Features of Remyelination after Transplantation of Olfactory Ensheathing Cells with Neurotrophic Factors into Spinal Cord Cysts.

This paper shows for the first time that co-transplantation of human olfactory ensheathing cells with neurotrophin-3 into spinal cord cysts is more effective for activation of remyelination than transplantation of cells with brain-derived neurotrophic factor and a combination of these two factors. The studied neurotrophic factors do not affect proliferation and migration of ensheathing cells in vitro. It can be concluded that the maximum improvement of motor function in rats receiving ensheathing cells with neurotrophin-3 is largely determined by activation of remyelination.

Rathke's Cleft Cyst and Craniopharyngioma: A Continuum of the Same Spectrum? Insights from an Interesting Case and Previous Literature.

Rathke's Cleft Cysts (RCCs) and Craniopharyngiomas (CPs) may represent disease entities on the same etio-pathological spectrum. We report the case of a 36-year-old female presenting with vision loss and menstrual irregularities, imaging shows a predominantly cystic lesion in the sellar region with suprasellar extension. She underwent a microscopic transnasal resection of the lesion. She later presented with recurrent symptoms and increased residual lesion size on imaging, a transcranial excision of the lesion was performed. Histopathology from the initial operative specimen revealed RCC with squamous metaplasia which was BRAF negative, while the specimen from the second surgery revealed BRAF positive papillary stratified squamous architecture suggestive of Papillary CP. This case adds to the evidence that both RCCs and papillary CPs may be the spectrum of the same disease. Further, papillary CPs may be an evolution from the RCCs.

Long-term outcome of surgically treated and conservatively managed Rathke cleft cysts.

OBJECTIVE: Rathke cleft cysts (RCC) are benign lesions of the sellar region that require surgical treatment in case of visual deterioration or progression of the cyst. However, the natural course is often stable and asymptomatic. We aimed to investigate the characteristics of patients with cyst progression during follow-up (FU) and to compare the natural history of patients with RCC with patients who underwent surgery. METHODS: Patients with an MR morphologic cystic sellar lesion classified as RCC between 04/2001 and 11/2020 were included. Functional outcomes, including ophthalmologic, endocrinologic, and MRI data, were retrospectively analyzed and compared between surgically treated patients, patients on a "watch and wait" strategy (WWS), and patients on a WWS who underwent secondary surgery due to cyst progression. RESULTS: One hundred forty patients (median age 42.8 years) with RCC on MRI were identified. 52/140 (37.1%) underwent primary surgery. Of 88 patients (62.9%) with initial WWS, 21 (23.9%) underwent surgery for secondary cyst progression. Patients on the WWS had significantly smaller cyst volumes (p = 0.0001) and fewer visual disturbances (p = 0.0004), but a similar rate of hormone deficiencies (p = 0.99) compared with surgically treated patients preoperatively. Postoperatively patients suffered significantly more often from hormone deficiencies than WWS patients (p = 0.001). Patients who switched to the surgical group were significantly more likely to have preoperative T1 hyperintense signals on MRI (p = 0.0001) and visual disturbances (p = 0.001) than patients with continuous WWS. Postoperatively, these patients suffered more frequently from new hormonal deficiencies (p = 0.001). Endocrine and ophthalmologic outcomes in patients with primary and secondary surgery were comparable. Multivariate analysis showed that WWS patients were at a higher risk of requiring surgery for cyst progression when perimetric deficits (p = 0.006), hyperprolactinemia (p = 0.003), and corticotropic deficits (p = 0.005) were present. CONCLUSION: Surgical treatment of RCC may cause new hormonal deficiencies, which are rare in the natural course. Therefore, the indication for surgery should be carefully evaluated. Hyperprolactinemia and corticotropic deficits were significant indicators for a secondary cyst progression in patients with RCC. However, a significant amount of almost 25% of initially conservatively managed cysts showed deterioration, necessary for surgical intervention.

Spontaneous and asymptomatic rupture of an RCC with resolution of symptoms.

There is no standard of care for management of Rathke cleft cysts (RCCs), and protocol for spontaneous rupture or residual capsule fragments is not well documented.Our case involves a Caucasian man in his 80s who presented with muscle weakness, fatigue, bitemporal hemianopia and pain. Further examination demonstrated decreased thyroid and cortisol levels. MRI revealed a 1.6×1.5×1.3 cm sellar homogenous mass with extension into the suprasellar cistern. While the size of the cyst was rather large, a decision was made to follow conservatively with serial MRI. At 3 years, the mass had spontaneously regressed. The patient was asymptomatic without imaging evidence of RCC recurrence at 4-year follow-up.Classic indications for surgical intervention in suprasellar cysts were subtle in our patient and his advanced age made us take a conservative approach. Current data are lacking regarding management of RCCs presenting with endocrine dysfunction. Our case suggests that RCCs presenting with endocrine dysfunction may be managed conservatively with serial imaging-based monitoring.

Microsurgical Resection of a Pineal Cyst via a Paramedian Supracerebellar Infratentorial Approach.

Pineal cysts are typically detected in around 1.3% to 4.3% of patients during routine magnetic resonance imaging (MRI) scans.1,2 The vast majority of pineal cysts are benign, asymptomatic, and typically do not necessitate surgical intervention. Large pineal cysts are known to cause hydrocephalus with its associated symptoms and thus can require in rare cases surgical resection. Even in the absence of hydrocephalus, selected patients with large pineal cysts causing headaches and visual disturbances can find relief after surgical resection.3,4 The supracerebellar infratentorial (SCIT) approach is widely used and represents an extraparenchymatous approach through a natural corridor to the pineal region.5 Performing this approach in a semisitting position allows for an optimal retraction of the cerebellum by gravity. We employ a minimally invasive paramedian SCIT approach for the resection of pineal cysts. In our experience, the paramedian SCIT approach allows for a less steep operating angle and a smaller craniotomy compared with the midline SCIT approach. We present a 24-year-old female complaining of headache. The initial MRI was conducted 2 years before surgery. Following the initial evaluation, the patient experienced progressive headaches without neurologic deficits. A subsequent MRI revealed enlargement of the pineal cyst, leading to the indication for surgical resection. The surgery was performed mainly under the operating microscope with endoscopic visualization in suitable situations as our small approach restricts bimanual dissection with an endoscope. In our experience, this approach provides a versatile and minimally invasive access to the pineal region, making it optimally suitable for pineal cysts requiring surgical resection.

Long-term outcomes and potential predictive recurrence factors after endonasal endoscopic surgical treatment of symptomatic Rathke's cleft cysts.

Although patients with symptomatic Rathke's cleft cysts (RCCs) receive surgical treatment, recurrence sometimes occurs after surgery. However, the mechanism underlying recurrence remains unclear. We evaluated the outcomes of RCC decompression over a long-term follow-up period. We retrospectively reviewed the medical records of 35 patients with symptomatic RCC who underwent endonasal endoscopic surgery (EES) at our institution between 2008 and 2023. Patients' characteristics, intraoperative findings, and postoperative follow-up outcomes were evaluated. A univariate regression model was used to identify the predictors of recurrence. The median patient age was 48.0 years, and 74.2% of the patients were female. The mean follow-up duration was 94.7 ± 47.6 months. Cyst content recurrence was observed in 15 patients (42.8%). Five patients (14.2%) with symptomatic recurrence underwent reoperation. Postoperative vision improved in all 23 patients (100%); headaches improved in 20 patients (90.9%). A new hormonal deficit occurred in two patients (5.7%). Complications included intraoperative cerebrospinal fluid (CSF) leak in 10 patients (28.5%), postoperative CSF leak in two patients (5.7%), permanent diabetes insipidus in two patients (5.7%), and postoperative infection in three patients (8.5%). Univariate analyses revealed that the position of the anterior pituitary lobe (p = 0.019) and preoperative visual disturbances (p = 0.008) significantly affected recurrence after surgery. Although EES was efficient, the recurrence rate was relatively high over a long-term period. The anterior pituitary lobe position and preoperative visual disturbances were significantly associated with recurrence. The anterior-inferior position can predict a high risk of recurrence before surgery.

Rathke's cleft cyst apoplexy in a boy treated by endoscopic endonasal surgery: case report and literature review.

Rathke's cleft cyst (RCC) apoplexy is an uncommon lesion attributed to abnormal vascular supply to the fragile RCC epithelial wall. It is rare in children and very difficult to diagnose without pathologic confirmation. Here, we report an 8-year-old boy who presented with headache and visual deficit. MRI and CT showed a cystic mass in the sellar region. He underwent endoscopic endonasal surgery, and the cystic mass was resected completely via a trans-sphenoidal approach. The lesion was confirmed as RCC apoplexy by intraoperative observation and histopathological examination. Headache was completely relieved and the visual field deficit improved remarkably after the operation. The authors recommend surgical management for pediatric RCC apoplexy patients who present with severe neuro-ophthalmic signs or deterioration of consciousness, although there are at present no standardized management guidelines for pediatric RCC apoplexy.

Measurement of the intersiphon distance for normal skull base development and estimation of the surgical window for the endoscopic transtuberculum approach in children.

OBJECTIVE: Due to the underdeveloped skull base in children, it is crucial to predict whether a sufficient surgical window for an endoscopic endonasal approach can be achieved. This study aimed to analyze the presumed surgical window through measurement of the intersiphon distance (ISD) and the planum-sella height (PSH) on the basis of age and its correlation with the actual surgical window for the endoscopic transtuberculum approach. METHODS: Twenty patients of each age from 3 to 18 years were included as the normal skull base population. ISD and PSH were measured and compared among consecutive ages. Additionally, 42 children with craniopharyngiomas or Rathke's cleft cysts who underwent treatment via the endoscopic transtuberculum approach were included. ISD and PSH were measured on preoperative images and then correlated with the dimensions of the surgical window on postoperative CT scans. The intraoperative endoscopic view was classified as narrow, intermediate, or wide based on operative photographs or videos, and relevant clinical factors were analyzed. RESULTS: In the normal skull base population, both ISD and the estimated area of the surgical window increased with age, particularly at 8 and 11 years old. On the other hand, PSH did not show an incremental pattern with age. Among the 42 children who underwent surgery, 24 had craniopharyngioma and 18 had Rathke's cleft cysts. ISD showed the strongest correlation with the actual area of the surgical window [r(40) = 0.69, p < 0.001] rather than with age or PSH. The visual grade of the intraoperative endoscopic view was narrow in 17 patients, intermediate in 21, and wide in 4. Preoperative ISD was 14.58 ± 1.29 mm in the narrow group, 16.13 ± 2.30 mm in the intermediate group, and 18.09 ± 3.43 mm in the wide group (p < 0.01). There were no differences in terms of extent of resection (p = 0.41); however, 2 patients in the narrow group had postoperative complications. CONCLUSIONS: Normal skull base development exhibited age-related growth. However, in children with suprasellar lesions, the measurement of the ISD showed a better correlation than age for predicting the surgical window for the endoscopic transtuberculum approach. Children with a small ISD should be approached with caution due to the limited surgical window.

Surgical management of Rathke cleft cysts in pediatric patients: a single institution experience.

OBJECTIVE: Rathke cleft cysts (RCCs) are benign, epithelial-lined sellar lesions that arise from remnants of the craniopharyngeal duct. Due to their rarity in the pediatric population, data are limited regarding the natural history and optimal management of growing or symptomatic RCCs. We present our institutional experience with the surgical management of RCCs. METHODS: We performed a retrospective study of consecutive RCC patients ≤ 18 years old treated surgically at our institution between 2006 and 2022. RESULTS: Overall, 567 patients with a diagnosis of pituitary mass or cyst were identified. Of these, 31 had a histopathological diagnosis of RCC, 58% female and 42% male. The mean age was 13.2 ± 4.2 years. Presenting symptoms included headache (58%), visual changes (32%), and endocrinopathies or growth delay (26%); 13% were identified incidentally and subsequently demonstrated growth on serial imaging. Six percent presented with symptomatic intralesional hemorrhage. Surgical approach was transsphenoidal for 90% of patients and orbitozygomatic for 10%. Preoperative headaches resolved in 61% of patients and preoperative visual deficits improvement in 55% after surgery. New pituitary axis deficits were seen in 9.7% of patients. Only two complications occurred from a first-time surgery: one cerebrospinal fluid leak requiring lumbar drain placement, and one case of epistaxis requiring cauterization. No patients experienced new visual or neurological deficits. Patients were followed postoperatively with serial imaging at a mean follow-up was 62.9 ± 58.4 months. Recurrence requiring reoperation occurred in 32% of patients. Five-year progression-free survival was 47.9%. Except for one patient with multiple neurological deficits from a concurrent tectal glioma, all patients had a modified Rankin Scale score of 0 or 1 (good outcome) at last follow-up. CONCLUSION: Due to their secretory epithelium, pediatric RCCs may demonstrate rapid growth and can cause symptoms due to local mass effect. Surgical management of symptomatic or growing pediatric RCCs via cyst fenestration or partial resection of the cyst wall can be performed safely, with good neurologic outcomes. There is a nontrivial risk of endocrinologic injury, and long-term follow up is needed due to high recurrence rates.

Endoscopic endonasal surgery of Rathke's cleft cysts-- preoperative imaging evaluation, personalized removal and multilevel sellar floor reconstruction.

OBJECTIVES: The purpose of this study was to evaluate the effectiveness of endoscopic endonasal surgery (EES) for Rathke's cleft cysts (RCCs) and the advantages of detailed preoperative imaging evaluation, intraoperative personalized removal and multilevel sellar floor reconstruction. METHODS: The clinical data of 43 patients with RCCs who were treated by EES in the neurosurgery department of affiliated hospital of Jiangnan University and Wuxi No.2 People's Hospital from January 2018 to January 2023 were retrospectively analyzed. The effectiveness of EES for RCCs was analyzed by imaging information, surgical procedures, symptom improvement and complications. RESULTS: All 43 RCCs were completely removed by EES, and all clinical symptoms improved to varying degrees. Postoperative relief of headache was achieved in 23 out of 26 patients (88.5 %); there was improvement in 10 out of 13 patients with visual field disorders (76.9 %) and in 8 out of 10 patients with endocrine abnormalities (80 %). New hormonal deficiency was discovered in 7 of all the patients postoperatively. There were 8 patients with postoperative diabetes insipidus and 1 patient with cerebrospinal fluid leakage. The incidence of new hormonal dysfunction and postoperative DI in expanded EES (33.3 %, 33.3 %) was higher than it in conventional EES (4 %, 8 %) (P < 0.05). The average follow-up time was 29.1 ± 14.8 months, and there were no deaths or infections. Three patients presented with cyst recurrence on MRI. CONCLUSIONS: The clinical manifestations and imaging characteristics of RCCs are variable, and a detailed preoperative review of the imaging is helpful for the development of surgical plans. RCCs can be treated more safely and thoroughly with less trauma and complications by intraoperative personalized removal and multilevel sellar floor reconstruction. The high incidence of new hormonal dysfunction and postoperative DI may be related to the disturbance of the pituitary stalk. EES has unique advantages and high clinical application value for the treatment of RCCs.

Rathke's cyst with xanthogranulomatous change or chronic cystic craniopharingioma? A rare case.

A 9-year-old girl presented to our neurosurgery clinic complaining of visual disturbances for a week. Magnetic resonance imaging showed an extensive sellar lesion with suprasellar involvement and compression of the optic chiasm. Based on these findings, a cystic craniopharyngioma, a pituitary macroadenoma and - less likely - a Rathke's cleft cyst were considered as differential diagnoses. In view of the progressive loss of vision, the parents agreed to resection of the lesion through an endoscopic endonasal transtubercular approach, with the aim of a gross total resection. Microscopic examination revealed that the lesion was cystic, surrounded by an epithelium that was partly composed of columnar ciliated cells with interspersed mucous cells and partly had a flattened appearance. The observed findings were complex to interpret: if, on the one hand, the clinical-surgical and neuroradiologic data suggested a craniopharyngioma, this hypothesis was not supported by the microscopic data, because the presence of columnar ciliated epithelium associated with mucous cells was a microscopic feature inconsistent with a craniopharyngioma and was instead consistent with a Rathke's cleft cyst, a histologic diagnosis that was made. The incidence of Rathke's cleft cyst, which mimics clinical and neuroradiologic aspects of craniopharyngiomas, is extremely unusual, as only 2 cases have been described in the literature.

Intraoperative ultrasound-assisted endoscopic endonasal transclival marsupialization of an ectopic retrosellar Rathke's cleft cyst: A rare case illustration and systematic review of the literature.

Not every Rathke's cleft cyst (RCC) is confined within the sella between the posterior and anterior lobes of the pituitary gland. Intracranial ectopic RCCs are extremely rare, with only seven cases reported in the literature. In this study, the authors presented a rare case of a symptomatic ectopic retrosellar RCC posterior to the pituitary gland, causing extensive clival erosion. The surgical nuances of the wide marsupialization of the cyst through intraoperative ultrasound-assisted endoscopic endonasal transclival approach are described, and a systematic literature review of intracranial ectopic RCCs is conducted.

Intrasellar Dermoid Cyst: Case Report of a Rare Lesion and Systematic Literature Review Comparing Intrasellar, Suprasellar, and Parasellar Locations.

OBJECTIVE: Intracranial dermoid cyst (DC) is a rare benign, slow-growing lesion, most commonly arising along the midline. They can occur in the supratentorial compartment, very rarely involve the sellar region and only exceptionally are intrasellar. The aim of our study is to address the challenges in the diagnosis and management of sellar DCs. METHODS: We performed a systematic review of sellar DCs, in keeping with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, and described an intrasellar DC in a 32-year-old female who presented with bilateral blurring vision. RESULTS: The review identified 4 intrasellar, 29 suprasellar, and 28 parasellar cases. Intrasellar DCs more likely present with progressive visual impairment and pituitary hormone dysfunctions during the fifth decade of life. Suprasellar and parasellar DCs are typically diagnosed during the third decade of life because of diplopia, ptosis, trigeminal hypoaesthesia/para-esthesia or cyst's rupture. Sellar DCs are typically hypodense on computed tomography scans and contain calcifications. Magnetic resonance imaging features include T1 hyperintensity, T2 heterogeneous intensity, no restriction on diffusion-weighted images, and no contrast enhancement. Surgery is the treatment of choice. Gross total resection is achieved in 60% of intrasellar and 61.9% of suprasellar and parasellar DCs. Early postoperative complications are reported in 40.0%, 16.7%, and 23.8% of intrasellar, suprasellar, and parasellar DCs, respectively. CONCLUSIONS: Intrasellar DCs are rare lesions typically diagnosed later than suprasellar and parasellar DCs due to their different clinical presentations. However, they should be considered in the differential diagnosis of cystic lesions of the sella, including epidermoid cysts, craniopharyngiomas, Rathke's cleft cysts, and teratomas.

Endoscopic endonasal marsupialization of rathke cleft cysts: clinical outcomes and risk factors analysis of visual impairment, pituitary dysfunction, and CSF leak.

OBJECTIVE: This paper assesses the clinical and imaging characteristics, histopathological findings, and treatment outcomes of patients with Rathke's cleft cyst (RCC), as well as identifies potential risk factors for preoperative visual and pituitary dysfunction, intraoperative cerebrospinal fluid (CSF) leak, and recurrence. Through analyzing these factors, the study aims to contribute to the current understanding of the management of RCCs and identify opportunities for improving patient outcomes. METHODS: We performed a retrospective analysis of 45 RCC patients between ages 18-80 treated by Endoscopic Endonasal Approach (EEA) and cyst marsupialization between 2010 and 2022 at a single institution. RESULTS: The median patient age was 34, and 73% were female. The mean follow-up was 70 ± 43 months. Preoperative visual impairment correlated with cyst diameter (OR = 1.41, 95% CI = 1.07 to 1.85, p-value = 0.01) and older age (OR = 1.06, 95% CI = 1.01 to 1.11, p-value = 0.02). Intraoperative CSF leaks were 11 times more likely for cysts ≥ 2 cm (OR = 11.3, 95% CI = 1.25 to 97.37, p-value = 0.03), with the odds of leakage doubling for every 0.1 cm increase in cyst size (OR = 1.41, 95% CI = 1.08 to 1.84, p-value = 0.01). Preoperative RCC appearing hypointense on T1 images demonstrated significantly higher CSF leak rates than hyperintense lesions (OR = 122.88, 95% CI = 1.5 to 10077.54, p-value = 0.03). Preoperative pituitary hypofunction was significantly more likely in patients with the presence of inflammation on histopathology (OR = 20.53, 95% CI = 2.20 to 191.45, p-value = 0.008 ) and T2 hyperintensity on magnetic resonance imaging (MRI) sequences (OR = 23.2, 95% CI = 2.56 to 211.02, p-value = 0.005). Notably, except for the hyperprolactinemia, no postoperative improvement was observed in pituitary function. CONCLUSION: Carefully considering risk factors, surgeons can appropriately counsel patients and deliver expectations for complications and long-term results. In contrast to preoperative visual impairment, preoperative pituitary dysfunction was found to have the least improvement post-surgery. It was the most significant permanent complication, with our data indicating the link to the cyst signal intensity on T2 MR and inflammation on histopathology. Earlier surgical intervention might improve the preservation of pituitary function.

Symptomatic intraventricular choroid plexus cysts. Illustrative case and systematic review.

Although choroid plexus cysts are a frequent incidental neuroimaging finding, symptomatic ones are rare-a series of more than five cases are hard to find. In the absence of high-volume studies, there are no generally accepted algorithms for diagnosis and treatment for this pathology. Proposed surgical techniques include microsurgical excision or fenestration and endoscopic excision or fenestration with or without additional shunting. No definitive conclusions exist about the superiority of a certain technique. Here, we introduce an illustrative case of a patient with a symptomatic choroid plexus cyst in the trigone of the lateral ventricle and a systematic review of 65 additional published cases with the aim of identifying epidemiological features, variants of localization of the cysts, their symptoms, persistence of concomitant obstructive hydrocephalus, and treatment modalities. A PRISMA-based literature search was performed on the PubMed, MEDLINE, Scopus, and Web of Knowledge databases. We include in the review case reports and case series of symptomatic choroid plexus cysts with full texts or valuable abstracts available online in English and published by April 2023. All abstracts of retrieved studies were assessed by two independent researchers to avoid bias. Only descriptive statistics were used for the presentation of the results. A total of 48 studies (39 case reports and 9 case series) with 65 depicted cases met the eligibility criteria. The review showed a slight predominance of choroid plexus cysts in men. The most common localizations of cysts were the trigone and the body of the lateral ventricle. Obstructive hydrocephalus is often present in patients with choroid plexus cysts. The most common symptoms of cysts were signs of increased ICP: headaches and vomiting. The main treatment approaches for symptomatic choroid plexus cysts were microsurgical excision, microsurgical fenestration, endoscopic fenestration, and total endoscopic excision. The tendency has been noted to shift from microsurgical to endoscopic procedures over the past two decades. Some data on the classification of cysts of the central nervous system and the underlying mechanisms of the pathogenesis of choroid plexus cysts are also presented.Although symptomatic cases of choroid plexus cysts are rare, by summarizing currently available data, one could clarify their common features and identify a preferable treatment modality.

Pineal cysts in children: a paediatric series treated over the last twenty years in Lyon.

BACKGROUND: Pineal cysts are a rare lesion of the pineal gland. Pineal cysts are benign lesions, generally asymptomatic, and are usually an incidental discovery on MRI performed for other problems. The management of pineal cysts in children remains a matter for debate. Here, we report our own retrospective paediatric cases that have been surgically treated and review the paediatric literature on this topic. METHODS: This is a retrospective monocentric study. All patients operated by the senior author (CM) for a benign pineal cyst from 2000 to 2021 were included. All other pineal region cystic lesions were excluded. Medical and surgical data were extracted from the hospital medical database. RESULTS: Twelve patients were included. The clinical symptomatology was characterized by headaches in seven patients, visual troubles in two patients, precocious puberty in one patient, signs of intracranial hypertension in two patients, seizures associated with headache in one patient, and headaches associated with behavioural troubles in another patient. No major post-operative complications were observed in this series. It is to noted that surgery was performed because a suspicion of a true pineal parenchymal tumour has been made. Histopathological study came back with the diagnosis of pineal cyst. CONCLUSIONS: Pineal cyst is rare. If the radiological diagnosis is clear, no surgery is advocated except in cases associated with hydrocephalus and rapid growth. In case of a suspicion of a true pineal parenchymal tumour, a surgery may be needed to confirm the diagnosis. Lastly, we stress that only cystic lesions of the pineal gland itself should be considered as pineal cyst.

Surgical indication of pediatric Rathke's cleft cyst based on a 20-year retrospective cohort.

OBJECTIVE: Rathke's cleft cyst (RCC) is the most commonly encountered pituitary incidentaloma in children. Because RCC is not frequently diagnosed in children, there are few reports on pediatric RCCs. The natural course of the disease and appropriate treatments are still obscure. The present study aimed to elucidate the natural history and surgical indications of RCCs in children. METHODS: The authors retrospectively reviewed the clinical presentations, imaging features, ophthalmological evaluations, endocrine evaluations, and surgical outcomes of pediatric RCCs at a single institution from January 2000 to October 2022. Clinical outcomes between the surgery and observation groups were compared. RESULTS: Among 93 patients, there were 41 patients in the surgery group and 52 patients in the observation group. The mean age at diagnosis was 10.9 years, and the mean follow-up period was 5.6 years. Headache fully or partially improved after surgery (86.2%), but the rate of improvement was not different from that of the observation group (70.0%). Ophthalmological abnormalities were effectively improved by surgical treatment (93.3%). Both the improvement and deterioration rates of endocrine abnormalities were significantly higher in the surgery group (p = 0.026 and p < 0.001, respectively), but the deterioration rate (43.9%) was higher than the improvement rate (14.6%). In the surgery group, the recurrence rate was 17.1% and the reoperation rate was 4.9%. Compared with total cyst wall resection, cyst fenestration with partial wall resection was associated with a higher recurrence rate (26.9%, p = 0.035) but a lower rate of endocrine abnormalities (30.8%, p = 0.049). CONCLUSIONS: Pediatric RCCs of ≥ 10 mm in size were analyzed. Ophthalmological abnormalities are the major surgical indications for pediatric RCCs. Headache and partial endocrine abnormalities may be improved with surgery, but they are not absolute indications for surgery. Cyst fenestration with partial wall resection via an endoscopic endonasal approach is the most recommended surgical method. Follow-up is essential to monitor for the occurrence of visual field defects and the recurrence of cysts.