Constitutional mismatch repair deficiency (CMMRD) is a rare and highly aggressive cancer predisposition syndrome caused by biallelic germline mutations in mismatch repair genes. This condition is characterized by early-onset malignancies across multiple organ systems, including central nervous system tumors, hematological cancers, and gastrointestinal malignancies. CMMRD-associated tumors exhibit hypermutation and microsatellite instability, resulting in a high tumor mutation burden and rendering these malignancies responsive to immune checkpoint inhibitors (ICIs). ICIs targeting programmed cell death protein-1 and programmed cell death ligand 1 have demonstrated remarkable efficacy, particularly in hypermutated tumors, providing durable responses and improving survival outcomes. Advances in genetic and molecular diagnostics have enhanced the ability to identify CMMRD early, allowing for the implementation of comprehensive surveillance programs and improved management strategies. A multidisciplinary and individualized approach is essential for managing CMMRD patients. This review underscores the importance of early diagnosis, surveillance, and emerging therapeutic approaches to improve outcomes and quality of life for individuals and families affected by this devastating syndrome.

Constitutional mismatch repair deficiency (CMMRD) is a rare and highly aggressive cancer predisposition syndrome caused by biallelic germline mutations in mismatch repair genes. This condition is characterized by early-onset malignancies across multiple organ systems, including central nervous system tumors, hematological cancers, and gastrointestinal malignancies. CMMRD-associated tumors exhibit hypermutation and microsatellite instability, resulting in a high tumor mutation burden and rendering these malignancies responsive to immune checkpoint inhibitors (ICIs). ICIs targeting programmed cell death protein-1 and programmed cell death ligand 1 have demonstrated remarkable efficacy, particularly in hypermutated tumors, providing durable responses and improving survival outcomes. Advances in genetic and molecular diagnostics have enhanced the ability to identify CMMRD early, allowing for the implementation of comprehensive surveillance programs and improved management strategies. A multidisciplinary and individualized approach is essential for managing CMMRD patients. This review underscores the importance of early diagnosis, surveillance, and emerging therapeutic approaches to improve outcomes and quality of life for individuals and families affected by this devastating syndrome.

Constitutional mismatch repair deficiency (CMMRD) is a rare and highly aggressive cancer predisposition syndrome caused by biallelic germline mutations in mismatch repair genes. This condition is characterized by early-onset malignancies across multiple organ systems, including central nervous system tumors, hematological cancers, and gastrointestinal malignancies. CMMRD-associated tumors exhibit hypermutation and microsatellite instability, resulting in a high tumor mutation burden and rendering these malignancies responsive to immune checkpoint inhibitors (ICIs). ICIs targeting programmed cell death protein-1 and programmed cell death ligand 1 have demonstrated remarkable efficacy, particularly in hypermutated tumors, providing durable responses and improving survival outcomes. Advances in genetic and molecular diagnostics have enhanced the ability to identify CMMRD early, allowing for the implementation of comprehensive surveillance programs and improved management strategies. A multidisciplinary and individualized approach is essential for managing CMMRD patients. This review underscores the importance of early diagnosis, surveillance, and emerging therapeutic approaches to improve outcomes and quality of life for individuals and families affected by this devastating syndrome.

Constitutional mismatch repair deficiency (CMMRD) is a rare and highly aggressive cancer predisposition syndrome caused by biallelic germline mutations in mismatch repair genes. This condition is characterized by early-onset malignancies across multiple organ systems, including central nervous system tumors, hematological cancers, and gastrointestinal malignancies. CMMRD-associated tumors exhibit hypermutation and microsatellite instability, resulting in a high tumor mutation burden and rendering these malignancies responsive to immune checkpoint inhibitors (ICIs). ICIs targeting programmed cell death protein-1 and programmed cell death ligand 1 have demonstrated remarkable efficacy, particularly in hypermutated tumors, providing durable responses and improving survival outcomes. Advances in genetic and molecular diagnostics have enhanced the ability to identify CMMRD early, allowing for the implementation of comprehensive surveillance programs and improved management strategies. A multidisciplinary and individualized approach is essential for managing CMMRD patients. This review underscores the importance of early diagnosis, surveillance, and emerging therapeutic approaches to improve outcomes and quality of life for individuals and families affected by this devastating syndrome.

Background: The Korean Occupational Stress Scale (KOSS) was developed in 2004. During this time, industrial structures have evolved, and societal awareness of occupational stress has changed. This study aims to develop and validate a revised version of the Korean Occupational Stress Scale (KOSS®19), tailored for workers, reflecting these changes. Methods: The KOSS®19 was developed based on the 26-item KOSS–short form (SF) through a review by eight experts. A survey was conducted including 359 service industry workers, comprising the KOSS®19, Burnout, and Depression scales. The KOSS®19 subscales were restructured, and their reliability and validity were evaluated. Results: The KOSS®19 composed of eight subscales: hazardous physical environment (2 items), high job demand (3 items), insufficient job control (2 items), low social support (2 items), job insecurity (2 items), organizational injustice (4 items), lack of reward (2 items), and work-life imbalance (2 items). The reliability and validity of the KOSS®19 were found to be satisfactory. Conclusions The KOSS®19 is a suitable tool for assessing occupational stress, effectively replacing the original KOSS and KOSS-SF.

Background: Emotional labor refers to the management of emotions and expressions to meet the emotional requirements of a job role. This study aimed to develop a revised version of the Korean Emotional Labor Scale (KELS®11), based on the first edition (KELS-24) introduced in 2014, and to provide practical applications and guidelines for its use in the Korean workplace through a validation process. Methods: The revised version of KELS®11 was derived from the 24-item KELS, following a review process involving eight experts. To validate the scale’s reliability and validity, a self-administered survey was conducted among 359 service industry workers using KELS®11, burnout, and depression scales. KELS®11 was reclassified, and its reliability and validity were evaluated. Receiver operating characteristic curve analysis was conducted to establish sex-specific cutoff values (normal vs. high-risk groups). Results: KELS®11 was designed to account for individual, organizational, and cultural contexts. It consists of four subscales and 11 items: “emotional regulation” (2 items), “emotional dissonance” (3 items), “organizational monitoring” (2 items), and “organizational protective system for emotional labor” (4 items). KELS®11 demonstrated good validity (content validity ratio: 0.84; item convergence/discriminant validity success rates: 100%; correlation with burnout: r = 0.185–0.436, p < 0.01; correlation with depression: r = 0.128–0.339, p < 0.05) and reliability (Cronbach’s alpha: 0.597–0.795). Additionally, sex-specific reference values were established to determine risk groups based on the intensity of emotional labor exposure. Conclusions KELS®11 is a validated and reliable measurement tool designed to assess the intensity and magnitude of emotional labor in the workplace. The revised tool reflects critical considerations in the development of emotional labor measurement scales.

Background: Workplace violence refers to any act or threat of physical violence, verbal abuse, harassment, intimidation, bullying, mobbing, or other aggressive and disruptive behaviors that occur at work. This study aims to develop and validate a revision of the Korean Workplace Violence Scale (KWVS®13), based on the first edition of the Korean Workplace Violence Scale (KWVS-24), and to provide practical applications and guidelines for the Korean workplace environment. Methods: The revised KWVS®13 was developed by restructuring the 24-item KWVS through a review process involving eight experts. To validate the reliability and validity of KWVS®13, a self-administered survey comprising KWVS®13, burnout, and depression scales was conducted among 359 service industry workers. KWVS®13 was reclassified, and its reliability and validity were assessed. Receiver operating characteristic curve analysis was performed to establish sex-specific cutoff values (normal vs. risk) of the scale. Results: KWVS®13 consists of 13 items across four subscales: “psychological and sexual violence from customers” (4 items), “psychological and sexual violence from supervisors or coworkers” (4 items), “physical assault from customers, supervisors, or coworkers” (2 items), and “organizational protective system for workplace violence” (3 items). We found that KWVS®13 shows relatively good validity (content validity ratio for content validity: 0.888; success rate of item convergent and discriminant validity: 100%, and significant correlation coefficient with burnout (r = 0.115–0.83, p < 0.05) and depression (r = 0.098–0.348, p < 0.05) with the exception of Organizational Violence Protection System for Workplace Violence) and reliability (Cronbach’s alpha: 0.827–0.860). The reference values for determining risk groups according to the intensity of exposure to workplace violence are presented separately by sex. Conclusions KWVS®13 is a robust and useful measurement tool to objectively and quantitatively assess the intensity and magnitude of workplace violence. It incorporates important considerations for workplace violence assessment and provides a reliable framework for evaluating workplace violence in various professional settings.

Background/Aims: In Korea, the incidence of primary diffuse large B-cell lymphoma of the central nervous system (PCNSL) is increasing and autologous stem cell transplantation (ASCT) has improved the survival of younger patients. We explored our real-world experience with PCNSL at Asan Medical Center (AMC). Methods: We used the AMC lymphoma registry to collect patient data prospectively. We analyzed 279 patients diagnosed from 2002 until August 2019. Results: The PCNSL incidence at AMC increased progressively and comprised 7.4-8.9% of new non-Hodgkin lymphoma patients annually during the most recent 4 years. The median age was 60 years (range, 17-85) and males comprised 55%. Patients under 65 years of age (n = 183) had no significant differences in characteristics compared to those aged 65 years or over, with the exception of less occipital lobe involvement and lower beta-2 microglobulin levels. Rituximab, methotrexate, procarbazine, and vincristine (R-MPV) combination induction had the best overall response, of 95%. The median overall survival was 3.8 years with 5- and 10-year survival rates of 41.5% and 30.2%, respectively. Survival was better in younger patients and those treated with ASCT. Thiotepa, busulfan, and cytoxan (TBC) conditioning chemotherapy had better survival than other combinations. The International Extranodal Lymphoma Study Group and Memorial Sloan Kettering Cancer Center prognostic score systems were valid in this cohort. Age and performance status were independent prognostic factors. Exclusive extra-central nervous system failure occurred in six patients (5.6%) among 107 failures. Conclusions The incidence of PCNSL is rising. R-MPV induction therapy followed by ASCT with TBC has improved the survival of young, fit PCNSL patients.

Background/Aims: In Korea, the incidence of primary diffuse large B-cell lymphoma of the central nervous system (PCNSL) is increasing and autologous stem cell transplantation (ASCT) has improved the survival of younger patients. We explored our real-world experience with PCNSL at Asan Medical Center (AMC). Methods: We used the AMC lymphoma registry to collect patient data prospectively. We analyzed 279 patients diagnosed from 2002 until August 2019. Results: The PCNSL incidence at AMC increased progressively and comprised 7.4-8.9% of new non-Hodgkin lymphoma patients annually during the most recent 4 years. The median age was 60 years (range, 17-85) and males comprised 55%. Patients under 65 years of age (n = 183) had no significant differences in characteristics compared to those aged 65 years or over, with the exception of less occipital lobe involvement and lower beta-2 microglobulin levels. Rituximab, methotrexate, procarbazine, and vincristine (R-MPV) combination induction had the best overall response, of 95%. The median overall survival was 3.8 years with 5- and 10-year survival rates of 41.5% and 30.2%, respectively. Survival was better in younger patients and those treated with ASCT. Thiotepa, busulfan, and cytoxan (TBC) conditioning chemotherapy had better survival than other combinations. The International Extranodal Lymphoma Study Group and Memorial Sloan Kettering Cancer Center prognostic score systems were valid in this cohort. Age and performance status were independent prognostic factors. Exclusive extra-central nervous system failure occurred in six patients (5.6%) among 107 failures. Conclusions The incidence of PCNSL is rising. R-MPV induction therapy followed by ASCT with TBC has improved the survival of young, fit PCNSL patients.

Background/Aims: In Korea, the incidence of primary diffuse large B-cell lymphoma of the central nervous system (PCNSL) is increasing and autologous stem cell transplantation (ASCT) has improved the survival of younger patients. We explored our real-world experience with PCNSL at Asan Medical Center (AMC). Methods: We used the AMC lymphoma registry to collect patient data prospectively. We analyzed 279 patients diagnosed from 2002 until August 2019. Results: The PCNSL incidence at AMC increased progressively and comprised 7.4-8.9% of new non-Hodgkin lymphoma patients annually during the most recent 4 years. The median age was 60 years (range, 17-85) and males comprised 55%. Patients under 65 years of age (n = 183) had no significant differences in characteristics compared to those aged 65 years or over, with the exception of less occipital lobe involvement and lower beta-2 microglobulin levels. Rituximab, methotrexate, procarbazine, and vincristine (R-MPV) combination induction had the best overall response, of 95%. The median overall survival was 3.8 years with 5- and 10-year survival rates of 41.5% and 30.2%, respectively. Survival was better in younger patients and those treated with ASCT. Thiotepa, busulfan, and cytoxan (TBC) conditioning chemotherapy had better survival than other combinations. The International Extranodal Lymphoma Study Group and Memorial Sloan Kettering Cancer Center prognostic score systems were valid in this cohort. Age and performance status were independent prognostic factors. Exclusive extra-central nervous system failure occurred in six patients (5.6%) among 107 failures. Conclusions The incidence of PCNSL is rising. R-MPV induction therapy followed by ASCT with TBC has improved the survival of young, fit PCNSL patients.