Rathke's Cleft Cyst Research Articles

Sellar Pathologies Mimicking Pituitary Tumors.

The most common sellar pathology that merits neurosurgical attention is the pituitary adenoma. However, some developmental, inflammatory, and neoplastic lesions may primarily or secondarily involve the sella, mimicking pituitary tumors. Advances in imaging and endocrinological assessment have helped in the recognition of these less common sellar, supra sellar pathologies, which may occasionally create confusion in management. The most common developmental anomaly is the Rathke's cleft cyst and an increasingly recognized inflammatory pathology is the spectrum of hypophysitis. Neoplasms, viz. Craniopharyngioma, Germinoma, Langerhans's cell histiocytosis or metastasis, have more distinctive features in various age groups and are more likely to be correctly diagnosed on current imaging and managed accordingly. This review looks at mainly intraparenchymal pathologies, namely Rathke's Cleft Cyst and various hypophysitides, and will discuss their management strategies.

Endonasal Endoscopic Fenestration of Rathke's Cleft Cysts: Whether to Leave the Fenestration Open or Closed?

Introduction Rathke's cleft cysts (RCC) are generally treated with transsphenoidal fenestration and cyst drainage. If no cerebrospinal fluid (CSF) leak is created, the fenestration can be left open. If CSF is encountered, a watertight closure must be created to prevent postoperative CSF leak, though sellar closure has theoretically been linked with higher recurrence rate. In this study, we investigate the relationship between sellar closure, rate of postoperative CSF leak, and RCC recurrence. Methods Retrospective review of a prospective database of all endoscopic endonasal RCC fenestrations and cases were divided based on closure. The "open" group included patients who underwent fenestration of the RCC, whereas the "closed" group included patients whose RCC was treated with fat and a rigid buttress ± a nasoseptal flap. The rate of intra- and postoperative CSF leak and radiographic recurrence was determined. Results The closed group had a higher rate of suprasellar extension (odds ratio [OR]: 8.0, p = 0.032) and intraoperative CSF leak ( p ≤ 0.001). There were 54.8% intraoperative CSF leaks and no postoperative CSF leaks. Radiologic recurrence rate for the closed group (35.0%) was three times higher than the open group (9.1%; risk ratio [RR] = 3.85, p = 0.203), but not powered to show significance. None of the radiologic recurrences required reoperation. Conclusion Maintaining a patent fenestration between an RCC and the sphenoid sinus is important in reducing the rate of radiographic recurrence. Closure of the fenestration may be required to prevent CSF leak. While closure increases the rate of radiographic recurrence, reoperation for recurrent RCC is still an uncommon event.

Imaging findings in hypophysitis: a review.

Hypophysitis (HP) is a rare acute or chronic inflammatory condition of the pituitary gland. The greatest challenge in the management of HP is establishing a diagnosis through clinical criteria and non-invasive methods and predicting the patients' clinical outcome. The aim of this review is to describe the neuroradiological findings of this rare disease, providing some information regarding the possible differential diagnosis in order to avoid unnecessary surgery. Gadolinium-enhanced pituitary magnetic resonance imaging (MRI) is considered the neuroradiological investigation of choice. The features suggestive for HP include an enlarged triangular- or dumbbell-shaped gland with a thickened and not obviously deviated stalk, further supported by the absence of posterior pituitary bright spot on T1weighted images, particularly in patients presenting with diabetes insipidus. Contrast enhancement pattern is quite variable; dural enhancement has been reported in some cases after intravenous contrast administration. The characterization of the unusual sellar mass is not straightforward and generally results in a wide differential. HP should be primarily differentiated from pituitary adenomas (including pituitary apoplexy), from pituitary metastases, and from other sellar and parasellar tumors, e.g., craniopharyngiomas, germinomas, gliomas, lymphomas, meningiomas, pituicytomas, chordomas, teratomas, dermoids and epidermoids, Rathke's cleft cysts, and abscesses. In patients suspected for secondary forms related to systemic pathology, additional imaging is helpful in identifying other involved sites. Neuroradiologists need to know MRI appearance of this rare disease, as well as its typical symptoms and serological markers. A strict collaboration with endocrinologists and neurosurgeons is mandatory in order to reach a definitive diagnosis, allowing to promptly initiating an appropriate treatment.

Usefulness of Contrast-Enhanced 3D-FLAIR MR Imaging for Differentiating Rathke Cleft Cyst from Cystic Craniopharyngioma.

Because it can be difficult to discriminate between a Rathke cleft cyst and cystic craniopharyngioma by conventional MR imaging alone, we investigated whether contrast-enhanced 3D T2-FLAIR MR imaging at 3T helps to distinguish a Rathke cleft cyst from a cystic craniopharyngioma. We evaluated pre- and postcontrast T1-weighted and 3D T2-FLAIR images of 17 patients with pathologically confirmed Rathke cleft cyst (n = 10) or cystic craniopharyngioma (n = 7). All underwent 3T MR imaging studies before surgery. Two neuroradiologists independently recorded the enhancement grade of the lesion wall as grade 2 (most of the wall enhanced), grade 1 (some of the wall enhanced), and grade 0 (none of the wall enhanced). One neuroradiologist performed a blinded reading study of conventional MR images with/without 3D T2-FLAIR images. Interobserver agreement was determined by calculating the κ coefficient. Statistical analyses, including receiver operating characteristic curve analysis were performed. Interobserver agreement for postcontrast T1WI and 3D T2-FLAIR images was excellent (κ = 0.824 and κ = 0.867, respectively). Although the difference in the mean enhancement grade of Rathke cleft cysts and cystic craniopharyngiomas was not significant on postcontrast T1WIs, it was significant on postcontrast 3D T2-FLAIR images (P = .0011). The area under the receiver operating characteristic curve of the conventional MR alone and conventional MR with 3D T2-FLAIR readings was 0.879 and 1.0, respectively, though there was no significant difference in the area under the curve between the 2 readings. Contrast-enhanced 3D T2-FLAIR imaging at 3T helps to distinguish a Rathke cleft cyst from cystic craniopharyngioma.

Cystic Glioblastoma Rupturing into the Ventricle

Cystic tumors, such as craniopharyngiomas and Rathke’s cleft cysts, as well as arachnoid cysts have been reported to rupture occasionally. Approximately 8–10% of glioblastomas (GBMs) are known to have a significant cystic component; however, to the best of our knowledge, no studies have reported cystic rupturing of GBMs. Here, we describe a unique case of cystic GBM rupturing and penetrating into the cerebral ventricle. A 77-year-old man with a right frontal lobe lesion suspected as GBM with a large cyst was referred to our hospital. At admission, disorientation and left facial weakness were detected. Consciousness disturbance worsened on the 8th day of hospitalization. Computed tomography (CT) revealed prominent shrinkage of the tumor and intratumoral cyst. Signs of meningeal irritation were observed, and chemical meningitis due to cystic tumor rupture and leakage of necrotic components into the ventricle was highly suspected. Surgical resection of the right frontal lobe tumor was performed on the 10th day of hospitalization. During the surgery, clear and colorless cerebrospinal fluid was obtained upon penetration of the tumoral cyst, suggesting traffic of tumor cysts and cerebral ventricle. Adjuvant chemoradiation therapy was initiated postoperatively. Local recurrence was noted at the corpus callosum 7 months postoperatively and was treated with a gamma knife. Further therapy was performed after this recurrence. However, his condition gradually deteriorated 15 months postoperatively, and he was subjected to terminal care. To the best of our knowledge, this is the first report on a cystic GBM rupture.

Cystic sellar salivary gland-like lesions

Introduction: Cystic sellar salivary gland-like lesions (CSSLs) are exceedingly rare, with fewer than a dozen case reports. They contain amorphous colloid identical to Rathke cleft cyst contents, but the cyst wall additionally shows cohesive aggregates of benign salivary glands. We report three new examples. Materials and methods: Two cases were seen at University of Colorado Denver and one at Memorial Sloan Kettering (MSK). Molecular testing was attempted on two of three. Results: Case 1 is a 20-year-old female who presented with panhypopituitarism and was found to have a suprasellar mass that proved to be a CSSL. She received no postoperative adjuvant therapy, but recurrence of headaches and blurred vision 2 years later prompted return to medical attention. A much smaller local cyst recurrence was now accompanied by a thickened, bulbous infundibular stalk. Second resection yielded a gliotic infundibular stalk and amorphous mucin, but no residual salivary-like glands. She is without further recurrence on 6-year follow-up. Case 2 is a 29-year-old female with headache; while seen initially at a tertiary care center, diagnosis was only made after consultation at MSK. Case 3 is 68-year-old female who had originally presented with apoplexy to an outside hospital 7 years prior to surgery and diagnosis. Molecular testing was uninformative on case 1 and negative for mutations or fusions on case 3. Conclusion: Few pathologists or neuropathologists have encountered CSSLs in their practices; case 1 produced recurrence and significant infundibular stalk damage, and case 3 originally manifested apoplexy, features not previously reported.

Collision sellar lesions: coexistence of pituitary adenoma and Rathke cleft cyst-a single-center experience.

Collision sellar lesions represent the coexistence of distinct histopathological lesions found in the sella turcica. They are uncommon entities and have mainly been reported as pituitary adenoma (PA) associated to Rathke cleft cyst (RCC). Pre- and perioperative diagnosis is difficult, since most of the cases appear clinically, radiologically, and macroscopically as solitary cystic sellar lesion. A retrospective study of histological reports from patients operated for PA from 2013 to 2018 in a single neurosurgery reference center was performed. Patients who also exhibited RCC in the histological sections were included. Clinical and biochemical data were collected from medical files. MRI scans and histopathological slides were also reviewed. Among 554 PA, five patients (0.9%) presented the association of PA and RCC. At diagnosis, patients had median age of 60 years (33-78) with, at least, one pituitary dysfunction, and visual field loss and/or headache. There was a female predominance (n = 3). All patients had nonfunctioning PA. MRI studies showed a predominantly cystic lesion and were unable to distinguish both lesions. The definitive diagnosis was made by histopathology. The association of PA and RCC is extremely rare. On MRI, they appear as a solid-cystic or cystic sellar tumors. RCC can rupture causing granulomatous reaction with cholesterol crystal formation, which can be mistaken for craniopharyngiomas during surgery. Therefore, collision sellar lesion must be included in the differential diagnosis of cystic sellar lesions. The definitive diagnosis is made by histological study.

MRI and pathological features of Rathke cleft cysts in the sellar region.

The aim of the present study was to investigate the MRI and pathological features of Rathke cleft cysts (RCC) in the sellar region. A total of 45 RCC cases were retrospectively analyzed. RCC size, location, intracyst nodules and general signals, as well as the posterior pituitary bright spot (PPBS) were analyzed using MRI-T1 weighted images (T1WI) and T2WI. The relationship between the presence of PPBS and histopathological features was additionally evaluated. On T1WI, there were 18 cases of isointense signal, 16 cases of hyperintense signal, 9 cases of hypointense signal, 1 case of heterogeneous signal and 1 case with a stratification effect, with isointense signal in the upper part and hyperintense signal in the lower part. On T2WI, there were 5 cases of isointense signal, 27 cases of hyperintense signal, 11 cases of hypointense signal and 1 case of the stratification effect. There were 10 cases of PPBS+ and 35 cases of PPBS-. There were no significant differences in the age, sex, cyst location and size between PPBS+ and PPBS- cases. However, PPBS+ cases had significantly lower inflammation than PPBS- cases. A total of 20 cases of intracystic nodules were identified on MRI scans, most of which exhibited T2 -hypointense signals. The shape of RCC nodules varied and there were 17 cases where the nodules were non-adherent to the cyst wall. The MRI signals of RCCs varied and most nodules were floating within cysts. Intracystic nodules are characteristic features of RCCs when observed by MRI and thus are of high diagnostic value. Most patients with RCC were also PPBS-, which may be associated with an increased inflammatory response.

Clinical investigation of pituitary incidentalomas: A two-center study.

Recent advances in imaging technology resulted in an increase in pituitary incidentalomas (PIs) detection. PIs were reported to be present in 1.6% persons with magnetic resonance imaging of the brain. Whereas, there were few studies about PIs with detailed investigation. We aimed to investigate the clinical and endocrinological characteristics of PIs. We evaluated 65 patients diagnosed with PIs who underwent detailed clinical and endocrinological evaluations. Of the 65 patients, 33 (50.8%) had non-functional pituitary adenomas (NFPAs), 11 (16.9%) had Rathke's cleft cysts (RCCs), 7 (10.8%) had functional pituitary adenomas (FPAs), 6 (9.2%) had benign extra-pituitary tumors (BEPTs), and 8 (12.3%) had malignant tumors (MTs). Compared with patients with NFPAs, those with MTs were significantly younger and had a significantly lower body mass index, lower prevalence of hypertension, and lower prevalence of dyslipidemia. Patients with MTs had significantly higher prevalence of central diabetes insipidus than those with NFPAs. In addition, patients with NFPAs had significantly higher prevalence of pituitary apoplexy than those with FPAs, BEPTs, and MTs. In conclusion, our study demonstrated clinical and endocrinological characteristics of PIs. Highly detailed clinical and endocrinological investigations should be performed for PIs. In addition, MTs should be considered in the differential diagnosis for young and lean patients with central diabetes insipidus.

Bilateral Smell Preservation is Routinely Possible following Endoscopic Endonasal Approach for Sellar/Suprasellar Lesions.

Objective The endoscopic endonasal approach (EEA) has been shown to be an effective means to access sellar lesions. However, there are limited studies centered on evaluating olfactory function after surgery. In this study, we assessed the pre- and postoperative olfactory function of patients who underwent EEA for sellar and suprasellar lesions. The impact of nasoseptal flap use on olfaction was further analyzed. Study Design A retrospective study. Setting A tertiary-care referral center in Taiwan. Participants Patients with sellar and suprasellar lesions who underwent EEA and pre- and postoperative olfaction assessment from August 2015 to March 2018 were included in the study. Main Outcome Measures The patients' olfactory function was examined pre-and postoperatively using the Sniffin' Sticks odor identification test. Data regarding demographics, olfactory scores, pathology, reconstructive technique, graft usage, and extent of resection were retrieved. Results A total of 106 patients (36 males and 70 females) were enrolled, with a mean age of 51 years. There were 76 pituitary tumors, 12 Rathke's cleft cysts, 7 craniopharyngiomas, 7 meningiomas, and 4 other lesions. The nasoseptal flap was used in 39 patients for skull base reconstruction, and these patients had no statistically significant change between pre- and postoperative olfactory scores ( p = 0.283). Moreover, a statistically significant improvement of olfactory scores was found in patients in whom the nasoseptal flap was not used. Conclusion Olfactory function may be reliably preserved after EEA, with or without nasoseptal flap harvest and use.

RARE-02. SYMPTOMATIC SALIVARY GLAND-LIKE TUMORS OF THE SELLAR REGION: ADDITIONAL FEATURES AND REVIEW OF THE LITERATURE

Abstract INTRODUCTION Sellar symptomatic salivary gland-like tumors (SSGTs) are exceedingly rare, with less than a dozen case reports in the literature. They usually consist of amorphous colloid identical to Rathke cleft cyst (RCC) contents, but additionally the cyst wall contains a large, cohesive collection of benign salivary glands. We report two new examples, one of which represents the first instance of recurrence of symptoms 2 years after first surgery, the second time with cyst confined to infundibular stalk and causing severe gliosis, indicative of mass effect. The second example underscores that SSGT remains poorly recognized, even today amongst experts. METHODS One case each was retrieved from the files of University of Colorado Denver (UCD) and Memorial Sloan Kettering (MSK); the UCD case represents the sole example in 2000+ sellar region masses seen at our institution since 1997. The MSK case had been recently received in consultation. RESULTS Case 1 is a 20-year-old female who presented with 3 weeks of symptoms referable to obstructive hydrocephalus; neuroimaging disclosed a sellar/suprasellar 2.4 x 2.2 x 2.4 cm mass. Following surgical resection, SSGT was diagnosed. She received no adjuvant therapy, but recurrence of headaches and blurred vision 2 years later prompted her return to medical attention. MRI revealed a recurrent cyst confined to the center of a thickened, bulbous infundibular stalk. Second resection yielded amorphous mucin within the cystic and severely-gliotic infundibular stalk, but no salivary-type glands. She has been followed for the last 6 years without tumor recurrence. Case 2 is a 29-year-old female with headache. The lesion was seen initially by a neuropathologist at another institution before it was sent to MSK for consultation and SSGT was diagnosed. CONCLUSION Few pathologists or neuropathologists have encountered SSGT in their practices; case 1 produced recurrence and significant local stalk damage, features not previously reported.

Magnetic resonance imaging characteristics and differential diagnosis of common sellar cystic lesions

Objective: To investigate magnetic resonance imaging (MRI) characteristic and differential diagnostic keypoints of common sellar regional cystic lesions with the purpose of improving differential diagnostic accuracy. Methods: In total, 174 cases of the pathologically diagnosed cystic lesions in sellar region between March 2016 and June 2019 were included in the current retrospective analysis. These cases included Rathke's cleft cyst (n=68), craniopharyngioma (n=48), pituitary adenoma (n=56) and arachnoid cyst (n=2) in the sellar region. The position, texture and morphology of leisions, signal of cystic and solid part, invasion of surrounding tissues and maximum diameter of cysts on the MRI images were evaluated and compared between different groups. SPSS 25.0 software was used for statistical analysis. Results: There were several specific changes on several MRI sequences in Rathke's cleft cysts, craniopharyngioma and pituitary adenoma groups. For Rathke's cleft cysts, round shape, rare polycystic structure, lack of solid part and maximum diameter of cysts which was significantly less than that of craniopharyngioma and pituitary adenoma groups ((17.37±6.12) mm vs (30.29±13.51) mm vs (28.18±11.13) mm, t value was 6.680, 5.838, respectively, all P<0.05), were favorable diagnostic criteria. While, the intracystic nodules were mostly found in craniopharyngioma. There was no high signal intensity in cystic wall on T1WI and T2WI of pituitary adenoma. MRI signal of arachnoid cyst in sellar region was basically the same as CSF signal, with low signal intensity on T1WI and DWI, high signal intensity on T2WI, without enhanced signal. Conclusions: Common sellar regional cystic lesions showed characteristic manifestations on different MRI sequences. Valuable information in the morphology, MRI signal intensity, maximum diameter measurements may improve accuracy of differential diagnosis for sellar regional cystic lesions.

Endoscopic endonasal cyst fenestration into the sphenoid sinus using the mucosa coupling method for symptomatic Rathke's cleft cyst: a novel method for maintaining cyst drainage to prevent recurrence.

Rathke's cleft cyst (RCC) is a benign cystic lesion with a relatively high incidence of local recurrence that occasionally requires repeat surgery. To prevent recurrence, simple cyst fenestration and drainage of the cyst contents to the sphenoid sinus is recommended, but it occasionally recurs. The authors postulated that obstruction of fenestration is a main cause of recurrence, and they developed a method, named the "mucosa coupling method (MC method)," that maintains persistent drainage. In this method, the RCC epithelium and the mucosa of the sphenoid sinus are connected, which promotes re-epithelialization between the two epithelia, maintaining persistent drainage. The outcome of this method was compared with that of conventional cyst fenestration. In a consecutive series of 40 patients with RCC, the surgical strategy was changed during the study period: from December 2009 to September 2014 (the conventional period), 24 patients were scheduled to be treated using the conventional fenestration method, whereas from September 2014 to September 2017 (the MC period), 16 patients were scheduled to be treated using the MC method. However, because of an intraoperative CSF leak, the fenestration was closed during surgery in 3 patients in the conventional period and 2 in the MC period; therefore, these 5 patients were excluded from the analysis. Twenty-one patients treated with the conventional fenestration method (conventional group) and 14 patients treated with the MC method (MC group) were analyzed. All patients regularly underwent MRI after surgery to detect reaccumulation of cyst contents. The rate of reaccumulation with and without reoperation, visual outcomes, endocrinological outcomes, and postoperative complications were compared between these two groups. The median follow-up period in all 35 patients was 48.0 months (range 1-96 months), 54.0 months (range 1-96 months) in the conventional group and 35.5 months (range 12-51 months) in the MC group. No reaccumulation was detected on MRI in the 14 patients in the MC group, whereas it was noted in 9 (42.9%) of 21 patients in the conventional group, and 2 of these 9 patients required repeat surgery. There were no significant differences in visual and endocrinological outcomes and complications between these two groups. The MC method for RCC is effective for preventing obstruction of cyst fenestration, which contributes to preventing cyst reaccumulation. Furthermore, this method is equivalent to the conventional fenestration method in terms of visual and endocrinological outcomes and the complication rate.

Differentiation of Pituitary Adenoma from Rathke Cleft Cyst: Combining MR Image Features with Texture Features.

Objectives To differentiate pituitary adenoma from Rathke cleft cyst in magnetic resonance (MR) scan by combing MR image features with texture features. Methods A total number of 133 patients were included in this study, 83 with pituitary adenoma and 50 with Rathke cleft cyst. Qualitative MR image features and quantitative texture features were evaluated by using the chi-square tests or Mann–Whitney U test. Binary logistic regression analysis was conducted to investigate their ability as independent predictors. ROC analysis was conducted subsequently on the independent predictors to assess their practical value in discrimination and was used to investigate the association between two types of features. Results Signal intensity on the contrast-enhanced image was found to be the only significantly different MR image feature between the two lesions. Two texture features from the contrast-enhanced images (Histo-Skewness and GLCM-Correlation) were found to be the independent predictors in discrimination, of which AUC values were 0.80 and 0.75, respectively. Besides, the above two texture features (Histo-Skewness and GLCM-Contrast) were suggested to be associated with signal intensity on the contrast-enhanced image. Conclusion Signal intensity on the contrast-enhanced image was the most significant MR image feature in differentiation between pituitary adenoma and Rathke cleft cyst, and texture features also showed promising and practical ability in discrimination. Moreover, two types of features could be coordinated with each other.

Differential Clinical Presentation, Intraoperative Management Strategies, and Surgical Outcomes After Endoscopic Endonasal Treatment of Cystic Sellar Masses

Cystic sellar masses (CSMs) pose diagnostic and therapeutic challenges associated with subtotal cyst wall resection, cerebrospinal fluid (CSF) leak repair, and disease recurrence. Current magnetic resonance imaging (MRI) interpretation often cannot reliably differentiate CSMs, mandating adaptable intraoperative strategies. We reviewed our diagnostic and therapeutic experience after endoscopic endonasal approaches (EEAs) for CSMs. A retrospective record review of patients with CSM managed via EEA at the University of Southern California from 2011 to 2018 was conducted. Patient demographics, preoperative characteristics, surgical details, pathologic findings, and postoperative outcomes were assessed. Analysis included 47 patients (mean age, 43.2 years); of these, 78.7% were women. Preoperative symptoms included headache (76.6%) and vision loss (42.6%). Histologically verified sellar pathology included 27 Rathke cleft cysts (RCCs) (57.4%), 17 cystic pituitary adenomas (CPAs) (36.2%), 2 arachnoid cysts (4.3%), and 1 xanthogranuloma (2.1%). Twelve patients (70.6%) with CPAs underwent complete resection and 5 (29.4%) underwent subtotal resection. All 27 patients with RCC and 2 patients with arachnoid cyst underwent complete fenestration and drainage. One xanthogranuloma was completely resected. There were 14 intraoperative (29.8%) and 4 postoperative CSF leaks (8.5%). Headaches, vision, and endocrinopathy improved in 69.2%, 80.0%, and 33.3% of patients with CPA and 73.9%, 71.4%, and 40.9% of patients with RCC, respectively. There were 2 RCC recurrences and 1 CPA recurrence over the follow-up period. Surgeons must prepare for versatile management strategies of CSMs based on pretest probability associated with MRI and intraoperative findings. Outcomes after EEA for CSMs show low complication profiles and excellent rates of headache and visual improvement, albeit lower rates of endocrine normalization.

Clinicopathologic Characteristics and Endoscopic Surgical Outcomes of Symptomatic Rathke's Cleft Cysts

The purpose of this study was to evaluate the preoperative clinical data and postoperative endocrinologic improvement, recurrence, and complications of Rathke's cleft cysts (RCCs) in a large series of endoscopic transsphenoidal surgery patients, operated in a single center. Between 1997 and April 2018, 84 patients underwent endoscopic transsphenoidal surgery for RCCs. The presentation symptoms, endocrinologic results by localization and content of the cysts, surgical resections, postoperative complications, and recurrences were evaluated retrospectively. Chronic headache was reported in 66.6% (56/84), visual field defects were present in 20.2% (17/84), and 23.8% (20/84) of the cases had pituitary dysfunction. Among the endocrinopathies, 17 of 17 patients had hyperprolactinemia, 2 of 7 patients had cortisol deficiency, and 2 of 13 had hypogonadism, which resolved after surgery. Preoperative pituitary hormone deficiency was associated with gelatinous tumor content (P= 0.044). However, there was no significant relationship between tumor localization and preoperative hormone insufficiency. Postoperative hypocortisolemia was seen more frequently in intrasellar RCCs (P= 0.048). Three new pituitary hormonal deficiencies were identified in postoperative patients (1hypocortisolism, 1 diabetes insipidus, 1 hypogonadism). Gross total resection was achieved in 20 cases (23.8%), 9 of which were performed using an extended approach. Postoperative complications included 3 cerebrospinal fluid leakages and 1 epistaxis. Two patients presented with cyst recurrence at follow-up at 12 and 26 months. Preoperative hormone deficiency was found to be associated with tumor content, independent of size or localization. The surgical aim for symptomatic RCCs should be decompression and this is effective for endocrinologic improvement. An extended endoscopic approach was adequate for total cyst wall removal in suprasellar cysts. Endoscopic transsphenoidal surgery was successful, with low complication rates, for decompression of intrasellar and suprasellar cysts in both complete cyst wall resection and cyst fenestration.

Benefit of Intracystic Bleomycin for Symptomatic Recurrent Rathke Cleft Cyst.

Management of recurrent Rathke cleft cysts (RCC) is problematic. The mainstay of treatment has been reoperation with cyst drainage. Radical cyst resection, although effective, results in a high incidence of diabetes insipidus. Several case reports suggest a potential benefit to radiation therapy or the use of intracystic bleomycin. The intracystic application of bleomycin is known to be beneficial in the treatment of cystic craniopharyngioma; however, its usefulness in the treatment of recurrent RCC has yet to be proven. To present our 6-yr experience using intracystic bleomycin for recurrent RCC. We performed a retrospective chart review of patients with RCC who underwent surgical resection between January 2010 and May 2016 by a single surgeon. Specific attention was paid to patients with recurrent RCC. Of the 59 patients operated on for RCC during this 6-yr interval, 6 patients with symptomatic recurrent RCC were identified and received intracystic bleomycin at the time of reoperation. To date, no symptomatic cyst recurrence has been documented in the patients receiving bleomycin (mean=38.8 mo, range 21.2-79.8 mo). The use of intracystic bleomycin appears to be a safe and potentially effective treatment option in patients with recurrent RCC. Additional studies with longer follow-up are needed to further define the role of bleomycin in recurrent RCC.

Delayed Cerebrospinal Fluid Leakage After Treatment of Skull Base Tumors: Case Series of 9 Patients

Cerebrospinal fluid (CSF) leaks usually happen in the early postoperative period. However, delayed CSF leak can happen several years after treatment. The purpose of this study was to review the characteristics and clinical course of delayed CSF leak after treating skull base tumors. We treated 9 patients with delayed CSF leak (occurring at least 3 months after treatment) between December 2015 and February 2018. Clinical data including initial treatment modality for skull base tumor, time between treatment and CSF rhinorrhea, and the result of endoscopic CSF repair were evaluated by retrospective chart review. Nine patients with delayed CSF leak were evaluated. The mean age was 42.3 ± 5.2 (mean ± SD) years. Six patients with pituitary adenomas, 1 with a Rathke cleft cyst, 1 with a meningioma, and 1 with an osteosarcoma were enrolled. Seven patients (78%) received radiation, and the mean radiation dose was 55.7 ± 2.6 Gy. The median time between the end of treatment for the skull base tumor and CSF rhinorrhea was 137.0 (interquartile range 24-145) months. Seven patients (78%) had meningitis at the time of operation. CSF rhinorrhea was treated with endoscopic reconstruction using a multilayer technique. Meningitis was improved without neurologic sequelae after reconstruction and antibiotic therapy. One patient had recurrence of CSF leak. Patients with delayed CSF leak are more likely to have a history of radiation and to present with meningitis at diagnosis. Therefore, CSF rhinorrhea should always be suspected, even several years after treatment, if early symptoms of CSF leak develop in patients with a history of skull base tumor.

Evaluation of pituitary structures and lesions with turbo spin-echo diffusion-weighted imaging

Background and purposeTurbo spin-echo diffusion-weighted imaging (TSE-DWI) has not been used for evaluating pituitary lesions. We compared the usefulness of TSE-DWI and echo-planar (EP)-DWI for assessing normal pituitary structures and lesions. Materials and methodsOur study included 41 consecutive patients (27 pituitary adenomas, 8 Rathke's cleft cysts, 4 craniopharyngiomas, 1 germinoma, 1 pituitary metastasis) who underwent conventional pre- and post-contrast magnetic resonance imaging (MRI) and TSE- and EP-DWI at 3T. Two observers independently performed qualitative assessment of normal pituitary structures and lesions on sagittal DWI and apparent diffusion coefficient (ADC) maps. One observer recorded ADC values of normal brain structures and pituitary lesions. Kappa (κ) statistics, Wilcoxon signed-rank test, intraclass correlation coefficient, Bland-Altman analysis, Pearson correlation coefficient and independent t-test were used for statistical analysis. ResultsInterobserver agreement for qualitative evaluations was good to excellent (κ = 0.65–1.0). On both DWI and ADC maps, visualization of the pituitary gland, of the spatial relationship between the lesion and its normal surroundings, and the whole image quality were significantly better on TSE- than EP sequences (p < .01). In normal brain structures, the ADC value on TSE- and EP-sequences was significantly correlated (r = 0.6979, p < .05). The TSE-ADC value was significantly lower for pituitary adenomas than craniopharyngiomas (p < .05). ConclusionsFor the evaluation of normal pituitary structures and lesions, TSE-DWI is more useful than EP-DWI. The TSE-ADC value may help to differentiate between pituitary adenoma and craniopharyngioma.

Outcomes following endoscopic endonasal resection of sellar and supresellar lesions in pediatric patients

The endoscopic endonasal approach (EEA) is a credible surgical alternative for the resection of sellar and suprasellar lesions such as pituitary adenomas, craniopharyngiomas, and Rathke cleft cysts. However, its application to pediatric patients poses several unique challenges that have not yet been well evaluated. The authors evaluate the safety, efficacy, and outcomes associated with the use of the EEA for treatment of these pathologic entities in pediatric patients. Retrospective review of 30 patients between the ages of two and 24 who underwent endoscopic endonasal resection of sellar or suprasellar lesions between January 2010 and December 2015. Endocrinological and ophthalmological outcomes, as well as extent of resection and complications were all evaluated. Gross total resection was achieved in eight of the nine pituitary adenomas, nine of the 12 craniopharyngiomas, and six of the nine Rathke cleft cysts. Of the 30 patients, 22 remained disease free at last follow-up. A total of six patients developed hypopituitarism and five developed diabetes insipidus. Eleven patients experienced improved vision, sixteen experienced no change, and one patient experienced visual worsening. Postoperative cerebrospinal fluid leak was seen in a single case and later resolved, vasospasm/stroke was experienced by 10%of patients, and new obesity was recorded in 10% of patients. There were no perioperative deaths. Endoscopic endonasal resection is a safe and effective surgical alternative for the management of sellar and suprasellar pathologies in pediatric populations with excellent outcomes, minimal complications, and a low risk of morbidity.