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Rathke cleft cysts (RCCs) are benign sellar lesions originating from remnants of primitive ectoderm. They have not been previously linked to other cystic lesions, such as pineal cysts (PCs). Our objective was to perform a multicenter cross-sectional neuroimaging study to examine prevalence rates of coexisting RCC and PC.We retrospectively queried prospectively maintained, institutional review board-approved, databases from the authors' centers. All patients undergoing transsphenoidal surgery for RCC between the years of 2011 and 2020 were included for analysis. Preoperative magnetic resonance imaging was reviewed to identify the coexistence of a PC. Patient demographics and neuroimaging characteristics were recorded. A control cohort comprised of 100 age- and sex-matched patients with nonfunctional pituitary adenoma (NFPA) who also underwent surgical intervention was utilized.Eighty-four patients with RCC were identified for analysis. A coexistent PC was identified in 40.5% (n = 34) of patients with RCC compared with 14.3% (n = 12) in the NFPA cohort (P0.001). There was no significant difference in PC size between patients with RCC and PA (8 vs. 8.8 mm, respectively; P = 0.77). Although the majority (85.7%; n = 72) of the RCC cohort were female patients, there was no sex predominance with respect to coexisting PC in either the RCC or PA cohort.This is the first study to report an increased prevalence of coexisting PC and RCC, possibly because of an embryologic link or other propensity for intracranial cyst formation. Additional studies in more generalizable populations can further explore the relation between RCC and PC, or other cyst formation.

Background The direct endoscopic endonasal approach (EEA) has become the primary technique used for resection of sellar pathology, meriting investigation into the risk factors for complications and predictors of postoperative outcomes after direct EEA. Methods We retrospectively analyzed the patient and tumor characteristics from 404 patients who had undergone direct EEA for sellar pathology at the USC Pituitary Center from September 2011 to December 2019. Results Of the 404 pathologic entities included, 349 (86%) were pituitary adenomas (PAs), 29 (7%) were Rathke cleft cysts, and 26 (6%) were other sellar lesions. The mean lesion diameter was 2.3 cm, with 34 microadenomas (10%) and 315 macroadenomas (90%). Cavernous sinus invasion was present in 39% of the PAs. No patient died. The surgical complications included internal carotid artery injury without neurological sequelae (0.2%), vision loss (0.7%), meningitis (0.7%), cerebrospinal fluid leak (4%), epistaxis (4%), sinusitis (1%), transient cranial nerve paresis (0.5%), and postoperative abscess (0.25%). New hypopituitarism developed in 3%. Gross total resection was achieved in 208 PA cases (58%). Clinical improvement of headaches and visual deficits were reported for 67% and 76% of cases, respectively. Hormonal remission was achieved in 82% of patients with functional PAs. The median hospital stay was 2 days, with 34 patients (8%) readmitted within 30 days and 10 (3%) undergoing early reoperation. Disease recurrence or progression developed in 10% and was less likely in the case of gross total resection and apoplexy. Conclusion In the present, large, consecutive, mostly single-surgeon series, the patients experienced clinical improvement in most preoperative symptoms and had low rates of perioperative morbidity. We have demonstrated that direct EEA can be efficiently, safely, and successfully performed by a neurosurgical team.

Background Although most patients with Rathke's cleft cysts (RCCs) remain asymptomatic throughout their lives, visual impairment in RCCs usually indicates surgical interventions, including endoscopic drainage of the cyst. We report a case of RCC with unique findings in the optic nerve root (ONR) by magnetic resonance imaging (MRI). Case Description A 58-year-old man admitted to our hospital complained of progressive left visual impairment. Preoperative computed tomography MRI revealed a suprasellar cystic lesion that extended anteriorly, and the bilateral ONRs were lateralized with the cyst and were partially enhanced with gadolinium administration. The cyst content was drained, and the cyst wall was partially removed by endoscopic transsphenoidal surgery. Postoperatively, the patient was administered corticosteroid intravenously for 3 days, and his visual acuity improved dramatically. Postoperative MRI revealed that the volume of the cyst decreased, and the position of the bilateral ONRs normalized. Additionally, enhancement of the ONRs with gadolinium administration was not observed. Histological examination of the surgical specimen revealed a ciliated single-layer columnar epithelium with inflammatory cell infiltration. Conclusions To our knowledge, we report the first case of a patient with RCC with ONR enhancement with gadolinium administration on MRI. This unique finding might prove that inflammation is one of the causes of visual impairment in RCC as in optic neuritis.

Highlights • Rathke’s cleft cysts are benign lesions from the remnants of the craniopharyngeal duct. • Its diagnosis is often confused with craniopharyngioma. • Our case of this cyst demonstrates the importance of proper diagnosis and management., Introduction Rathke’s cleft cysts (RCCs) are benign cystic lesions from the remnant cells of the craniopharyngeal duct within Rathke’s pouch. Anticipation of such condition will help planning the treatment course. Presentation of case We present a case of a 46-year-old male, referred to our tertiary center with a history of seizures, confusion and agitation for the past two weeks. After thorough investigations, he was found to have hyponatremia with low serum cortisol and hypothyroidism. After being sedated, intubated and stabilized, Magnetic Resonance Imaging (MRI) brain showed relatively medium to large sellar lesion which was highly suspicious of a craniopharyngioma (CP). However, after performing an endoscopic transphenoidal surgical resection of the lesion, clinical diagnosis supported RCC. Discussion The best treatment approach for CP is a complete surgical resection via a transphenoidal approach. Considering the fluid component of RCC, it may be treated with a more conservative surgery and does not necessitate a surgery as invasive as that of CP. Conclusion Given the mixed solid and cystic nature of CPs, it is possible to confuse it with RCC and vice versa. The presented case highlights that Rathke’s cyst may be challenging in terms of pre-operative diagnosis, peri-surgical management and histopathological examination.

Purpose To determine the incidence of the ‘dot sign’ in patients with colloid cysts of the third ventricle and to characterise its MRI appearances. Materials and Methods Single institution retrospective analysis between January 2007 and October 2016 of all patients with either an imaging or imaging and histology-confirmed diagnosis of colloid cysts of the 3rd ventricle was undertaken. For all cases, MRI signal intensities of the cyst fluid component were graded by two independent radiologists relative to brain parenchyma. Presence of a dot, and if present, its size and relative position within the cyst were recorded. Signal intensities of the dot were then similarly assessed. Results 37 cases of colloid cyst were identified. Of these 37.8% (n = 14) demonstrated the dot sign. The majority (11 of 14) were observed inferiorly within the cyst; two cases were anteroinferior, and one other was posterior. All identified intracystic nodules displayed low signal intensity with respect to cyst fluid on T2 weighted sequences, and only two nodules were hypointense to fluid on T1-weighted imaging, with the remainder either iso- or hyperintense. Conclusions An intracystic low T2 ‘dot’ is a common MRI feature of colloid cysts of the third ventricle, and to our knowledge not previously systematically described. Presence of such a dot should not only not dissuade from a diagnosis of colloid cyst being made, but should in fact be used to strengthen the imaging diagnosis.

The ACR Incidental Findings Committee presents recommendations for managing pituitary findings that are incidentally detected on CT, MRI and 18F-fluorodeoxyglucose PET. The Pituitary Subcommittee, which included radiologists practicing neuroradiology and an endocrinologist, developed this algorithm. The recommendations draw from published evidence and expert opinion and were finalized by informal iterative consensus. Algorithm branches successively categorize pituitary findings on the basis of imaging features. They terminate with an ascertainment of an indolent lesion (with sufficient confidence to discontinue follow-up) or a management recommendation. The algorithm addresses most, but not all, pathologies and clinical scenarios. The goal is to improve the quality of care by providing guidance on how to manage incidentally detected pituitary findings.

Background Lymphocytic hypophysitis is a rare inflammatory lesion in the sellar region. Lymphocytic hypophysitis secondary to ruptured Rathke cleft cyst (RCC) is even more rarely seen. Clinical characteristics, treatment strategies, and prognosis remain elusive. Case Description A 58-year-old Chinese woman with a 3-year history of intermittent headache and new development of polydipsia, polyuria, and binocular visual acuity decline over the past 4 months was admitted to our hospital. On admission, endocrine results were normal. Ophthalmic testing showed bitemporal visual field deficits and decreased visual acuity of both eyes. Pituitary dynamic magnetic resonance imaging showed a cystic sellar lesion measuring 5.6 × 5.2 × 6.2 mm with tumor shrinkage compared with initial neuroimaging. Transsphenoidal surgery was performed for tumor resection. An additional biopsy of the anterior pituitary lobe was performed because of intraoperative abnormal appearances of the pituitary gland. Pathology examinations revealed the specimen of the cystic lesion to be RCC and exhibited infiltration of massive lymphocytes, diffused plasmocytes, and eosinophils that were diagnostic of lymphocytic hypophysitis in the anterior pituitary lobe. During follow-up, the lesion of lymphocytic hypophysitis showed spontaneous shrinkage in the absence of steroid treatment after RCC resection. Final diagnosis was lymphocytic hypophysitis secondary to ruptured RCC. Conclusions We present a rare case of lymphocytic hypophysitis secondary to ruptured RCC and provide clinical data of such lesions in the literature. Full understanding of the clinical features plays an important role in diagnosis and treatment of lymphocytic hypophysitis secondary to ruptured RCC.

Objectives It is well-known that Rathke’s cleft cysts (RCCs) infrequently cause headache, endocrinological dysfunction, and visual disturbance, and in rare cases, cause diabetes insipidus (DI). Although surgical evacuation of the cyst content can result in high rates of symptomatic improvement, not only the treatment efficacy but also the pathophysiology of DI with RCC are undetermined. The aim of this study is to elucidate the underlying mechanisms and outcomes of DI associated with RCCs. Patients and Methods We retrospectively studied 109 patients with RCCs treated at Kanazawa University Hospital between 2000 and 2016. Their age, sex, symptoms, endocrinological status, DI, visual disturbance, neuroradiological findings, pathological appearances, and pre-/post-operative hormone levels and status of anti-diuretic hormone replacements were assessed. Results Among 109 cases of RCCs, five cases (4.6%, 2 males and 3 females) manifested with DI as initial presentation were included. These five cases could be divided into two types: the acute type and the chronic type, based on the onset and duration of symptoms. Three acute onset cases presented with not only strong thirst but also sudden headaches without pituitary dysfunction, whereas the two chronic onset cases presented with chronic headaches and hypopituitarism. Pathological examination in the acute type revealed inflammatory cell infiltration into only the posterior lobe of the pituitary and disruption of the cyst wall adjacent to the posterior lobe, which might suggest RCC rupture. In contrast, the chronic type showed inflammatory cell infiltration into both the anterior and posterior lobes of the pituitary and thickened fibrosis beneath the cyst wall. Postoperatively, two cases of the acute type could be controlled with a smaller amount of 1-deamino-8-D-arginine vasopressin (DDAVP) than that required preoperatively, whereas no change was observed in the cases of the chronic type. Conclusion The cases of DI onset caused by RCCs could be divided into the acute type and the chronic type. In the chronic type, surgical treatment could not affect the status of DI. However, in acute type, urgent surgical intervention partially relieved DI.

Background Although pituitary adenoma (PA) and Rathke cleft cyst (RCC) share a common embryologic origin, concurrent appearances have rarely been reported. We present a case of intractable RCC hidden behind a coexisting giant PA. Case Description A 39-year-old woman presented to an ophthalmologist with visual disturbance. Brain magnetic resonance imaging findings showed a giant mass in the intrasellar and suprasellar portion, with a cystic portion contained in the posterior part. Endoscopic endonasal surgery was performed to remove the tumor, and histopathologic findings revealed a nonfunctioning PA. Further brain magnetic resonance imaging showed the appearance of a residual cyst. A second endonasal endoscopic surgery was performed to relieve compression of the optic chiasm by the growing cyst, followed by cyst drainage and irrigation, and portions of the cyst walls were obtained. The histopathologic diagnosis was RCC with inflammation. Over the course of 1 year, 3 separate recurrences of the growing cyst were noted, with cyst drainage and irrigation performed each time. Finally, ethanol cauterization was performed, which stopped the symptomatic RCC recurrence. Eight years after the final procedure, there was no cyst recurrence, and the patient's condition was good with improved visual acuity and normal pituitary function. Conclusions The presence of a nonenhanced cyst with a PA on magnetic resonance imaging suggests the possibility of coexisting RCC and PA.

The spontaneous involution and rapid recurrence of a Rathke’s cleft cyst (RCC) is not well described. A 16-year-old female with a putatively incidental nearly 2 cm suprasellar lesion arising from the posterior aspect of the infundibulum found on a brain MRI during workup for aseptic meningitis demonstrated a spontaneous and near-complete involution of this lesion on close follow up (leaving only a thickened infundibulum measuring up to 3.6 mm). After a period of cyclic headaches coinciding with subtle changes in pituitary stalk thickness, she developed progressive fatigue, severe headaches, and bitemporal blurry vision, with rapid re-growth of the suprasellar lesion to near its original size seen on repeat MRI. Due to its purely suprasellar and retro-infundibular location an anterolateral rather than an anterior transnasal approach was recommended. She underwent a successful orbitofrontal craniotomy and cyst fenestration/partial resection, with pathologic analysis confirming an RCC. Post-operatively, she had normal vision and no diabetes insipidus or other hormonal disturbances, and improvement of her headaches on ten-month follow up. Awareness of the potentially dynamic nature of RCCs is important for their timely diagnosis. Purely suprasellar RCCs can require an anterolateral surgical approach, especially when located posterior to the infundibulum.

The prevalence of pituitary disease is increasing mainly because of advances in modern imaging techniques and an increased awareness among the medical community. Pituitary tumours constitute 10–15% of all diagnosed intracranial neoplasms, and their clinical manifestations result from local mass effects (mostly neurological, visual, hypopituitarism) and/or hypersecretion. Pituitary adenomas are the most common pituitary tumours and are clinically classified as functioning or non-functioning. Most are sporadic, but in rare cases they can be related to hereditary syndromes. Other lesions involving the (para)sellar region include inflammatory and infiltrative diseases, cysts, primary or metastatic neoplasms, abscesses and internal carotid artery aneurysms. The clinical manifestations of hypopituitarism depend mainly on the type, number and severity of hormonal deficits. Establishing the diagnosis requires hormonal measurements (basal or after dynamic tests), and management includes relevant hormonal replacement and life-long monitoring.

Objective Repeated chronic inflammation, such as under circumstances of ruptured Rathke's cleft cyst (RCC), is known as the fundamental basis of various pathological changes including neoplastic change, therefore to examine accurate incidence of pituitary adenomas is important for the preventive medicine. Materials and methods As a prospective study, patients were initially screened by the symptoms such as periodic headache and dizziness together with the abnormal findings such as seller floor bone defect on 3D-CT. Those screened patients who showed abnormal basal pituitary hormone values were additionally examined by several loading test and 3 T magnetic resonance imaging (MRI) by three-dimensional reconstructions of Flair cube images. The presence of pituitary adenomas was evaluated by composite 3T MRI and 11 C methionine MET positron-emission tomography imaging. Immunohistochemical studies were employed for surgical biopsy specimens to demonstrate hormone production. Results 308 cases of RCC were diagnosed by both radiologic and intraoperative findings. Pathological examination confirmed 111 pituitary adenomas in 106 patients with ruptured RCC, therefore the rate of coincidence was revealed 34%. In 78 patients with abnormalities of pituitary hormone secretion, MET uptake was observed in the pituitary gland and verified pituitary adenoma pathologically. In addition, 28 adenomas were verified without MET-PET study on pathological examination only, for a total of 106 cases. Conclusions High coincidence (34%) of ruptured RCC and pituitary adenomas was demonstrated, which is a proof that ruptured RCC is contributed to a risk factor of pituitary adenoma.

We report a rare patient with an intrasphenoidal Rathke's cleft cyst (RCC) and review the literature. RCC are benign epithelium lined cysts containing mucoid material, which typically occur in a location that is either entirely intrasellar, or intrasellar with suprasellar extension (intra-suprasellar). RCC in a completely extrasellar location are uncommon. An intrasphenoidal RCC is extremely rare with only two patients reported in the literature to date. Preoperative diagnosis is difficult due to the uncommon location and absence of any characteristic radiological features. However, it remains of utmost clinical relevance because it may limit the operative management to biopsy sampling of the cyst wall and drainage of the contents via the transsphenoidal route.

Rathke’s cleft cysts (RCC) are benign lesions that originate from remnants of Rathke’s pouch. They can compress adjacent structures causing visual loss and endocrine dysfunction. The endoscopic endonasal transsphenoidal approach (EETA) has gained popularity in the surgical management of pituitary and parasellar tumors. However, postoperative cyst recurrence and endocrine dysfunction are still major concerns. A retrospective chart review was performed on 11 patients who underwent a purely EETA. Subtotal resection of the cyst wall with drainage of the intracystic contents followed by obliteration of the cyst with a fat graft was performed in all patients. Two patients underwent repeated surgeries for symptomatic cyst recurrence. One patient ultimately underwent extracapsular removal of the entire cyst wall because of multiple recurrences after simple drainage. There were no incidences of new permanent hypopituitarism, visual deficits, or postoperative cerebrospinal fluid leaks. All patients reported an improvement of initial preoperative symptoms. A non-aggressive strategy of partial cyst wall removal and simple drainage of cyst contents via EETA is a viable approach for surgical treatment of RCC with a low rate of postoperative endocrine and visual complications. A more aggressive strategy of extracapsular removal of the cyst wall may be indicated in patients with repeated recurrence.

Rathke's pouch cysts are uncommonly found on imaging and are usually incidental in nature. Herein, we report a most unusual case of Rathke's pouch cyst verified to have resolved and then 20 years later, to have returned. The germane literature regarding such a phenomenon is reviewed. We believe this to be the first report of a patient with an appearing, disappearing, and reappearing Rathke's cleft cyst. Keywords: Sella turcica, Pituitary gland, Skull base, Anomaly, Imaging

Rathke's cleft cysts (RCC) arise from the development of the Rathke's cleft pouch. These commonly occurring cysts are typically asymptomatic, but sometimes present with headaches, endocrine dysfunction, and visual loss. Recurrence is common after either drainage or surgical removal. The purpose of this study was to review published outcomes for RCC management, and determine whether specific factors, including patient demographics, cyst pathology, radiologic parameters, or surgical techniques predispose to their recurrence. A systematic review of studies for RCC from 1990 to 2012 was conducted. Patients were identified using a Medline/PubMed search, and from the bibliographies of relevant articles obtained from the primary search. Relevant studies reporting recurrence rate were identified, and data were extracted regarding patient demographics, presenting symptoms, cyst characteristics, surgical treatment, and outcomes. A meta-analysis for recurrence rates was also performed. Twenty-eight journal articles comprising a total of 1151 RCC revealed an average follow-up of 38 months (range 16-79 months). In the studies reviewed, there was a relatively equal distribution of treatment approaches, with 35% subtotal resection, 33% gross total resection, and 32% complete drainage with wall biopsy. The microsurgical transsphenoidal approach was found to have a higher recurrence rate (14% versus 8%) and new endocrine dysfunction rate (25% versus 10%) compared to the endoscopic approach. The data demonstrates a notable overall recurrence rate for RCC (12.5%). However, there appears to be no conclusive evidence that more aggressive resection of the cyst wall results in lower rates of recurrence.

AIM To evaluate clinical findings and magnetic resonance imaging (MRI) characteristics of Rathke's cleft cyst (RCC) in 22 patients. Materials and Methods Twenty-two patients were imaged using non-enhanced MRI and 17 underwent an additional contrast-enhanced MRI examination. Fifteen patients received an additional non-enhanced computed tomography (CT) examination, and amongst these, two underwent contrast-enhanced CT. Two radiologists read the images retrospectively. The imaging data were studied with regards to location, size, margin, signal intensity, enhancement characteristics, haemorrhage, and presence of calcifications. Clinical data, such as presenting signs and symptoms, physical findings, and medical histories, were collected. Histopathological studies were performed and analysed by two pathologists. Results Nine lesions were located in the intrasellar region, 12 in both the intra- and suprasellar regions and one in the suprasellar region. The maximum diameter of the RCCs varied from 0.7 to 4 cm, with an average size of 1.7 ± 0.7 cm. MRI features of RCC were divided into three groups based on T1-weighted imaging (T1WI): hypo- ( n = 6), iso- ( n = 9), and hyperintensity group ( n = 7). Patients in the latter two groups were statistically younger than that in the former group. The lesion size in the iso- and hyperintensity groups was significantly less than that in the hypointensity group (F = 6.421, p = 0.007). Only two cases showed enhancement after contrast injection in the cohort. One lesion with haemorrhage was found as were two cases with intracystic nodules. Conclusion Although MRI features of RCCs are variable, RCCs should be suspected when the following conditions occur: lesions located in the intrasellar region or involving both intra and suprasellar regions, less than 1.5 cm in diameter, iso- or hyperdense on T1WI and no signal enhancement after contrast injection. In addition, the first case of a RCC with a markedly enhanced intracystic nodule is reported.

Rathke's cleft cyst (RCC) is a rare lesion of the pituitary gland that is often asymptomatic. The objective of this study was to further characterise the presenting symptoms, endocrinopathy and potential for recurrence of this lesion in an Australian population. The files of 12 patients with pathologically confirmed RCCs were reviewed retrospectively. Common presentations included headache, endocrinopathy, and visual disturbance. Uncommon presentations included 1 patient with pituitary apoplexy. The cysts varied in size from 6 mm to 20 mm; 8 had a suprasellar component. 1 patient had evidence of a RCC with xanthogranulomatous change. RCC recurrence was noted on follow-up MRI in 5 patients. In conclusion, RCCs are rare lesions that can present with an array of clinical symptoms. The relatively high rate of recurrence may indicate a link between this pathology and craniopharyngioma, although many patients with recurrent RCC do not require repeat resection and remain asymptomatic.

Background Transsphenoidal surgery for parasellar nonadenomatous lesions has the possibility to either improve or worsen pituitary hormonal function. Herein we present the rates and risk factors of new hormonal failure and recovery in patients undergoing surgery for either an RCC, craniopharyngioma, or tuberculum sella meningioma. Methods All consecutive patients treated over an 8-year period by endonasal surgery for an RCC, craniopharyngioma, or tuberculum sella meningioma were analyzed. Patients treated with prior sellar radiotherapy were excluded. Preoperative and postoperative pituitary hormonal status was determined. Patient characteristics, tumor size, intraoperative and postoperative events, and extent of tumor resection were correlated with new or resolved hypopituitarism. Results In total, 50 patients with an RCC, 18 with a craniopharyngioma and 13 with tuberculum sellae meningioma, were analyzed. New anterior pituitary failure and permanent DI occurred as follows: in RCCs, 6% and 2%; in craniopharyngiomas, 31% and 39%; and in meningiomas, 9% and 0%. Overall, improved hormonal function occurred in 57% of patients with an RCC including recovery of one or more anterior axes in 9 (41%) of 22 patients and resolution of hyperprolactinemia in 12 (67%) of 18 patients; no patients with a craniopharyngioma or meningioma had resolution of hypopituitarism. Younger age was predictive of hormonal recovery in patients with an RCC ( P = .026). Conclusions New hypopituitarism after transsphenoidal surgery occurs in approximately one third of patients with a craniopharyngioma and in less than 10% of patients with an RCC or suprasellar meningioma. Hormonal function improves in the majority of patients undergoing drainage of an RCC but is unlikely to occur after removal of a craniopharyngioma or suprasellar meningioma.

Background The transsphenoidal approach for resecting pituitary tumors has classically included reconstruction of the anterior wall of the sella turcica. A variety of materials have been used in this process for the purpose of both recreating the original anatomy and preventing postoperative cerebrospinal fluid (CSF) leaks. We studied the use of an ePTFE dural substitute (GORE Preclude MVP, W.L. Gore & Associates, Flagstaff, Arizona, USA), as a method for obtaining reliable reconstruction of the sellar dura. Methods A prospective alternate case trial was designed to assess this new dural substitute wherein 60 patients who underwent transsphenoidal surgical resection of intrasellar lesions larger than 1 cm were included. Thirty patients had the sellar dura closed with ePTFE dural substitute, whereas 30 patients underwent anterior wall reconstruction as ordinarily performed in our institution. Results Each patient was reassessed at closure, and the operative site and sphenoid sinus were evaluated with 3-months-postoperative magnetic resonance imaging. Three patients experienced postoperative CSF leaks: 1 with ePTFE dural substitute and 2 without. All 3 patients underwent a second operation to repair the CSF leak, without further complications. No other postoperative complications were noted in the series. Conclusion Expanded polytetrafluoroethylene dural substitute provides a safe and effective adjunctive method for reconstruction of the sellar dura.

To report a case of uncommon presentation of hemorrhagic Rathke's cleft cyst (RCC) extending into the cavernous sinus, causing diplopia.We present clinical, radiologic, and histologic findings on this patient, and review related medical literature.A 34-year-old man presented with a 4-month duration of worsening left-sided headache with acute development of diplopia which worsened upon looking to the left. Magnetic resonance imaging identified a left pituitary mass with posterior extension and involvement of the cavernous sinus. Hormone level evaluation revealed a low free thyroxine index and elevated prolactin levels, and normal levels of thyroid stimulating hormone, growth hormone, insulin-like growth factor type 1, and cortisol. The patient underwent transsphenoidal resection of a yellow fibrous mass from the left side of the sella. Tumor tissue pathology was consistent with a RCC that may have undergone secondary hemorrhage and rupture. Postoperatively, this patient developed transient diabetes insipidus, and required levothyroxine therapy for hypothyroidism. The diplopia improved, though he continues to complain of episodic headaches.RCCs are challenging to diagnose, as they can cause symptoms related to enlargement and hemorrhage. Imaging studies and other diagnostic measures may, therefore, describe heterogeneous features. The most common clinical presentation of this disorder is a manifestation of pituitary dysfunction, such as hyperprolactinemia, diabetes insipidus, and, occasionally, apoplexy. These symptoms may be accompanied by headache. Visual disturbances often appear, usually as visual field defects and decreased visual acuity. This case demonstrates that nonpituitary masses such as RCC can hemorrhage and extend into the cavernous sinus, causing acute ophthalmoplegia.

This is the first report that the authors are aware of concerning fluctuating visual field defects in patients with Rathke's cleft cysts. Among 11 patients with histologically proven Rathke's cleft cyst, three patients presented with a visual field defect which spontaneously resolved. The symptom became overt again in the subsequent 1 to 14 month follow-up period and a surgical procedure was then chosen. Magnetic resonance imaging (MRI) observation clearly revealed change in the cyst size without signal intensity change in two cases. In the remaining case, multiple spotty high intensities on T1-weighted images were found corresponding to fluctuations in the clinical course. In the last case, enlargement of the cyst was considered to be due to repeated haemorrhage. In the other two cases, minor haemorrhage, undetectable on MRI, may have played a role in the increased cyst size. Another possible mechanism is an imbalance between secretion from, and absorption into, the cyst wall. The size change and neurological fluctuations were previously unknown aspects of the natural history of this lesion. Surgical indications for such patients with transient symptoms followed by cyst size reduction have been controversial, since pituitary cystic lesions may enlarge again or disappear. Our observations suggest that this change in cyst size may be more frequently noted in cases with iso- or high intensity cysts on T1-weighted images, indicating the need for closer observation of patients with this MRI finding.

A very rare case of a growth hormone-secreting pituitary adenoma with a Rathke's cleft cyst is reported. Operative findings, histology and immunohistochemistry showed that both entities to be totally separate and unrelated. These findings suggest that the coexistence of a pituitary adenoma and a Rathke's cleft cyst in our case is merely a coincidence.

Rathke's cleft cysts (RCCs) are considered to arise from the remnants of Rathke's pouch, an invagination of the stomodeum. They are classically described as benign epithelium lined intrasellar cysts containing mucoid material, and also found in 2-33% of routine autopsy series. The most common presenting symptoms are visual impairment, hypothalamic dysfunction, hypopituitarism and headache. Diabetes insipidus has been described in patients with RCC. Very few cases presented with only diabetes insipidus in adults. To our knowledge, our patient is the first case of RCC presenting with only diabetes insipidus in childhood. A 9-year-old girl presented with diabetes insipidus. The physical, neurological and endocrinological examinations were normal, except for diabetes insipidus. Magnetic resonance imaging scan revealed a hyperintense lesion with supra sellar extension in the posterior pituitary both on T1 and T2 weighted images. Subtotal excision of RCC was performed via transsphenoidal surgery. However, diabetes insipidus persisted after the surgery.

A 42-year-old man presented with classical symptoms of Cushing's disease. Endocrinological examinations indicated the presence of a adrenocorticotropin-(ACTH) producing pituitary adenoma. Magnetic resonance imaging showed a cystic sellar lesion, which had features of a Rathke's cleft cyst, whereas the adenoma was not depicted. The ratio of blood ACTH concentration between cubital vein, left, and right cavernous sinus was 1: 1.4: 20. During transsphenoidal surgery, in addition to the Rathke's left cyst, a tiny adenoma was detected beneath the left pituitary lobe. Cavernous sinus sampling is a useful auxiliary in the localization of an ACTH-producing adenoma, as in this case.

A 65-year-old man presented with bitemporal hemianopsia. He had undergone frontal craniotomy for a Rathke's cleft cyst associated with narrowed visual field 8 years earlier. His vision had returned to normal soon after surgery and he remained asymptomatic until the present complaint. Neurodiagnostic imaging revealed a recurrence of the intrasellar cyst extending into the suprasellar cistern. His vision improved rapidly after a second surgery. The histological findings were the same as those of the previous operation; the cyst wall was composed of a single layer of ciliated columnar epithelium resting on a collagenous connective tissue stroma.

Two unusual cases of pituitary cysts are described. Both patients presented with sudden onset of severe headache, and magnetic resonance imaging (MRI) demonstrated pituitary cysts, which regressed over months. Although the precise etiology of the cysts was unproven, the cystic lesions in our patients are thought to have shrunken after “pituitary apoplexy.”

The patient was a 62 year old woman who presented with a visual field defect. Magnetic resonance images showed an intra-and supra-sellar low intensity mass on T1 weighted images. The preoperative diagnosis was craniopharyngioma, Rathke's cleft cyst (RCC) or arachnoid cyst. The patient underwent transsphenoidal surgery & the pathological diagnosis was RCC. We conclude that although RCC has a round, ovoid or dumb-bell shaped configuration, it may present a unusual shape in cases with preconditions, as were seen in our patient.

Epithelial cells composing symptomatic Rathke's cleft cysts were examined electron microscopically in two cases, and the fine structures were compared with those of the mucosa in the normal sphenoid sinus, of the normal choroid plexuses, and of a craniopharyngioma with goblet and ciliated cells. The Rathke's cleft cysts were composed of a single or pseudostratified epithelial layer abutting on the connective tissue. The epithelial cells were of four different kinds of cells, such as goblet, ciliated, flat, and basal cells. The flat cells containing bundles of tonofilaments and desmosomes were the squamous cells. They were present in a single-epithelial layer in both cases. The basal cells were present as small, wedge-shaped cells, even if the number was very small. The epithelial cells in Rathke's cleft cysts were more similar to those of the mucosa in the sphenoid sinus and those of a craniopharyngioma with goblet and ciliated cells than those of the choroid plexus. This study indicates the close relationship between Rathke's cleft cysts and mucosa in the sphenoid sinus or craniopharyngioma with ciliated and goblet cells.

An abscess in a Rathke's cleft cyst was surgically treated in a 39-year-old man. The patient presented with headaches, fever, and visual deficits. Transcranial decompression of the optic chiasm was carried out first. The abscess recurred, however, and drainage of the abscess and removal of its wall via the transsphenoidal route was carried out 4 weeks later. Visual evoked responses were useful in the successful management of this lesion. The patient regained normal pituitary function and visual function after the operation.

A case of this rare parasellar cyst is reported in a patient presenting for orthognathic surgery. The pathogenesis, clinical, histologic and radiologic features of this cyst are reviewed. This case underscores the necessity of careful evaluation of cephalometric radiographs.

Reports of epidermoid tumors from 1965 through 1986 were reviewed with emphasis on new and unusual findings. Clinical data considered were rapid onset, rare symptoms, size and rate of growth, and complications. Uncommon locations included multiple sites in one patient and the anterior fontanelle. The radiologic data from computed tomography were the main contributors to diagnosis. An immunohistochemical test disclosed that cells of craniopharyngiomas are keratin-positive. Histologically benign epidermoids can behave as highly malignant tumors, and can become carcinomatous. Postoperative results for benign tumors were often excellent. Evidence was presented of the close relation among epidermoid and dermoid tumor, craniopharyngioma, ameloblastoma (adamantinoma), Rathke's cleft cyst, and aural cholesteatoma.