Rathke’s cleft cyst with xanthogranulomatous change: A case report and review of the literature

Background: Rathke’s cleft cysts (RCCs) are benign, typically asymptomatic sellar lesions found incidentally in adults, with a dramatically lower incidence in pediatric patients (<18 years). We present a case of RCC with xanthogranulomatous change (XGC) – an even less common subtype of RCC – treated by endoscopic endonasal surgical resection. This is the second reported instance of an RCC with XGC occurring in a pediatric patient. Case Description: The patient is a 17-year-old male with delayed puberty who presented with bitemporal hemianopsia and was found to have a 2.6 cm lesion, initially thought to be a craniopharyngioma. He subsequently underwent uncomplicated transsphenoidal endoscopic endonasal resection. Histology confirmed the diagnosis of RCC and demonstrated marked degenerative XGCs with squamous metaplasia. The patient tolerated the procedure well with improvement in visual symptoms. Conclusion: RCC with XGC is a very rare pathology, particularly in the pediatric population. These lesions, while benign, can manifest clinically with significant symptoms. While treatment paradigms are not fully established with a small cohort of cases, endoscopic endonasal approaches have made surgical resection of these lesions a safe and effective treatment strategy, even in the pediatric population.

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Presentation and outcomes in surgically and conservatively managed pediatric Rathke cleft cysts

Journal of Neurosurgery Pediatrics ◽

10.3171/2017.9.peds17400 ◽

2018 ◽

Vol 21 (3) ◽

pp. 308-314 ◽

Cited By ~ 2

Author(s):

Matthew J. Shepard ◽

Mohamed A. Elzoghby ◽

Erin N. Kiehna ◽

Spencer C. Payne ◽

John A. Jane

Keyword(s):

Visual Field ◽

Pediatric Patients ◽

Pediatric Population ◽

Study Patient ◽

Endoscopic Endonasal ◽

Diagnostic Uncertainty ◽

Conservative Group ◽

Sellar Lesions ◽

Operative Notes ◽

Cyst Fenestration

OBJECTIVERathke cleft cysts (RCCs) are sellar lesions that are commonly encountered in adults but infrequently diagnosed in the pediatric population. As a result, the optimal management of pediatric RCCs remains a subject of controversy. Only 2 prior surgical series have been published on pediatric RCCs and no study has compared the presentation and outcomes of surgically versus conservatively managed cases. The authors therefore performed a comparative analysis of pediatric cases of RCC in which patients were treated with surgery or managed in a conservative manner.METHODSAll cases involving pediatric patients diagnosed with an RCC at the University of Virginia between 2000 and 2016 were included in this study. Patient medical records, operative notes, and neuroimaging findings were reviewed. Patients who developed visual field deficits, radiographic evidence of chiasmal compression, or medically refractory headaches were considered candidates for surgical intervention. All patients who were selected for surgery underwent an endoscopic endonasal approach with cyst fenestration.RESULTSA total of 24 pediatric patients were diagnosed with an RCC over a 16-year period. Seven patients ultimately underwent transsphenoidal cyst fenestration, and 17 were managed conservatively. The patients’ age at diagnosis, cyst size, and pituitary function at the time of RCC diagnosis were similar in the conservatively and surgically managed cohorts. At diagnosis, 19 of 24 patients endorsed headaches that led to neuroimaging. All patients in the surgical cohort endorsed severe headaches at diagnosis compared with 71% in the conservative group. For the 7 patients treated with surgery, complete cyst evacuation was achieved in 86% of cases. Transient postoperative endocrinopathy occurred in 4 (57%) of 7 surgically treated individuals and resolved in all cases. In the conservative cohort, 1 patient developed a delayed pituitary-related endocrinopathy. Headache resolution occurred in 5 (71%) of the 7 patients who underwent surgery and 7 (58%) of the 12 who were treated without surgery. Cyst recurrence was documented in 1 individual in the surgical cohort who underwent a subtotal cyst fenestration that ultimately required re-intervention. In the conservative cohort, spontaneous cyst shrinkage occurred in 35% of patients with a median time to regression of 23.5 months.CONCLUSIONSPediatric RCCs are benign sellar lesions that often present with headaches. While cyst fenestration mitigates headaches in most patients, the majority of conservatively managed pediatric patients with RCCs will have spontaneous headache resolution. Furthermore, spontaneous RCC regression occurs in a substantial number of individuals. Thus, in the absence of optic compression, visual field deficit, or diagnostic uncertainty, many pediatric cases of RCC can be managed conservatively.

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The clinical course of Rathke’s cleft cysts in pediatric patients: impact on growth and pubertal development

Clinical Pediatric Endocrinology ◽

10.1297/cpe.2021-0034 ◽

2021 ◽

Author(s):

Yousuke Higuchi ◽

Kosei Hasegawa ◽

Toshihide Kubo ◽

Hiroyuki Tanaka ◽

Hirokazu Tsukahara

Keyword(s):

Pediatric Patients ◽

Clinical Course ◽

Pubertal Development ◽

Rathke's Cleft Cysts ◽

Rathke's Cleft

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Extended endoscopic endonasal approach for suprasellar Rathke’s cleft cysts

Journal of Clinical Neuroscience ◽

10.1016/j.jocn.2013.07.023 ◽

2014 ◽

Vol 21 (5) ◽

pp. 779-785 ◽

Cited By ~ 7

Author(s):

Arman Jahangiri ◽

Matthew Potts ◽

Sandeep Kunwar ◽

Lewis Blevins ◽

Ivan H. El-Sayed ◽

...

Keyword(s):

Endoscopic Endonasal Approach ◽

Endonasal Approach ◽

Endoscopic Endonasal ◽

Rathke's Cleft Cysts ◽

Rathke's Cleft

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Recurrent Rathke's Cleft Cysts: Incidence and Surgical Management in a Tertiary Pituitary Center over 2 Decades

Operative Neurosurgery ◽

10.1093/ons/opy258 ◽

2018 ◽

Vol 16 (6) ◽

pp. 675-684 ◽

Cited By ~ 3

Author(s):

Michelle A Wedemeyer ◽

Michelle Lin ◽

Vance L Fredrickson ◽

Anush Arakelyan ◽

Daniel Bradley ◽

...

Keyword(s):

Cerebrospinal Fluid ◽

Vision Loss ◽

Squamous Metaplasia ◽

Cerebrospinal Fluid Leak ◽

Pathological Examination ◽

Limited Data ◽

Rathke's Cleft Cysts ◽

Rathke's Cleft ◽

Fluid Leak

Abstract Background Limited data exist pertaining to outcomes following surgery for recurrent Rathke's cleft cysts (RCC). Objective To determine treatment outcomes in patients undergoing reoperation for recurrent or residual RCCs. Methods A retrospective analysis of 112 consecutive RCC operations in 109 patients between 1995 and 2017 was conducted. Results Eighteen patients underwent 21 RCC reoperations with a mean follow-up of 58 mo. Patient symptoms prior to reoperation included headaches (14, 66.7%) and vision loss (12, 57.1%). Thirteen of 18 patients (72.2%) required hormone supplementation prior to reoperation including 5 with diabetes insipidus (DI). Mean RCC diameter was 16 mm and 76% had suprasellar extension. Compared to index RCC cases, intraoperative cerebrospinal fluid leak repair was more common in reoperation cases (15/21, 71% vs 43/91, 47%, P = .05). There was 1 carotid artery injury without neurological sequelae, and 2 postoperative cerebrospinal fluid (CSF) leaks (9.5%). Rates of transient hyponatremia (3/10, 30% vs 4/91, 4.4%, P = .04) and transient DI (5/10, 50% vs 17/91, 18.7%, P = .04) were higher in the reoperation vs index group. Improved headaches and vision were reported in 4/12 (33%) and 8/12 (61.5%) of RCC reoperation patients, respectively. Two patients developed new permanent DI. A higher proportion of reoperation patients had RCC squamous metaplasia (24% vs 5.4%, P = .02) or wall inflammation (42.9% vs 2.2%, P < .001) on pathological examination. CONCLUSION Reoperation for RCCs is generally safe at tertiary pituitary centers and often results in improved vision. Hypopituitarism is less likely to improve following reoperation for recurrent RCCs. Several histopathological features may help characterize “atypical RCCs” with a higher likelihood of recurrence/progression.

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Rathke cleft cysts in pediatric patients: presentation, surgical management, and postoperative outcomes

Neurosurgical FOCUS ◽

10.3171/2011.5.focus1178 ◽

2011 ◽

Vol 31 (1) ◽

pp. E3 ◽

Cited By ~ 27

Author(s):

Arman Jahangiri ◽

Annette M. Molinaro ◽

Phiroz E. Tarapore ◽

Lewis Blevins ◽

Kurtis I. Auguste ◽

...

Keyword(s):

San Francisco ◽

Pediatric Patients ◽

Pediatric Population ◽

Growth Delay ◽

Common Symptom ◽

Imaging Findings ◽

Presenting Symptoms ◽

Sellar Lesions ◽

Kaplan Meier ◽

Study Results

Object Rathke cleft cysts (RCC) are benign sellar lesions most often found in adults, and more infrequently in children. They are generally asymptomatic but sometimes require surgical treatment through a transsphenoidal corridor. The purpose of this study was to compare adult versus pediatric cases of RCC. Methods The authors retrospectively reviewed presenting symptoms, MR imaging findings, laboratory study results, and pathological findings in 147 adult and 14 pediatric patients who underwent surgery for treatment of RCCs at the University of Californial at San Francisco between 1996 and 2008. Results In both the adult and pediatric groups, most patients were female (78% of adults, 79% of pediatric patients, p = 0.9). Headache was the most common symptom in both groups (reported by 50% of pediatric patients and 33% of adults, p = 0.2). Preoperative hypopituitarism occurred in 41% of adults and 45% of pediatric patients (p = 0.8). Growth delay, a uniquely pediatric finding, was a presenting sign in 29% of pediatric patients. Visual complaints were a presenting symptom in 16% of adult and 7% of pediatric patients (p = 0.4). There was no difference between median cyst size in adults versus pediatric patients (1.2 cm in both, p = 0.7). Temporary or permanent postoperative diabetes insipidus occurred in 12% of adults and 21% of pediatric patients (p = 0.4). Kaplan-Meier analysis revealed an 8% RCC recurrence rate at 2 years for each group (p = 0.5). Conclusions The incidence of RCCs is much lower in the pediatric population; however, symptoms, imaging findings, and outcomes are similar, suggesting that pediatric RCCs arise from growth of remnants of the embryonic Rathke pouch earlier in life than adult RCCs but do not differ in any other way. It is important to consider RCCs in the differential diagnosis when pediatric patients present with visual impairment, unexplained headache, or hypopituitarism including growth delay. Although the average RCC size was similar in our pediatric and adult patient groups, the smaller size of the pituitary gland in pediatric patients suggests an increased relative RCC size.

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

Journal of Neurological Surgery Part B Skull Base ◽

10.1055/s-0039-3402042 ◽

2019 ◽

Author(s):

Leopold Arko ◽

Jonathan C.M. Lee ◽

Saniya Godil ◽

Samuel Z. Hanz ◽

Vijay K. Anand ◽

...

Keyword(s):

Recurrence Rate ◽

Open Group ◽

Csf Leak ◽

Closure Rate ◽

Endoscopic Endonasal ◽

Closed Group ◽

Rathke's Cleft Cysts ◽

Watertight Closure ◽

Rathke's Cleft ◽

Csf Leaks

Abstract 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.

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The long-term recurrence of Rathke's cleft cysts as predicted by histology but not by surgical procedure

Journal of Neurosurgery ◽

10.3171/2015.8.jns151282 ◽

2016 ◽

Vol 125 (4) ◽

pp. 1002-1007 ◽

Cited By ~ 9

Author(s):

Yasuyuki Kinoshita ◽

Atsushi Tominaga ◽

Satoshi Usui ◽

Kazunori Arita ◽

Tetsuhiko Sakoguchi ◽

...

Keyword(s):

Surgical Procedure ◽

Squamous Metaplasia ◽

Visual Disturbance ◽

Initial Surgery ◽

Long Term Follow Up ◽

Rathke's Cleft Cysts ◽

Rathke's Cleft ◽

Term Monitoring

OBJECTIVE Patients with symptomatic Rathke's cleft cysts (RCCs) managed by surgical treatment often experience recurrence. The authors attempted to clarify the outcome of surgically treated RCCs over a long-term follow-up period. METHODS Ninety-one consecutive RCC patients with a follow-up period of more than 12 months (mean 80.2 months, range 12–297 months) were retrospectively studied. The authors examined the clinical features and postoperative course of patients who experienced a reaccumulation of cyst contents visible on MRI after the initial surgery, and they investigated data from the patients who underwent reoperation for symptomatic recurrent RCCs. RESULTS Reaccumulation of cyst contents occurred in 36 patients (39.6%). In 34 of these patients, a reaccumulation occurred in the first 5 years after surgery. The initial cysts in these patients were most often large, with squamous metaplasia in the cyst walls. Thirteen patients (14.3%) with recurrent symptoms underwent a reoperation, and 10 of the 13 patients had a reaccumulation of RCCs within the 1st year after surgery. The reoperations were performed in the 1st year (61.5%) or several years later (23.1%). Patients were likely to initially have had a visual disturbance and the cyst walls likely included squamous metaplasia. However, no association was observed between the incidence of reaccumulation/reoperation of RCCs and the surgical procedure for RCCs. CONCLUSIONS The reaccumulation rate of RCC is high in the long-term period, and it is associated with the histological findings but not with the surgical procedure. Long-term monitoring, for a period of at least 5 years, should therefore be conducted to identify and assess any RCC reaccumulation.

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