Purpose: We investigated the results of endoscopic fenestration for deeply located intracranial cysts (DLICs), risk factors for reoperation, and symptom improvement. Materials and Methods: We included 51 patients with DLICs who underwent endoscopic fenestration between November 2006 and October 2022. The median age was 5±20 years (6 days–67 years), and 36 (70.6%) patients were aged <20 years. The male-tofemale ratio was 1.3:1. The ventriculoscope was used to fenestrate the cysts, which had diameters under 4.5 mm. The volume of DLICs was measured separately on serial magnetic resonance imaging, and the patients were followed up for 32±40 months. Results: The mean preoperative volume of DLICs was 63.5±87.4 cm, 3 , which decreased to 23.7±56.2 cm 3postoperatively, with a 45.4%±32.1% decrease rate in 32 months. All DLICs were approached appropriately, avoiding the eloquent areas. Overall, 39 (76.5%) patients showed symptom improvement after a single operation, which was preserved without recurrence, whereas 12 (23.5%) underwent a second operation [shunting (17.6%) or repeating the endoscopic fenestration (5.9%)] owing to symptom aggravation and recurrent cysts. Patients aged <12 months showed 7.4 times more re-operation rate (p>0.046) and 7.4 times less symptom improvement (p=0.038) compared to those with older age. Females showed 6.5 times more re-operation rate (p=0.037) and 7.1 times less symptom improvement (p=0.027) than males. No patients experienced complications such as cerebrospinal fluid leakage, postoperative hemorrhage, or infection. Conclusion Endoscopic surgery is feasible for the treatment of DLICs. Female sex and age <12 months are risk factors for re-operation and less symptom improvement.

Purpose: There is few evidence regarding the optimal salvage treatment options for loco-reginal recurrence of esophageal cancer. This study aimed to evaluate the clinical outcomes of salvage radiotherapy (RT) in patients with loco-regional recurrence (LRR) after surgery for esophageal cancer. Materials and Methods: We retrospectively reviewed 147 esophageal cancer patients who received salvage RT for loco-regional recurrence between 1996 and December 2019. A total dose of 60 Gy in 20 fractions was used for RT alone and 60-70 Gy in 30-35 fractions for concurrent chemoradiotherapy (CCRT). Results: The patients’ median age was 65 years (range, 41 to 86 years). The median disease-free interval was 13.5 months (1.0 to 97.4 months). After a median 18.8 months follow-up, the 2-year overall survival (OS) and progression-free survival (PFS) rates were 38.1% and 25.9%, respectively. The median OS and PFS were 18.8 and 8.4 months, respectively. The CCRT could not improve OS compared to RT (p=0.336), but there was a trend of better PFS in the CCRT group. Regarding toxicities, the rate of grade 3 or higher toxicity was 10.9% occurring in 16 patients, and it was higher in patients who received CCRT than in the RT alone group (19.6% vs. 6.3%, p=0.023). Conclusion Salvage RT alone as well as CCRT could be effective in patients with locoregionally recurrent esophageal cancer.

Purpose: We investigated the results of endoscopic fenestration for deeply located intracranial cysts (DLICs), risk factors for reoperation, and symptom improvement. Materials and Methods: We included 51 patients with DLICs who underwent endoscopic fenestration between November 2006 and October 2022. The median age was 5±20 years (6 days–67 years), and 36 (70.6%) patients were aged <20 years. The male-tofemale ratio was 1.3:1. The ventriculoscope was used to fenestrate the cysts, which had diameters under 4.5 mm. The volume of DLICs was measured separately on serial magnetic resonance imaging, and the patients were followed up for 32±40 months. Results: The mean preoperative volume of DLICs was 63.5±87.4 cm, 3 , which decreased to 23.7±56.2 cm 3postoperatively, with a 45.4%±32.1% decrease rate in 32 months. All DLICs were approached appropriately, avoiding the eloquent areas. Overall, 39 (76.5%) patients showed symptom improvement after a single operation, which was preserved without recurrence, whereas 12 (23.5%) underwent a second operation [shunting (17.6%) or repeating the endoscopic fenestration (5.9%)] owing to symptom aggravation and recurrent cysts. Patients aged <12 months showed 7.4 times more re-operation rate (p>0.046) and 7.4 times less symptom improvement (p=0.038) compared to those with older age. Females showed 6.5 times more re-operation rate (p=0.037) and 7.1 times less symptom improvement (p=0.027) than males. No patients experienced complications such as cerebrospinal fluid leakage, postoperative hemorrhage, or infection. Conclusion Endoscopic surgery is feasible for the treatment of DLICs. Female sex and age <12 months are risk factors for re-operation and less symptom improvement.

Purpose: We investigated the results of endoscopic fenestration for deeply located intracranial cysts (DLICs), risk factors for reoperation, and symptom improvement. Materials and Methods: We included 51 patients with DLICs who underwent endoscopic fenestration between November 2006 and October 2022. The median age was 5±20 years (6 days–67 years), and 36 (70.6%) patients were aged <20 years. The male-tofemale ratio was 1.3:1. The ventriculoscope was used to fenestrate the cysts, which had diameters under 4.5 mm. The volume of DLICs was measured separately on serial magnetic resonance imaging, and the patients were followed up for 32±40 months. Results: The mean preoperative volume of DLICs was 63.5±87.4 cm, 3 , which decreased to 23.7±56.2 cm 3postoperatively, with a 45.4%±32.1% decrease rate in 32 months. All DLICs were approached appropriately, avoiding the eloquent areas. Overall, 39 (76.5%) patients showed symptom improvement after a single operation, which was preserved without recurrence, whereas 12 (23.5%) underwent a second operation [shunting (17.6%) or repeating the endoscopic fenestration (5.9%)] owing to symptom aggravation and recurrent cysts. Patients aged <12 months showed 7.4 times more re-operation rate (p>0.046) and 7.4 times less symptom improvement (p=0.038) compared to those with older age. Females showed 6.5 times more re-operation rate (p=0.037) and 7.1 times less symptom improvement (p=0.027) than males. No patients experienced complications such as cerebrospinal fluid leakage, postoperative hemorrhage, or infection. Conclusion Endoscopic surgery is feasible for the treatment of DLICs. Female sex and age <12 months are risk factors for re-operation and less symptom improvement.

Purpose: There is few evidence regarding the optimal salvage treatment options for loco-reginal recurrence of esophageal cancer. This study aimed to evaluate the clinical outcomes of salvage radiotherapy (RT) in patients with loco-regional recurrence (LRR) after surgery for esophageal cancer. Materials and Methods: We retrospectively reviewed 147 esophageal cancer patients who received salvage RT for loco-regional recurrence between 1996 and December 2019. A total dose of 60 Gy in 20 fractions was used for RT alone and 60-70 Gy in 30-35 fractions for concurrent chemoradiotherapy (CCRT). Results: The patients’ median age was 65 years (range, 41 to 86 years). The median disease-free interval was 13.5 months (1.0 to 97.4 months). After a median 18.8 months follow-up, the 2-year overall survival (OS) and progression-free survival (PFS) rates were 38.1% and 25.9%, respectively. The median OS and PFS were 18.8 and 8.4 months, respectively. The CCRT could not improve OS compared to RT (p=0.336), but there was a trend of better PFS in the CCRT group. Regarding toxicities, the rate of grade 3 or higher toxicity was 10.9% occurring in 16 patients, and it was higher in patients who received CCRT than in the RT alone group (19.6% vs. 6.3%, p=0.023). Conclusion Salvage RT alone as well as CCRT could be effective in patients with locoregionally recurrent esophageal cancer.

Purpose: We investigated the results of endoscopic fenestration for deeply located intracranial cysts (DLICs), risk factors for reoperation, and symptom improvement. Materials and Methods: We included 51 patients with DLICs who underwent endoscopic fenestration between November 2006 and October 2022. The median age was 5±20 years (6 days–67 years), and 36 (70.6%) patients were aged <20 years. The male-tofemale ratio was 1.3:1. The ventriculoscope was used to fenestrate the cysts, which had diameters under 4.5 mm. The volume of DLICs was measured separately on serial magnetic resonance imaging, and the patients were followed up for 32±40 months. Results: The mean preoperative volume of DLICs was 63.5±87.4 cm, 3 , which decreased to 23.7±56.2 cm 3postoperatively, with a 45.4%±32.1% decrease rate in 32 months. All DLICs were approached appropriately, avoiding the eloquent areas. Overall, 39 (76.5%) patients showed symptom improvement after a single operation, which was preserved without recurrence, whereas 12 (23.5%) underwent a second operation [shunting (17.6%) or repeating the endoscopic fenestration (5.9%)] owing to symptom aggravation and recurrent cysts. Patients aged <12 months showed 7.4 times more re-operation rate (p>0.046) and 7.4 times less symptom improvement (p=0.038) compared to those with older age. Females showed 6.5 times more re-operation rate (p=0.037) and 7.1 times less symptom improvement (p=0.027) than males. No patients experienced complications such as cerebrospinal fluid leakage, postoperative hemorrhage, or infection. Conclusion Endoscopic surgery is feasible for the treatment of DLICs. Female sex and age <12 months are risk factors for re-operation and less symptom improvement.

Purpose: There is few evidence regarding the optimal salvage treatment options for loco-reginal recurrence of esophageal cancer. This study aimed to evaluate the clinical outcomes of salvage radiotherapy (RT) in patients with loco-regional recurrence (LRR) after surgery for esophageal cancer. Materials and Methods: We retrospectively reviewed 147 esophageal cancer patients who received salvage RT for loco-regional recurrence between 1996 and December 2019. A total dose of 60 Gy in 20 fractions was used for RT alone and 60-70 Gy in 30-35 fractions for concurrent chemoradiotherapy (CCRT). Results: The patients’ median age was 65 years (range, 41 to 86 years). The median disease-free interval was 13.5 months (1.0 to 97.4 months). After a median 18.8 months follow-up, the 2-year overall survival (OS) and progression-free survival (PFS) rates were 38.1% and 25.9%, respectively. The median OS and PFS were 18.8 and 8.4 months, respectively. The CCRT could not improve OS compared to RT (p=0.336), but there was a trend of better PFS in the CCRT group. Regarding toxicities, the rate of grade 3 or higher toxicity was 10.9% occurring in 16 patients, and it was higher in patients who received CCRT than in the RT alone group (19.6% vs. 6.3%, p=0.023). Conclusion Salvage RT alone as well as CCRT could be effective in patients with locoregionally recurrent esophageal cancer.

Purpose: We investigated the results of endoscopic fenestration for deeply located intracranial cysts (DLICs), risk factors for reoperation, and symptom improvement. Materials and Methods: We included 51 patients with DLICs who underwent endoscopic fenestration between November 2006 and October 2022. The median age was 5±20 years (6 days–67 years), and 36 (70.6%) patients were aged <20 years. The male-tofemale ratio was 1.3:1. The ventriculoscope was used to fenestrate the cysts, which had diameters under 4.5 mm. The volume of DLICs was measured separately on serial magnetic resonance imaging, and the patients were followed up for 32±40 months. Results: The mean preoperative volume of DLICs was 63.5±87.4 cm, 3 , which decreased to 23.7±56.2 cm 3postoperatively, with a 45.4%±32.1% decrease rate in 32 months. All DLICs were approached appropriately, avoiding the eloquent areas. Overall, 39 (76.5%) patients showed symptom improvement after a single operation, which was preserved without recurrence, whereas 12 (23.5%) underwent a second operation [shunting (17.6%) or repeating the endoscopic fenestration (5.9%)] owing to symptom aggravation and recurrent cysts. Patients aged <12 months showed 7.4 times more re-operation rate (p>0.046) and 7.4 times less symptom improvement (p=0.038) compared to those with older age. Females showed 6.5 times more re-operation rate (p=0.037) and 7.1 times less symptom improvement (p=0.027) than males. No patients experienced complications such as cerebrospinal fluid leakage, postoperative hemorrhage, or infection. Conclusion Endoscopic surgery is feasible for the treatment of DLICs. Female sex and age <12 months are risk factors for re-operation and less symptom improvement.

This paper introduces the current status of Seoul National University Hospital Dementia Brain Bank (SNUH-DBB), focusing on the concordance rate between clinical diagnoses and postmortem neuropathological diagnoses. We detail SNUH-DBB operations, including protocols for specimen handling, induced pluripotent stem cells (iPSC) and cerebral organoids establishment from postmortem dural fibroblasts, and adult neural progenitor cell cultures. We assessed clinical-neuropathological diagnostic concordance rate. Between 2015 and September 2024, 162 brain specimens were collected via brain donation and autopsy. The median donor age was 73 years (1-94) with a male-to -female ratio of 2:1. The median postmortem interval was 9.5 hours (range: 2.5-65). Common neuropathological diagnoses included pure Lewy body disease (10.6%), Lewy body disease (LBD) with other brain diseases (10.6%), pure Alzheimer's disease-neuropathological change (ADNC) (6.0%), ADNC with other brain diseases (10.7%), vascular brain injury (15.2%), and primary age-related tauopathy (7.3%). APOE genotype distribution was following: ε3/ε3: 62.3%, ε2/ε3:9.6%, ε2/ε4: 3.4%, ε3/ε4: 24.0%, and ε4/ε4: 0.7%. Concordance rates between pathological and clinical diagnoses were: ADNC/AD at 42.4%; LBD at 59.0%; PSP at 100%; ALS at 85.7%; Huntington’s disease 100%. The varying concordance rates across different diseases emphasize the need for improved diagnostic criteria and biomarkers, particularly for AD and LBD. Tissues have been distributed to over 40 national studies. SNUH-DBB provides high-quality brain tissues and cell models for neuroscience research, operating under standardized procedures and international guidelines. It supports translational research in dementia and neurodegenerative diseases, potentially advancing diagnostic and therapeutic strategies.

Rare endocrine diseases are complex conditions that require lifelong specialized care due to their chronic nature and associated long-term complications. In Korea, a lack of nationwide data on clinical practice and outcomes has limited progress in patient care. Therefore, the Multicenter Networks for Ideal Outcomes of Pediatric Rare Endocrine and Metabolic Disease (OUTSPREAD) study was initiated. This study involves 30 centers across Korea. The study aims to improve the long-term prognosis of Korean patients with rare endocrine diseases by collecting comprehensive clinical data, biospecimens, and patient-reported outcomes to identify complications and unmet needs in patient care. Patients with childhood-onset pituitary, adrenal, or gonadal disorders, such as craniopharyngioma, congenital adrenal hyperplasia (CAH), and Turner syndrome were prioritized. The planned enrollment is 1,300 patients during the first study phase (2022–2024). Clinical, biochemical, and imaging data from diagnosis, treatment, and follow-up during 1980–2023 were retrospectively reviewed. For patients who agreed to participate in the prospective cohort, clinical data and biospecimens will be prospectively collected to discover ideal biomarkers that predict the effectiveness of disease control measures and prognosis. Patient-reported outcomes, including quality of life and depression scales, will be evaluated to assess psychosocial outcomes. Additionally, a substudy on CAH patients will develop a steroid hormone profiling method using liquid chromatography-tandem mass spectrometry to improve diagnosis and monitoring of treatment outcomes. This study will address unmet clinical needs by discovering ideal biomarkers, introducing evidence-based treatment guidelines, and ultimately improving long-term outcomes in the areas of rare endocrine and metabolic diseases.