Objective: Dysphagia is a common clinical condition characterized by difficulty in swallowing. It is sub-classified into oropharyngeal dysphagia, which refers to problems in the mouth and pharynx, and esophageal dysphagia, which refers to problems in the esophageal body and esophagogastric junction. Dysphagia can have a significant negative impact one’s physical health and quality of life as its severity increases. Therefore, proper assessment and management of dysphagia are critical for improving swallowing function and preventing complications. Thus a guideline was developed to provide evidence-based recommendations for assessment and management in patients with dysphagia. Methods: Nineteen key questions on dysphagia were developed. These questions dealt with various aspects of problems related to dysphagia, including assessment, management, and complications. A literature search for relevant articles was conducted using Pubmed, Embase, the Cochrane Library, and one domestic database of KoreaMed, until April 2021. The level of evidence and recommendation grade were established according to the Grading of Recommendation Assessment, Development and Evaluation methodology. Results: Early screening and assessment of videofluoroscopic swallowing were recommended for assessing the presence of dysphagia. Therapeutic methods, such as tongue and pharyngeal muscle strengthening exercises and neuromuscular electrical stimulation with swallowing therapy, were effective in improving swallowing function and quality of life in patients with dysphagia. Nutritional intervention and an oral care program were also recommended. Conclusion This guideline presents recommendations for the assessment and management of patients with oropharyngeal dysphagia, including rehabilitative strategies.

Activated phosphoinositide 3-kinase δ syndrome (APDS)1 is caused by gain-of-function mutations in PIK3CD, which encodes the catalytic p110δ subunit of phosphoinositide 3 kinase. We describe three patients with APDS1, the first thereof in Korea. Therein, we investigated clinical manifestations of APDS1 and collected data on the efficacy and safety profile of sirolimus, a mammalian target of rapamycin inhibitor and pathway-specific targeted medicine. The same heterozygous PIK3CD mutation was detected in all three patients (E1021K). After genetic diagnosis, all patients received sirolimus and experienced an excellent response, including amelioration of lymphoproliferation and improvement of nodular mucosal lymphoid hyperplasia in the gastrointestinal tract. The median trough level of sirolimus was 5.5 ng/mL (range, 2.8–7.5) at a dose of 2.6–3.6 mg/m2. Two patients who needed highdose, short-interval, immunoglobulin-replacement treatment (IGRT) had a reduced requirement for IGRT after initiating sirolimus, and the dosing interval was extended from 2 and 3 weeks to 4 weeks. The IgG trough level after sirolimus treatment (median, 594 mg/dL; range, 332–799 mg/dL) was significantly higher than that before sirolimus treatment (median, 290 mg/dL; range, 163–346 mg/dL) (p<0.001). One episode of elevated serum creatinine with a surge of sirolimus (Patient 2) and episodes of neutropenia and oral stomatitis (Patient 1) were observed. We diagnosed the first three patients with APDS1 in Korea. Low-dose sirolimus may alleviate clinical manifestations thereof, including hypogammaglobulinemia.

Purpose: The treatment outcome of brentuximab vedotin (BV) has not been related with CD30 expressionin previous studies enrolling patients with a wide range of CD30 expression level.Thus, this study explored the efficacy of BV in high-CD30–expressing non-Hodgkin lymphoma(NHL) patients most likely to benefit. Materials and Methods: This phase II study (Clinicaltrials.gov: NCT02280785) enrolled relapsed or refractory high-CD30–expressing NHL, with BV administered intravenously at 1.8 mg/kg every 3 weeks.The primary endpoint was > 40% disease control rate, consisting of complete response(CR), partial response (PR), or stable disease. We defined high CD30 expression as ! 30%tumor cells positive for CD30 by immunohistochemistry. Results: High-CD30-expressing NHL patients (n=33) were enrolled except anaplastic large cell lymphoma.The disease control rate was 48.5% (16/33) including six CR and six PR; six patients(4CR, 2PR) maintained their response over 16 completed cycles. Response to BV and survivalwere not associated with CD30 expression levels. Over a median of 29.2 months offollow-up, the median progression-free and overall survival rates were 1.9 months and 6.1months, respectively. The most common adverse events were fever (39%), neutropenia(30%), fatigue (24%), and peripheral sensory neuropathy (27%). In a post-hoc analysis forthe association of multiple myeloma oncogene 1 (MUM1) on treatment outcome, MUM1-negative patients showed a higher response (55.6%, 5/9) than MUM1-positive patients(13.3%, 2/15). Conclusion BV performance as a single agent was acceptable in terms of disease control rates and toxicityprofiles, especially MUM1-negative patients.

BACKGROUND: Recent findings in molecular pathology suggest that genetic translocation and/or overexpression of oncoproteins is important in salivary gland tumorigenesis and diagnosis. We investigated PLAG1, SOX10, and Myb protein expression in various salivary gland neoplasm tissues. METHODS: A total of 113 cases of surgically resected salivary gland neoplasms at the National Cancer Center from January 2007 to March 2017 were identified. Immunohistochemical staining of PLAG1, SOX10, and Myb in tissue samples was performed using tissue microarrays. RESULTS: Among the 113 cases, 82 (72.6%) were benign and 31 (27.4%) were malignant. PLAG1 showed nuclear staining and normal parotid gland was not stained. Among 48 cases of pleomorphic adenoma, 29 (60.4%) were positive for PLAG1. All other benign and malignant salivary gland neoplasms were PLAG1-negative. SOX10 showed nuclear staining. In normal salivary gland tissues SOX10 was expressed in cells of acinus and intercalated ducts. In benign tumors, SOX10 expression was observed in all pleomorphic adenoma (48/48), and basal cell adenoma (3/3), but not in other benign tumors. SOX10 positivity was observed in nine of 31 (29.0%) malignant tumors. Myb showed nuclear staining but was not detected in normal parotid glands. Four of 31 (12.9%) malignant tumors showed Myb positivity: three adenoid cystic carcinomas (AdCC) and one myoepithelial carcinoma with focal AdCC-like histology. CONCLUSIONS: PLAG1 expression is specific to pleomorphic adenoma. SOX10 expression is helpful to rule out excretory duct origin tumor, but its diagnostic value is relatively low. Myb is useful for diagnosing AdCC when histology is unclear in the surgical specimen.
Adenoma ; Adenoma, Pleomorphic ; Antibody-Dependent Cell Cytotoxicity ; Carcinogenesis ; Carcinoma, Adenoid Cystic ; Diagnosis ; Immunohistochemistry ; Oncogene Proteins ; Oncogene Proteins v-myb ; Parotid Gland ; Pathology, Molecular ; Salivary Gland Neoplasms ; Salivary Glands ; SOX Transcription Factors ; Translocation, Genetic

PURPOSE: Despite the benefits of minimally invasive surgery for cervical cancer, there are a lack of randomized trials comparing laparoscopic radical hysterectomy and abdominal radical hysterectomy. We compared morbidity, cost of care, and survival between abdominal radical hysterectomy and laparoscopic radical hysterectomy for cervical cancer. MATERIALS AND METHODS: We used the Korean nationwide database to identify women with cervical cancer who underwent radical hysterectomy from January 1, 2011 to December 31, 2014. Patients who underwent abdominal radical hysterectomy were compared to those who underwent laparoscopic radical hysterectomy. Perioperative morbidity, the use of adjuvant therapy, and survival were evaluated after propensity score balancing. RESULTS: We identified 6,335 patients, including 3,235 who underwent abdominal radical hysterectomy and 3,100 who underwent laparoscopic radical hysterectomy. The use of laparoscopic radical hysterectomy increased from 46.1% in 2011 to 51.8% in 2014. Patients who were younger, had a more recent year of diagnosis, and were treated in the metropolitan area were more likely to undergo a laparoscopic procedure (p < 0.001). Compared to abdominal radical hysterectomy, laparoscopic radical hysterectomy was associated with lower rates of complication, fewertransfusions, a shorter hospital stay, less adjuvant therapy, and reduced total medical costs (p < 0.001). Laparoscopic surgery was associated with a better overall survival than abdominal operation (hazard ratio, 0.74; 95% confidence interval, 0.64 to 0.85). CONCLUSION: In the postdissemination era, laparoscopic radical hysterectomy was associated with more favorable morbidity profiles, a lower cost of care, and comparable survival than abdominal radical hysterectomy.
Diagnosis ; Female ; Humans ; Hysterectomy ; Laparoscopy ; Length of Stay ; Minimally Invasive Surgical Procedures ; Propensity Score ; Uterine Cervical Neoplasms

PURPOSE: Secondary primary cancers (SPCs) commonly arise in patients with renal cell carcinoma (RCC). We designed the present study to estimate the SPC incidence in Korean patients with RCC. MATERIALS AND METHODS: The study cohort was population-based and consisted of 40,347 individuals from the Korean Central Cancer Registry who were diagnosed with primary renal cancer between 1993 and 2013. Standardized incidence ratios (SIRs) for SPCs were estimated for different ages at diagnosis, latencies, diagnostic periods, and treatments. RESULTS: For patients with primary RCC, the risk of developing a SPC was higher than the risk of developing cancer in the general population (SIR, 1.13; 95% confidence interval, 1.08 to 1.18). Most cancer types showed higher incidences in patients with RCC than in the general population. However, the relative incidence of gastric cancer as an SPC varied by age. Gastric cancer incidence was elevated in young patients (< 30 years) with RCC, but reduced in older (≥ 30) patients with RCC. Patients with advanced RCC died prematurely, regardless of SPC development. In contrast, those with early-stage RCC survived for longer periods, although SPC development affected their post-RCC survival. After SPC development, women had better survival than men. CONCLUSION: In Korean patients with primary RCC, the incidence of SPC was 13% higher than the incidence of cancer in the general population. These findings may play important roles in the conduct of follow-up evaluations and education for patients with RCC.
Carcinoma, Renal Cell ; Cohort Studies ; Diagnosis ; Education ; Female ; Follow-Up Studies ; Humans ; Incidence ; Kidney Neoplasms ; Kidney ; Korea ; Male ; Neoplasms, Second Primary ; Prognosis ; Stomach Neoplasms

PURPOSE: This study aimed to evaluate the prognostic significance of smoking status in muscle invasive bladder cancer (MIBC) and non-MIBC in recurrence-free (RFS), progression-free (PFS), disease-free survival (DFS), and cancer-specific survival (CSS). MATERIALS AND METHODS: We retrospectively evaluated 541 patients with MIBC and non-MIBC who were surgically treated during 2002–2013. Smoking status was defined as never smokers (NS; n=160, 30%), former smokers (FS; smoking cessation for ≥1 year, n=176, 33%), and current smokers (CS; >100 cigarettes, n=198, 37%). We statistically compared these groups' clinicopathological facCtors for the predictive factors for RFS and PFS for non-MIBC (NMIBC) and DFS for MIBC, and CSS using multivariate model. RESULTS: The CS, FS, and NS groups exhibited insignificantly different pathological staging, grades, and immunohistological characteristics (p>0.05). Among the 441 patients with NMIBC, pathologic tumor size was a significant risk factor for RFS (1–3 cm: hazard ratio [HR], 1.88; >3 cm: HR, 2.21; p < 0.05); age (HR, 1.06), intravesical therapy (HR, 0.25), and high-grade cancer (HR, 8.33) significant for PFS; and age (HR, 1.08), intravesical instillation (HR, 0.26), and smoking status (FS: HR, 0.40; CS: HR, 0.44) significant for CSS (p < 0.05). The 93 patients with MIBC had no significant risk factors for DFS, although their significant risk factors for CSS were age (HR, 1.05), female sex (HR, 2.64), and carcinoma in situ (HR, 4.72) (p < 0.05). CONCLUSIONS: Smoking status only significantly affected CSS in patients with NMIBC.
Administration, Intravesical ; Carcinoma in Situ ; Disease-Free Survival ; Female ; Humans ; Muscles ; Prognosis ; Retrospective Studies* ; Risk Factors ; Smoke* ; Smoking Cessation ; Smoking* ; Tobacco Products ; Urinary Bladder Neoplasms* ; Urinary Bladder*