Background: We aimed to evaluate long-term outcomes of gamma knife radiosurgery (GKS) for cerebral cavernous malformations (CCMs). Methods: Among the 233 CCM patients who underwent GKS, 79 adult patients (96 lesions) followed for over 10 years were included and analyzed retrospectively. Annual hemorrhage rate (AHR) was analyzed the entire cohort of 233 patients and the subset of 79 enrolled patients by dividing lesions into overall CCM lesions and brainstem lesions. AHR, neurologic outcome, adverse radiation effect (ARE), and changes of lesions in magnetic resonance imaging (MRI) were compared before and after GKS. Cox-regression analysis was performed to identify risk factors for hemorrhage following GKS. Results: Mean follow-up duration of 79 enrolled patients was 14 years (range, 10–23 years).The AHR of all CCMs for entire cohort at each time point was 17.8% (pre-GKS), 5.9% (≤ 2 years post-GKS), 1.8% (≤ 10 years post-GKS). The AHR of all CCM for 79 enrolled patients was 21.4% (pre-GKS), 3.8% (2 years post-GKS), 1.4% (10 years post-GKS), and 2.3% (> 10 years post-GKS). The AHR of brainstem cavernous malformation (CM) for entire cohort at each time point was 22.4% (pre-GKS), 10.1% (≤ 2 years post-GKS), 3.2% (≤ 10 years post-GKS). The AHR of brainstem CM for 79 enrolled patients was 27.2% (pre-GKS), 5.8% (2 years post-GKS), 3.4% (10 years post-GKS), and 3.5% (> 10 years post-GKS). Out of the 79 enrolled patients, 35 presented with focal neurologic deficits at the initial clinical visit. Among these patients, 74.3% showed recovery at the last follow-up. Symptomatic ARE occurred in five (6.4%) patients. No mortality occurred. Most lesions were decreased in size at the last follow-up MRI. Previous hemorrhage history (hazard ratio [HR], 8.38; 95% confidence interval [CI], 1.07–65.88; P = 0.043), and brainstem location (HR, 3.10; 95% CI, 1.26–7.64; P = 0.014) were significant risk factors for hemorrhage event. Conclusion GKS for CCM showed favorable long-term outcomes. GKS should be considered for CCM, especially when it has a previous hemorrhage history and brainstem location.

Background: Treatment for large (> 10 mL) arteriovenous malformations (AVMs) remains highly challenging. This study evaluated long-term effect of time-staged gamma knife radiosurgery (GKS) for large AVMs. Methods: For patients with large AVMs treated by time-staged GKS over 10 years, timestaged GKS was repeated every three years targeting the entire nidus if total obliteration was not achieved. Obliteration rate and post-GKS complications were assessed based on 10 mL volume interval of AVMs. Prognostic factors for these outcomes were evaluated using Cox regression analysis. Results: Ninety-six patients were analyzed. For AVMs in the 10–20 mL subgroup, a dose ≥ 13.5Gy yielded higher obliteration rate in the first GKS. In the 20–30 mL subgroup, a second GKS significantly boosted obliteration. AVMs > 30 mL did not achieve any obliteration with the first GKS. Among 35 (36.4%) cases lost to follow-up, 7 (7.2%) were lost due to GKS complications. Kaplan-Meier analysis showed that each subgroup needed different time for achieving 50% favorable obliteration outcome rate: 3.5, 6.5, and 8.2 years for 10–20 mL, 20–30 mL, and > 30 mL subgroup, respectively. Total obliteration rate calculated by intention-to-treat method: 73%, 51.7%, 35.7%, respectively, 61.5% overall. Post-GKS hemorrhage and chronic encapsulated expanding hematoma (CEEH) occurred in 13.5% and 8.3% of cases, respectively.Two patients died. Dose and volume were significant prognostic factors for obliteration. Initial AVM volume was a significant prognostic factor of post-GKS hemorrhage and CEEH. Conclusion Time-staged GKS for large AVMs less than 30 mL has highly favorable long-term outcome and a tolerable complication rate.

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.

Background/Objectives: Pediatric parotid malignancies are rare but represent a critical subset of head and neck cancers. This study integrates nationwide incidence trends in Korea with detailed surgical outcomes from a single tertiary institution to better understand the characteristics and management of these tumors.Materials & Methods: Nationwide data from the Korea Central Cancer Registry (1999-2019) were analyzed to assess trends in pediatric parotid malignancy incidence. A retrospective review was conducted on 31 pediatric parotidectomy cases at a tertiary hospital from 2011 to 2024. Clinical data, surgical methods, pathology results, and follow-up outcomes were examined. Results: Nationwide analysis revealed an annual mean of 9.0 ± 4.0 cases of pediatric parotid malignancies, with a significant rise in incidence among patients aged 10-19 years (APC 5.4%, 95% CI 1.1-9.8, p=0.016). Institutional data showed that the median age of patients underwent parotidectomy was 15.0 years, with males comprising 67.7%. Among 31 cases, 19 (61.3%) were benign, primarily pleomorphic adenomas (68.4%), and 12 (38.7%) were malignant, predominantly mucoepidermoid carcinoma (87.5%). Superficial or partial parotidectomy was the most common surgical approach (71.0%), and no unexpected complications or recurrences were observed. Conclusion Pediatric parotid malignancies in Korea exhibit rising incidence rates, particularly among teenagers. Surgical outcomes confirm the predominance of pleomorphic adenomas and mucoepidermoid carcinomas. Pediatric parotid surgery is a procedure with minimized complications and a promising prognosis. Tailored treatment strategies adapted to the unique characteristics of pediatric patients are essential to optimize outcomes.

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.

Background/Objectives: Pediatric parotid malignancies are rare but represent a critical subset of head and neck cancers. This study integrates nationwide incidence trends in Korea with detailed surgical outcomes from a single tertiary institution to better understand the characteristics and management of these tumors.Materials & Methods: Nationwide data from the Korea Central Cancer Registry (1999-2019) were analyzed to assess trends in pediatric parotid malignancy incidence. A retrospective review was conducted on 31 pediatric parotidectomy cases at a tertiary hospital from 2011 to 2024. Clinical data, surgical methods, pathology results, and follow-up outcomes were examined. Results: Nationwide analysis revealed an annual mean of 9.0 ± 4.0 cases of pediatric parotid malignancies, with a significant rise in incidence among patients aged 10-19 years (APC 5.4%, 95% CI 1.1-9.8, p=0.016). Institutional data showed that the median age of patients underwent parotidectomy was 15.0 years, with males comprising 67.7%. Among 31 cases, 19 (61.3%) were benign, primarily pleomorphic adenomas (68.4%), and 12 (38.7%) were malignant, predominantly mucoepidermoid carcinoma (87.5%). Superficial or partial parotidectomy was the most common surgical approach (71.0%), and no unexpected complications or recurrences were observed. Conclusion Pediatric parotid malignancies in Korea exhibit rising incidence rates, particularly among teenagers. Surgical outcomes confirm the predominance of pleomorphic adenomas and mucoepidermoid carcinomas. Pediatric parotid surgery is a procedure with minimized complications and a promising prognosis. Tailored treatment strategies adapted to the unique characteristics of pediatric patients are essential to optimize outcomes.

Background/Objectives: Pediatric parotid malignancies are rare but represent a critical subset of head and neck cancers. This study integrates nationwide incidence trends in Korea with detailed surgical outcomes from a single tertiary institution to better understand the characteristics and management of these tumors.Materials & Methods: Nationwide data from the Korea Central Cancer Registry (1999-2019) were analyzed to assess trends in pediatric parotid malignancy incidence. A retrospective review was conducted on 31 pediatric parotidectomy cases at a tertiary hospital from 2011 to 2024. Clinical data, surgical methods, pathology results, and follow-up outcomes were examined. Results: Nationwide analysis revealed an annual mean of 9.0 ± 4.0 cases of pediatric parotid malignancies, with a significant rise in incidence among patients aged 10-19 years (APC 5.4%, 95% CI 1.1-9.8, p=0.016). Institutional data showed that the median age of patients underwent parotidectomy was 15.0 years, with males comprising 67.7%. Among 31 cases, 19 (61.3%) were benign, primarily pleomorphic adenomas (68.4%), and 12 (38.7%) were malignant, predominantly mucoepidermoid carcinoma (87.5%). Superficial or partial parotidectomy was the most common surgical approach (71.0%), and no unexpected complications or recurrences were observed. Conclusion Pediatric parotid malignancies in Korea exhibit rising incidence rates, particularly among teenagers. Surgical outcomes confirm the predominance of pleomorphic adenomas and mucoepidermoid carcinomas. Pediatric parotid surgery is a procedure with minimized complications and a promising prognosis. Tailored treatment strategies adapted to the unique characteristics of pediatric patients are essential to optimize outcomes.

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.

Background/Objectives: Pediatric parotid malignancies are rare but represent a critical subset of head and neck cancers. This study integrates nationwide incidence trends in Korea with detailed surgical outcomes from a single tertiary institution to better understand the characteristics and management of these tumors.Materials & Methods: Nationwide data from the Korea Central Cancer Registry (1999-2019) were analyzed to assess trends in pediatric parotid malignancy incidence. A retrospective review was conducted on 31 pediatric parotidectomy cases at a tertiary hospital from 2011 to 2024. Clinical data, surgical methods, pathology results, and follow-up outcomes were examined. Results: Nationwide analysis revealed an annual mean of 9.0 ± 4.0 cases of pediatric parotid malignancies, with a significant rise in incidence among patients aged 10-19 years (APC 5.4%, 95% CI 1.1-9.8, p=0.016). Institutional data showed that the median age of patients underwent parotidectomy was 15.0 years, with males comprising 67.7%. Among 31 cases, 19 (61.3%) were benign, primarily pleomorphic adenomas (68.4%), and 12 (38.7%) were malignant, predominantly mucoepidermoid carcinoma (87.5%). Superficial or partial parotidectomy was the most common surgical approach (71.0%), and no unexpected complications or recurrences were observed. Conclusion Pediatric parotid malignancies in Korea exhibit rising incidence rates, particularly among teenagers. Surgical outcomes confirm the predominance of pleomorphic adenomas and mucoepidermoid carcinomas. Pediatric parotid surgery is a procedure with minimized complications and a promising prognosis. Tailored treatment strategies adapted to the unique characteristics of pediatric patients are essential to optimize outcomes.

Allergen immunotherapy (AIT) has been used for over a century and has been demonstrated to be effective in treating patients with various allergic diseases. AIT allergens can be administered through various routes, including subcutaneous, sublingual, intralymphatic, oral, or epicutaneous routes. Sublingual immunotherapy (SLIT) has recently gained clinical interest, and it is considered an alternative treatment for allergic rhinitis (AR) and asthma. This review provides an overview of the current evidence-based studies that address the use of SLIT for treating AR, including (1) mechanisms of action, (2) appropriate patient selection for SLIT, (3) the current available SLIT products in Korea, and (4) updated information on its efficacy and safety. Finally, this guideline aims to provide the clinician with practical considerations for SLIT.