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

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

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

2020 ◽  
Vol 11 ◽  
pp. 246
Author(s):  
Annelise Sprau ◽  
Anil Mahavadi ◽  
Michael Zhang ◽  
Micah Saste ◽  
Michael Deftos ◽  
...  
Keyword(s):  
Surgical Resection ◽  
Pediatric Patients ◽  
Pediatric Population ◽  
Squamous Metaplasia ◽  
Delayed Puberty ◽  
Endoscopic Endonasal ◽  
Sellar Lesions ◽  
Rathke's Cleft Cysts ◽  
Small Cohort ◽  
Rathke's Cleft

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.

Endonasal Endoscopic Surgery for Pediatric Sellar and Suprasellar Lesions: A Systematic Review and Meta-analysis

2020 ◽  
Vol 163 (2) ◽  
pp. 284-292
Author(s):  
Joshua A. Lee ◽  
Rebecca L. Cooper ◽  
Shaun A. Nguyen ◽  
Rodney J. Schlosser ◽  
David A. Gudis
Keyword(s):  
Systematic Review ◽  
Pituitary Adenomas ◽  
Pediatric Patients ◽  
Meta Analysis ◽  
Csf Leak ◽  
Cochrane Library ◽  
Management Approach ◽  
Tumor Type ◽  
Rathke's Cleft Cysts ◽  
Rathke's Cleft

Objectives The advent of endonasal endoscopic skull base surgery (ESBS) has redefined the management of pediatric sellar and suprasellar lesions. To date, the outcomes of these procedures have not been systematically reviewed. This study performed a systematic review with meta-analysis of surgical outcomes for pediatric patients undergoing ESBS for sellar and suprasellar lesions. Data Sources PubMed (National Library of Medicine, National Institutes of Health), Scopus (Elsevier), and Cochrane Library (Wiley). Review Methods Articles reporting on pediatric patients undergoing ESBS for craniopharyngiomas, pituitary adenomas, and Rathke’s cleft cysts were reviewed. The primary outcome was postoperative cerebrospinal fluid (CSF) leak. Secondary outcomes included endocrine, visual, and other complications. Results Twenty-five articles reporting on 554 patients were included. Overall postoperative CSF leak rate was 8.6%, with tumor-specific rates of 10.6% in craniopharyngiomas, 6.5% in pituitary adenomas, and 7.2% in Rathke’s cleft cysts ( P > .05). Older studies demonstrate higher postoperative CSF leak rates as compared with more recent studies (12.5% vs 6.1%, P = .0082). Younger children (8.9-12.6 years old) experienced a higher rate of postoperative CSF leaks as compared with older children (13.0-16.6 years old; 12.9% vs 4.9%, P = .0016). Additional postoperative complications included diabetes insipidus (26.7%), hypopituitarism (46.6%), visual deficits (2.6%), meningitis (3.4%), and weight gain (3.4%). Conclusion ESBS for pediatric sellar and suprasellar lesions is overall an effective management approach with an increasingly favorable risk-benefit profile. Younger children may be more susceptible to postoperative CSF leak as compared with older pediatric patients. Tumor type does not appear to be an independent risk factor for postoperative CSF leak in this population.

Treatment Outcomes of Rathke’s Cleft Cysts Managed with Marsupialization

2016 ◽  
Vol 77 (S 01) ◽  
Author(s):  
Edward Kuan ◽  
Frederick Yoo ◽  
Marvin Bergsneider ◽  
Marilene Wang
Keyword(s):  
Treatment Outcomes ◽  
Rathke's Cleft Cysts ◽  
Rathke's Cleft

Recurrence of Rathke’s Cleft Cysts Based on Gross Total Resection of Cyst Wall: A Meta-analysis

2020 ◽  
Author(s):  
Victor Lu ◽  
Avital Perry ◽  
Christopher Graffeo ◽  
Krishnan Ravindran ◽  
Jamie Van Gompel
Keyword(s):  
Cyst Wall ◽  
Meta Analysis ◽  
Gross Total Resection ◽  
Total Resection ◽  
Rathke's Cleft Cysts ◽  
Rathke's Cleft

Clinico-pathological differentiation of cystic craniopharyngiomas from Rathke's cleft cysts

2006 ◽  
Vol 114 (S 1) ◽  
Author(s):  
B Hofmann ◽  
J Kreutzer ◽  
W Saeger ◽  
I Blümcke ◽  
R Fahlbusch ◽  
...  
Keyword(s):  
Pathological Differentiation ◽  
Rathke's Cleft Cysts ◽  
Rathke's Cleft

Stereotactic radiosurgery to treat presumed Rathke's cleft cysts

2012 ◽  
Vol 26 (5) ◽  
pp. 684-691 ◽  
Author(s):  
Xin Yu ◽  
Runsheng Huang ◽  
Wei Qian ◽  
Jingyu Fang ◽  
Caizhen Wu ◽  
...  
Keyword(s):  
Stereotactic Radiosurgery ◽  
Rathke's Cleft Cysts ◽  
Rathke's Cleft

An isolated primary Rathke's cleft cyst in the cerebellopontine angle

2014 ◽  
Vol 121 (4) ◽  
pp. 846-850 ◽  
Author(s):  
Jun Fan ◽  
Songtao Qi ◽  
Yuping Peng ◽  
Xi-an Zhang ◽  
Binghui Qiu ◽  
...  
Keyword(s):  
Cerebellopontine Angle ◽  
Imaging Features ◽  
Good Recovery ◽  
Suprasellar Region ◽  
Adjacent Structures ◽  
Facial Numbness ◽  
History Of ◽  
Rathke's Cleft Cysts ◽  
Rathke's Cleft

Rathke's cleft cysts (RCCs) are benign cysts typically located in the sellar or suprasellar region; ectopic isolated lesions are extremely rare. The authors describe the case of a 25-year-old man with a giant symptomatic RCC arising primarily at the cerebellopontine angle (CPA), only the second case reported thus far. The patient presented with a 2-year history of right hearing impairment and tinnitus accompanied by vertigo and headache and a 2-week history of right facial numbness. Subsequently, he underwent total cyst removal via retrosigmoid craniotomy with a good recovery. He experienced no recurrence during a 64-month follow-up period. The possible pathogenesis, differential diagnosis, and surgical treatment of such cysts are discussed in this article. Isolated ectopic RCCs can arise from the ectopic migration of Rathke's pouch cells during the embryonic period. It is still difficult to distinguish ectopic RCCs from other cystic lesions of the CPA given the lack of specific imaging features. Aggressive resection of the cyst wall is not recommended, except when lesions do not closely adhere to adjacent structures.

The clinical course and role of surgery in pediatric malignant peripheral nerve sheath tumors: a database study

2021 ◽  
pp. 1-8
Author(s):  
Victor M. Lu ◽  
Shelly Wang ◽  
David J. Daniels ◽  
Robert J. Spinner ◽  
Allan D. Levi ◽  
...  
Keyword(s):  
Overall Survival ◽  
Radiation Therapy ◽  
Peripheral Nerve ◽  
Pediatric Patients ◽  
Clinical Course ◽  
The Body ◽  
National Database ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

OBJECTIVE Malignant peripheral nerve sheath tumors (MPNSTs) are rare tumors found throughout the body, with their clinical course in children still not completely understood. Correspondingly, this study aimed to determine survival outcomes and specific clinical predictors of survival in this population from a large national database. METHODS All patients with MPNSTs aged ≤ 18 years in the US National Cancer Database (NCDB) between 2005 and 2016 were retrospectively reviewed. Data were summarized, and overall survival was modeled using Kaplan-Meier and Cox regression analyses. RESULTS A total of 251 pediatric patients with MPNSTs (132 [53%] females and 119 [47%] males) were identified; the mean age at diagnosis was 13.1 years (range 1–18 years). There were 84 (33%) MPNSTs located in the extremities, 127 (51%) were smaller than 1 cm, and 22 (9%) had metastasis at the time of diagnosis. In terms of treatment, surgery was pursued in 187 patients (74%), chemotherapy in 116 patients (46%), and radiation therapy in 129 patients (61%). The 5-year overall survival rate was estimated at 52% (95% CI 45%–59%), with a median survival of 64 months (range 36–136 months). Multivariate regression revealed that older age (HR 1.10, p < 0.01), metastases at the time of diagnosis (HR 2.14, p = 0.01), and undergoing biopsy only (HR 2.98, p < 0.01) significantly and independently predicted a shorter overall survival. Chemotherapy and radiation therapy were not statistically significant. CONCLUSIONS In this study, the authors found that older patient age, tumor metastases at the time of diagnosis, and undergoing only biopsy significantly and independently predicted poorer outcomes. Only approximately half of patients survived to 5 years. These results have shown a clear survival benefit in pursuing maximal safe resection in pediatric patients with MPNSTs. As such, judicious workup with meticulous resection by an expert team should be considered the standard of care for these tumors in children.

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