type of RCCs, which originate from the pituitary stalk above the diaphragmatic sellae, and the treatment strategy via expanded EEA is a rational choice. Classification based on growth patterns would be beneficial to reduce endocrine disorders after surgery.

Rare Case of Misdiagnosed Rathke Pouch Cleft Leading to Severe Visual Loss

A rare case of symptomatic Rathke cleft cyst resulting in severe visual compromise in a young 45 yr/f who presented with gradual progressive painless diminution of vision in both eyes. Patient noticed diminution of vision in the Left eye an year ago and later on in the Right eye for which she consulted ophthalmologists. She was diagnosed as optic neuritis and underwent treatment for the same, but vision didn’t improve. At presentation,vision in Both eyes was same, counting fingers at 2 meters. Pupillary reactions were normal, however there was bilateral disc pallor and rest fundus was normal. On systemic evaluation patient gave history of irregular menstrual periods since 3-4 months. on further investigation her S. Prolactin levels were markedly raised(59.99ng/ml). Radiology showed well defined hyperintense suprasellar lesion. Patient was operated for right frontotemporal craniotomy with decompression of RCCs by a neurosurgeon. 2 months post surgery visual acuity in RE 4/60 and LE is counting finger close to face.

Incidence and risk factors of delayed postoperative hyponatremia after endoscopic endonasal surgery for Rathke's cleft cyst: A single-center study.

BackgroudDelayed postoperative hyponatremia (DPH) is common for sellar lesions. However, the true prevalence and associated factors of DPH after endoscopic endonasal surgery (EES) for Rathke’s cleft cyst (RCC) have not been studied in a large patient cohort.MethodsA retrospective analysis was conducted over 6 years at our institution, and patients with RCC treated by EES were enrolled according to our inclusion criteria. Patient demographics, clinical characteristics, images, and surgical procedures were documented. Serum sodium was routinely measured before surgery, on postoperative day 1, and every 2 days thereafter until discharge. For patients with DPH, electrolyte, hematocrit, serum protein levels, and plasma and urinary osmolality were daily measured to explore potential etiology.ResultsOf the 149 eligible patients, 25 (16.8%) developed DPH, which was similar to other sellar lesions, except craniopharyngioma, in the same period in our institution. Significant risk factors suggested by univariate analysis were cyst location, requirement of postoperative hydrocortisone therapy, postoperative meningitis, intraoperative cerebrospinal fluid (CSF) leakage, and subtotal resection (STR) of the cyst wall (all p < 0.05). In addition, other supplementary 11 cases of suprasellar RCC with different surgical strategies (aggressive resection) and relevant factors were enrolled into multivariate analysis. Suprasellar location [odds ratio (OR) 8.387, 95% confidence interval (CI) 1.014–69.365, p = 0.049], requirement of postoperative hydrocortisone therapy (OR 4.208, 95%CI 1.246–14.209, p = 0.021), and intraoperative CSF leakage (OR 6.631, 95%CI 1.728–25.440, p = 0.006) were found to be the independent predictors of DPH.ConclusionDPH is a common complication after EES for RCC. Suprasellar location, requirement of postoperative hydrocortisone therapy, and intraoperative CSF leakage are the most reliable risk factors. Cortisol deficiency and syndrome of inappropriate antidiuretic hormone (SIADH) are considered as the main etiologies of DPH in RCC. Conservative excision of the cyst wall may reduce DPH occurrence.

Pituitary adenoma-Gonadotroph type in a 47 years old female patient: A case report

Pituitary adenomas are tumors of the anterior pituitary. Most pituitary tumors are slow-growing and benign. They are classified based on size or cell of origin. Pituitary adenoma can be classified as microadenoma, macroadenoma, and giant tumors based on size.Pituitary adenomas are mostly found incidentally on imaging modalities obtained for other reasons. Mutations in known Oncogenes have little role in the pathogenesis of most sporadic pituitary adenomas. Case presentation : A 47 years old female patient presented with complain of diminished vision , severe headache and dizziness since 1 months.MRI was advised which showed an ill-defined low signal intensity lesion of approximate 3.4x3.2x3 cm noted in clivus, bilateral spenoid sinus and sella region. Histologically, near-signature appearance is that of elongated cells surrounding blood vessels in a pseudorosette-like ependymoma-mimicking pattern. cells with small round nuclei and there is no significant mitotic activity. Conclusion : Pitutary adenoma-gonadotroph type is benign and can cause symptoms from mass effect or by effecting the level of the hormones. Differetial diagnosis includes ependymoma, Pitutary blastoma, Pitutary nodular hyperplasia, dermoid cyst, low grade astrocytoma, meningioma, rathke cleft cyst.

Trans-eyebrow supraorbital keyhole approach for suprasellar and intra-suprasellar Rathke cleft cysts: the experience of 16 cases and a literature review

Background Purely suprasellar and some complex intra-suprasellar Rathke cleft cysts (RCCs) are commonly treated via extended endonasal endoscopic approach or traditional transcranial approach. The feasibility of the trans-eyebrow supraorbital keyhole approach (TSKA) for RCCs was evaluated in this retrospective study. Methods A cohort of 16 patients (11 females and 5 males) with RCC was surgically treated via TSKA between January 2013 and November 2021. The medical data and follow-up results were analyzed. Results Eight patients had a purely suprasellar RCC, and 8 patients had an intra-suprasellar RCC with most of pituitary gland occupying the sellar floor. The mean maximal size of the cysts was 15.4 (range 7–29) mm. Postoperatively, complete cyst drainage was achieved in 15 (93.7%) patients. Preoperative headache, visual dysfunction, and hypopituitarism improved in 12 (100.0%), 3 (75.0%) and 3 (75.0%) patients, respectively, and hyperprolactinemia normalized in all patients. Except 2 (12.5%) cases of transient diabetes insipidus (TDI), no other complications were observed. During the mean follow-up period of 41.0 (range 4–102) months, 1 (6.3%) radiological recurrence was found. Conclusions For the treatment of purely suprasellar and some intra-suprasellar RCCs with most of the pituitary occupying the sellar floor, the endoscopic TSKA has the advantage of the minimal invasiveness, excellent visualization of the intrasellar compartment, no additional damage to the underlying pituitary gland, and no risk of CSF leakage. TDI or DI is relative commonly found in treatment of these RCCs. During surgery, care should be taken to avoid damage to the surrounding structures, including the pituitary stalk.

Conservative management of complicated Rathke's cleft cyst mimicking pituitary apoplexy.

SummaryComplicated Rathke’s cleft cyst (RCC) is a rare occurrence of symptomatic bleeding or growth of a previously asymptomatic (and often undiagnosed) intrasellar cyst derived from remnants of Rathke’s pouch, situated on the midline between the adeno- and neurohypophysis. Symptoms may be identical to those of pituitary apoplexy: acute onset of headache, hypopituitarism, and neurological disturbances. Both syndromes may also exhibit a similar appearance of a large haemorrhagic sellar mass at initial radiological evaluation. We report on two patients who presented with headache and complete hypopituitarism. Based on the initial MRI, they were first diagnosed with pituitary apoplexy but managed conservatively with hormone therapy alone because of the absence of severe visual or neurological threat. Upon follow-up at 4 months, clinical evolution was good in both patients but their pituitary mass had not reduced in size and, after careful radiologic reviewing, was more indicative of a large midline complicated RCC. In conclusion, the diagnosis of complicated RCC is challenging because it can mimic pituitary apoplexy clinically, biologically, and radiologically. Clinicians should distinguish between the two entities using specific radiological signs or evolution of the mass at MRI if the patient does not undergo surgery. To our knowledge, we report conservative management of this rare condition for the first time, though it seems appropriate in the absence of neurological compromise or visual compression. Long-term follow-up is however mandatory.Learning pointsComplicated Rathke’s cleft cyst can mimic pituitary apoplexy, presenting with sudden onset of headache, hypopituitarism, and visual and neurological compromise in the most severe cases.At diagnosis, pituitary MRI may not be able to differentiate between the two entities, showing a large haemorrhagic mass inside the sella, with little or no normal pituitary tissue visible. Patients are often diagnosed with apoplexy at this stage and may undergo pituitary surgery.When surgery has not been performed initially in these patients, repeat imaging at 3–6 months is unchanged and does not show the expected involution usually seen after adenoma apoplexy.Conservative management with hormonal replacement seems a valid option in the absence of visual or neurological deficits that would require trans-sphenoidal surgery.

Central hypogonadism in a man with a Rathke's cleft cyst

Central hypogonadism in a man with a Rathke's cleft cyst

Transsphenoidal hypophysectomy as a treatment for Rathke's cleft cyst in a dog

AbstractThe objective of the study was to describe the clinical, imaging, surgical and histological findings in a dog with Rathke's cleft cyst of the pituitary gland. A 6‐year‐old, female, neutered Staffordshire bull terrier was presented with an acute onset of abnormal behaviour. Magnetic resonance imaging of the skull showed a pituitary mass of 12.9 mm (height) × 8.8 mm (width) × 10.2 mm (length) with a pituitary height/brain area value of 0.73 (reference &lt;0.31). Magnetic resonance imaging findings were suggestive of pituitary apoplexy or neoplasia. Transsphenoidal hypophysectomy was performed and a cystic mass was removed. Histopathology revealed a Rathke's cleft cyst lined by a layer of pseudo‐stratified ciliated columnar epithelial cells and mucin‐secreting goblet cells with remnant pituitary tissue with positive immunostaining against adrenocorticotropic hormone, alpha melanocyte and growth hormone in the periphery. Rathke's cleft cyst should be included in the differential diagnosis of pituitary masses in the dog, and transsphenoidal hypophysectomy is an effective treatment.

Ruptured rathke's cleft cyst (RCC) with irritation of optic apparatus and rapidly evolving panhypopituitarism

Ruptured rathke's cleft cyst (RCC) with irritation of optic apparatus and rapidly evolving panhypopituitarism

Two case: hemorrhagic rathke cleft cysts mimicking a hemorrhagic adenoma

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Two cases: hemorrhagic rathke cleft cysts mimicking a hemorrhagic adenoma

Two cases: hemorrhagic rathke cleft cysts mimicking a hemorrhagic adenoma

Abstract #1183775: A Picture is Worth a Thousand Words: A Case of SIADH in the Setting of Suspected Rathke’s Cleft Cyst Hemorrhage

Rathke’s cleft cysts (RCC) are benign epithelium-lined cysts in the pituitary gland that arise from the remnants of Rathke’s pouch. RCC rarely cause symptoms. However, there are case reports of RCC causing water and electrolyte disorders with associated pituitary dysfunction most often due to suprasellar compression effects. We present a case of syndrome of inappropriate antidiuretic hormone secretion (SIADH) in the setting of suspected RCC hemorrhage.

Pituitary MRI in Cushing's disease - an update.

Pituitary MRI is essential in the diagnosis of ACTH-dependent Cushing's syndrome, but its results are inconsistent. The demonstration of a sellar image compatible with the diagnosis of corticotropinoma varies from 40% to 90%, depending on the centre where the imaging is performed. In fact, the expertise of the neuroradiologist, use of a Tesla 3.0 MRI and choice of sequences are fundamental. The T2 and 3D gradient echo sequences after gadolinium injection are the most informative and today allow the detection of macro- and microadenomas in almost all cases. The diagnosis of numerous picoadenomas (<3-4 mm) is more challenging. The 2D and 3D spin echo or delayed T1 SE or FLAIR sequences after gadolinium can be used as a complement or to confirm a suspicious image. Characterization of corticotropinomas remains problematic. However, the correct assessment of so-called incidentalomas by recognizing artifacts, anatomical variants and frequent Rathke's cleft cysts eliminates around 90% of the incidentalomas that mimic pituitary adenomas, as repetitively reported in the literature. For the time being, there is reason to believe that hybrid imaging combining PET and MRI such as 11C-methionine PET coregistered with volumetric MRI will solve the diagnosis of corticotropinomas in the near future.

Studies on anti-rabphilin-3A antibodies in 15 consecutive patients presenting with central diabetes insipidus at a single referral center

Central diabetes insipidus (CDI) is a rare condition caused by various underlying diseases including inflammatory and autoimmune diseases, and neoplasms. Obtaining an accurate definitive diagnosis of the underlying cause of CDI is difficult. Recently, anti-rabphilin-3A antibodies were demonstrated to be a highly sensitive and specific marker of lymphocytic infundibuloneurohypophysitis (LINH). Here, we report a detailed case series, and evaluated the significance of anti-rabphilin-3A antibodies in differentiating the etiologies of CDI. A prospective analysis was conducted in 15 consecutive patients with CDI from 2013 to 2020 at a single referral center. Anti-rabphilin-3A antibodies were measured and the relationship between antibody positivity and the clinical/histopathological diagnoses was evaluated. Among 15 CDI patients, the positive anti-rabphilin-3A antibodies were found in 4 of 5 LINH cases, 3 of 4 lymphocytic panhypophysitis (LPH) cases, one of 2 sarcoidosis cases, and one intracranial germinoma case, respectively. Two Rathke cleft cyst cases and one craniopharyngioma case were negative. This is the first report of anti-rabphilin-3A antibodies positivity in CDI patients with biopsy-proven LPH. Measurement of anti-rabphilin-3A antibodies may be valuable for differentiating CDI etiologies.

Enterogenous Cyst and Glioblastoma: A Brief Histopathological Review of Two Uncommon Cystic Lesions of the Central Nervous System

AbstractIntracranial cystic lesions are common findings in cerebral imaging and might represent a broad spectrum of conditions. These entities can be divided into nonneoplastic lesions, comprising Rathke cleft cyst, arachnoid cyst, and colloid cyst, as well as neoplastic lesions, including benign and malignant components of neoplasms such as pilocytic astrocytoma, hemangioblastoma, and ganglioglioma. Surgical resection and histological evaluation are currently the most effective methods to classify cysts of the central nervous system. The authors report two uncommon cases presenting as cystic lesions of the encephalic parenchyma—a enterogenous cyst and a glioblastoma—and discuss typical histological findings and differential diagnosis.

Rapidly recurrent recalcitrant Rathke Cleft Cyst: Case report and review of the literature

BackgroundRathke Cleft Cysts (RCC) are cystic sellar or suprasellar nonneoplastic lesions that are thought to originate from remnants of the rathke pouch. Postoperatively, RCCs have a tendency to reaccumulate, after which preoperative symptoms may recur. However, there exists very little evidence on which treatments are most effective in these patients. Case-descriptionIn this report, we present a unique case of a symptomatic RCC that recurred four times after initial transsphenoidal surgery. Following each surgery, the patient had significant visual improvement with post-op imaging displaying decompression of the neural elements. However, RCC reaccumulated in strikingly rapid time intervals of 1.5 months, 0.5 months, 1.5 years, and 5 months after each respective prior surgery. Repeat interventions with transsphenoidal, pterional and supraorbital approaches were unsuccessful in providing a durable treatment response. The patient ultimately underwent radiotherapy after a final surgical marsupialization of the cyst and has since displayed stable imaging with improved vision. This patient represented a ‘perfect storm’ of factors that may contribute to cyst recurrence, including substantial visual field deficits, large cyst size, peripheral wall enhancements on MRI, an intraoperative CSF leak, use of a fat graft, subtotal resection of the portion of cyst wall that adhered to important suprasellar structures, squamous metaplasia noted in cyst wall, and suprasellar extension. ConclusionsWe demonstrate that different surgical approaches through repeat surgeries may not assist in prevention of further recurrence; instead, we propose that radiotherapy should be offered early in the treatment course of recurrent cases that have additional risk factors for further reoccurrence.

Intraoperative Alcoholization of the Pituitary Gland Does Not Reduce the Recurrence Rate of Growth Hormone Secreting Pituitary Adenomas.

Objective Although the role of intraoperative alcoholization of the pituitary gland has been examined for the management of malignant tumor metastases and Rathke's cleft cysts, no such studies have been conducted relating to growth hormone (GH) secreting pituitary tumors, despite the high rate of recurrence in this cohort of patients. Here, we sought to understand the impact of adjunctive intraoperative alcoholization of the pituitary gland on recurrence rates and perioperative complications associated with resection of GH-secreting tumors. Methods This is a single-institution retrospective cohort study analyzing recurrence rates and complications among patients with GH-secreting tumors who received intraoperative alcoholization of the pituitary gland postresection versus those that did not. Welch's t -tests and analysis of variance (ANOVA) analyses were employed to compare continuous variables between groups, whereas chi-squared tests for independence or Fisher's exact tests were used for comparing categorical variables. Results A total of 42 patients ( n = 22 no alcohol and n = 20 alcohol) were included in the final analysis. The overall recurrence rates did not significantly differ between the alcohol and no alcohol groups (35 and 22.7%, respectively; p = 0.59). The average time to recurrence in the alcohol and no alcohol groups was 22.9 and 39 months, respectively ( p = 0.63), with a mean follow-up of 41.2 and 53.5 months ( p = 0.34). Complications, including diabetes insipidus, were not significantly different between the alcohol and no alcohol groups (30.0 vs. 27.2%, p = 0.99). Conclusion Intraoperative alcoholization of the pituitary gland after resection of GH-secreting pituitary adenomas does not reduce recurrence rates or increase perioperative complications.

Analysis of the Clinical Characteristics and Pituitary Function of Patients in Central China With Rathke's Cleft Cysts.

ObjectiveA Rathke’s cleft cyst (RCC) is a common, benign, cystic disease that often leads to hypophyseal dysfunction or head symptoms. The relationship between RCCs and pituitary gland function is not clear. We therefore carried out a study to examine this relationship in greater detail.MethodsThe study was a retrospective, cohort design in patients diagnosed with a RCC between January 2019 to July 2021 at the First Affiliated Hospital of Zhengzhou University, China.ResultsA total of 221 patients were enrolled and then divided into study cohorts according to the diameter of the RCC, clinical manifestations, and surgical treatment received. The majority of patients were symptomatic (143/221), including 83 cases of dizziness and headache, 9 of vision loss and visual field defect, and 2 of diabetes insipidus. 52 cases had abnormal pituitary function, with 8 cases interestingly showing high adrenocorticotropic-hormone (ACTH) and cortisone levels, while 8 juvenile cases had developed central precocious puberty. Patients with larger RCCs were more likely to present with headaches and dizziness, with subjects who suffered from these symptoms having high ACTH and cortisone levels.ConclusionAlthough the size of a RCC is not an important factor influencing hypopituitary function, we consider that endocrine evaluation should be carried out in all patients with a RCC.

Long-Term Outcomes of Endoscopic Cyst Fenestration for Rathke Cleft Cyst

We sought to elucidate the long-term surgical outcomes and incidence of recurrence and reoperation of endoscopic endonasal cyst fenestration for Rathke cleft cyst (RCC). A retrospective review of the chart and operation record of RCC surgical cases between January 2008 and August 2021 at our institution was conducted. Patient characteristics, intraoperative findings, and postoperative follow-up outcomes were evaluated. A total of 27 patients were analyzed, with a median postoperative follow-up period of 52 months (range, 1-150 months). Preoperative symptoms were visual dysfunction (59%), headache (41%), and pituitary dysfunction (22%). Endoscopic cyst fenestration was performed in all patients. Ten (37%) patients had intraoperative cerebrospinal fluid leakage. Among them, the only patient in whom sellar floor reconstruction was not performed experienced a repair operation due to postoperative cerebrospinal fluid leakage. No patients experienced postoperative hypopituitarism. Preoperative headache, visual dysfunction, and pituitary hormone disorder improved in 73%, 75%, and 67% of patients, respectively. Although postoperative cyst regrowth was observed in 8 patients (30%), no patient experienced worsening or novel symptoms and none required reoperation. Patients with a symptomatic RCC can be effectively treated with endoscopic endonasal cyst fenestration. Reversal of the presenting symptoms resulted, including headache, visual dysfunction, and pituitary hormone dysfunction, in the majority of patients. In our series, appropriate reconstruction of the sellar floor reduced the risk of postoperative cerebrospinal fluid leakage without impacting cyst regrowth. This simple technique appears to effectively disrupt cyst progression in most cases, even after a relatively long-term follow-up period.

Surgical outcomes and quality of life in Rathke's cleft cysts undergoing endoscopic transsphenoidal resection: a multicentre study and systematic review of the literature.

To establish the effect of endoscopic endonasal surgery (EES) on quality-of-life (QoL) in symptomatic Rathke cleft cyst (RCC). Analysis of 38 patients with RCC treated by EES, with regular overall (ASBQ-35) and sinonasal-specific (SNOT-22) QoL assessment during the first postoperative year. A systematic literature review of large case series was performed with pooled analysis. In our series, mean age was 53.6years with a female predominance (73.7%). Larger cysts were seen in males (p < 0.01), those with hypogonadism (p = 0.04), and visual dysfunction (p = 0.04). Complete normalisation of vision was seen in 83.3%. Persistence of visual dysfunction postoperatively was associated with diabetes (p = 0.005), hypertension (p = 0.02), suprasellar only location (p = 0.001), and monocular field cut (p = 0.02). Surgery did not significantly effect hormonal function. Sinonasal QoL transiently worsened after surgery, resolving within 3weeks. A parallel transient worsening of overall QoL normalised by 6weeks, and remained at preoperative baseline thereafter. These results were comparable to the literature, where 76.4% demonstrated improvement of vision and 13.1% had recurrence after treatment. There was no significant difference in outcomes between EES and microscopic approaches. We report longitudinal QoL outcomes in RCC for the first time. Vision commonly improves with surgery, but endocrinopathy is likely to persist. Microvascular risk-factors may compromise visual improvement. Surgery causes a transient worsening of sinonasal symptoms that resolves within 3-6weeks, but patients may not experience significant improvement in QoL within the first postoperative year.