Purpose: This multicenter, open-label, phase II trial evaluated the efficacy and safety of bortezomib combined with dexamethasone for the treatment of relapsed/refractory cutaneous T-cell lymphoma (CTCL) in previously treated patients across 14 institutions in South Korea. Materials and Methods: Between September 2017 and July 2020, 29 patients with histologically confirmed CTCL received treatment, consisting of eight 4-week cycles of induction therapy followed by maintenance therapy, contingent upon response, for up to one year. The primary endpoint was the proportion of patients achieving an objective global response. Results: Thirteen of the 29 patients (44.8%) achieved an objective global response, including two complete responses. The median progression-free survival (PFS) was 5.8 months, with responders showing a median PFS of 14.0 months. Treatment-emergent adverse events were generally mild, with a low incidence of peripheral neuropathy and hematologic toxicities. Despite the trend toward shorter PFS in patients with higher mutation burdens, genomic profiling before and after treatment showed no significant emergence of new mutations indicative of disease progression. Conclusion This study supports the use of bortezomib and dexamethasone as a viable and safe treatment option for previously treated CTCL, demonstrating substantial efficacy and manageability in adverse effects. Further research with a larger cohort is suggested to validate these findings and explore the prognostic value of mutation profiles.

Purpose: This multicenter, open-label, phase II trial evaluated the efficacy and safety of bortezomib combined with dexamethasone for the treatment of relapsed/refractory cutaneous T-cell lymphoma (CTCL) in previously treated patients across 14 institutions in South Korea. Materials and Methods: Between September 2017 and July 2020, 29 patients with histologically confirmed CTCL received treatment, consisting of eight 4-week cycles of induction therapy followed by maintenance therapy, contingent upon response, for up to one year. The primary endpoint was the proportion of patients achieving an objective global response. Results: Thirteen of the 29 patients (44.8%) achieved an objective global response, including two complete responses. The median progression-free survival (PFS) was 5.8 months, with responders showing a median PFS of 14.0 months. Treatment-emergent adverse events were generally mild, with a low incidence of peripheral neuropathy and hematologic toxicities. Despite the trend toward shorter PFS in patients with higher mutation burdens, genomic profiling before and after treatment showed no significant emergence of new mutations indicative of disease progression. Conclusion This study supports the use of bortezomib and dexamethasone as a viable and safe treatment option for previously treated CTCL, demonstrating substantial efficacy and manageability in adverse effects. Further research with a larger cohort is suggested to validate these findings and explore the prognostic value of mutation profiles.

Purpose: This multicenter, open-label, phase II trial evaluated the efficacy and safety of bortezomib combined with dexamethasone for the treatment of relapsed/refractory cutaneous T-cell lymphoma (CTCL) in previously treated patients across 14 institutions in South Korea. Materials and Methods: Between September 2017 and July 2020, 29 patients with histologically confirmed CTCL received treatment, consisting of eight 4-week cycles of induction therapy followed by maintenance therapy, contingent upon response, for up to one year. The primary endpoint was the proportion of patients achieving an objective global response. Results: Thirteen of the 29 patients (44.8%) achieved an objective global response, including two complete responses. The median progression-free survival (PFS) was 5.8 months, with responders showing a median PFS of 14.0 months. Treatment-emergent adverse events were generally mild, with a low incidence of peripheral neuropathy and hematologic toxicities. Despite the trend toward shorter PFS in patients with higher mutation burdens, genomic profiling before and after treatment showed no significant emergence of new mutations indicative of disease progression. Conclusion This study supports the use of bortezomib and dexamethasone as a viable and safe treatment option for previously treated CTCL, demonstrating substantial efficacy and manageability in adverse effects. Further research with a larger cohort is suggested to validate these findings and explore the prognostic value of mutation profiles.

Objective: The aim of this study was to analyze the origins of prostatic arteries (PAs) in the Korean population and compare them with those reported in the literature. Materials and Methods: From April 2018 to February 2024, 108 male (mean age ± standard deviation: 71.6 ± 9.7 years) with lower urinary tract symptoms (n = 102) or refractory hematuria (n = 6) underwent prostatic artery embolization (PAE). Computed tomography and angiography images were retrospectively reviewed. The branching pattern of the internal iliac artery (IIA) was classified according to the Yamaki system. The origin of the PA was categorized using the de Assis definition, and the incidence of each type was recorded. A systematic literature review was conducted and the most common types of PA were investigated. Results: PAE was successfully implemented on 211 of the 216 pelvic sidewalls. PA cannulation failed in five sidewalls due to a steno-occlusive state. The most common IIA type was type A, in which the IIA was divided into the superior gluteal artery and gluteal–pudendal trunk (77%). Of 226 PAs analyzed, including 15 in 211 sidewalls exhibiting dual PAs, the most common PA origin was the internal pudendal artery (type IV, 35%), followed by the superior vesical (type I, 25%) and obturator (type III, 21%) arteries. Anterior division of IIA (type II) was less common (10%). Type V (uncommon origins) occurred in 8% of cases, including five distal internal pudendal arteries, four quadfurcations, three inferior gluteal arteries, three trifurcations, two medial femoral circumflex arteries, and two rectal arteries. Two of the five patients with surgically or endovascularly altered anatomy were successfully treated via PAs originating from the medial femoral circumflex arteries. Globally, type I is the most common PA type. Conclusion In the Korean population, the most common IIA pattern and PA origin were types A and IV, respectively.

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.

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.

Objective: The aim of this study was to analyze the origins of prostatic arteries (PAs) in the Korean population and compare them with those reported in the literature. Materials and Methods: From April 2018 to February 2024, 108 male (mean age ± standard deviation: 71.6 ± 9.7 years) with lower urinary tract symptoms (n = 102) or refractory hematuria (n = 6) underwent prostatic artery embolization (PAE). Computed tomography and angiography images were retrospectively reviewed. The branching pattern of the internal iliac artery (IIA) was classified according to the Yamaki system. The origin of the PA was categorized using the de Assis definition, and the incidence of each type was recorded. A systematic literature review was conducted and the most common types of PA were investigated. Results: PAE was successfully implemented on 211 of the 216 pelvic sidewalls. PA cannulation failed in five sidewalls due to a steno-occlusive state. The most common IIA type was type A, in which the IIA was divided into the superior gluteal artery and gluteal–pudendal trunk (77%). Of 226 PAs analyzed, including 15 in 211 sidewalls exhibiting dual PAs, the most common PA origin was the internal pudendal artery (type IV, 35%), followed by the superior vesical (type I, 25%) and obturator (type III, 21%) arteries. Anterior division of IIA (type II) was less common (10%). Type V (uncommon origins) occurred in 8% of cases, including five distal internal pudendal arteries, four quadfurcations, three inferior gluteal arteries, three trifurcations, two medial femoral circumflex arteries, and two rectal arteries. Two of the five patients with surgically or endovascularly altered anatomy were successfully treated via PAs originating from the medial femoral circumflex arteries. Globally, type I is the most common PA type. Conclusion In the Korean population, the most common IIA pattern and PA origin were types A and IV, respectively.

Objective: The aim of this study was to analyze the origins of prostatic arteries (PAs) in the Korean population and compare them with those reported in the literature. Materials and Methods: From April 2018 to February 2024, 108 male (mean age ± standard deviation: 71.6 ± 9.7 years) with lower urinary tract symptoms (n = 102) or refractory hematuria (n = 6) underwent prostatic artery embolization (PAE). Computed tomography and angiography images were retrospectively reviewed. The branching pattern of the internal iliac artery (IIA) was classified according to the Yamaki system. The origin of the PA was categorized using the de Assis definition, and the incidence of each type was recorded. A systematic literature review was conducted and the most common types of PA were investigated. Results: PAE was successfully implemented on 211 of the 216 pelvic sidewalls. PA cannulation failed in five sidewalls due to a steno-occlusive state. The most common IIA type was type A, in which the IIA was divided into the superior gluteal artery and gluteal–pudendal trunk (77%). Of 226 PAs analyzed, including 15 in 211 sidewalls exhibiting dual PAs, the most common PA origin was the internal pudendal artery (type IV, 35%), followed by the superior vesical (type I, 25%) and obturator (type III, 21%) arteries. Anterior division of IIA (type II) was less common (10%). Type V (uncommon origins) occurred in 8% of cases, including five distal internal pudendal arteries, four quadfurcations, three inferior gluteal arteries, three trifurcations, two medial femoral circumflex arteries, and two rectal arteries. Two of the five patients with surgically or endovascularly altered anatomy were successfully treated via PAs originating from the medial femoral circumflex arteries. Globally, type I is the most common PA type. Conclusion In the Korean population, the most common IIA pattern and PA origin were types A and IV, respectively.

Objective: The aim of this study was to analyze the origins of prostatic arteries (PAs) in the Korean population and compare them with those reported in the literature. Materials and Methods: From April 2018 to February 2024, 108 male (mean age ± standard deviation: 71.6 ± 9.7 years) with lower urinary tract symptoms (n = 102) or refractory hematuria (n = 6) underwent prostatic artery embolization (PAE). Computed tomography and angiography images were retrospectively reviewed. The branching pattern of the internal iliac artery (IIA) was classified according to the Yamaki system. The origin of the PA was categorized using the de Assis definition, and the incidence of each type was recorded. A systematic literature review was conducted and the most common types of PA were investigated. Results: PAE was successfully implemented on 211 of the 216 pelvic sidewalls. PA cannulation failed in five sidewalls due to a steno-occlusive state. The most common IIA type was type A, in which the IIA was divided into the superior gluteal artery and gluteal–pudendal trunk (77%). Of 226 PAs analyzed, including 15 in 211 sidewalls exhibiting dual PAs, the most common PA origin was the internal pudendal artery (type IV, 35%), followed by the superior vesical (type I, 25%) and obturator (type III, 21%) arteries. Anterior division of IIA (type II) was less common (10%). Type V (uncommon origins) occurred in 8% of cases, including five distal internal pudendal arteries, four quadfurcations, three inferior gluteal arteries, three trifurcations, two medial femoral circumflex arteries, and two rectal arteries. Two of the five patients with surgically or endovascularly altered anatomy were successfully treated via PAs originating from the medial femoral circumflex arteries. Globally, type I is the most common PA type. Conclusion In the Korean population, the most common IIA pattern and PA origin were types A and IV, respectively.

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.