Background: s/Aims: Reported incidence of extrahepatic bile duct cancer is higher in Asians than in Western populations. Korea, in particular, is one of the countries with the highest incidence rates of extrahepatic bile duct cancer in the world. Although research and innovative therapeutic modalities for extrahepatic bile duct cancer are emerging, clinical guidelines are currently unavailable in Korea. The Korean Society of Hepato-Biliary-Pancreatic Surgery in collaboration with related societies (Korean Pancreatic and Biliary Surgery Society, Korean Society of Abdominal Radiology, Korean Society of Medical Oncology, Korean Society of Radiation Oncology, Korean Society of Pathologists, and Korean Society of Nuclear Medicine) decided to establish clinical guideline for extrahepatic bile duct cancer in June 2021. Methods: Contents of the guidelines were developed through subgroup meetings for each key question and a preliminary draft was finalized through a Clinical Guidelines Committee workshop. Results: In November 2021, the finalized draft was presented for public scrutiny during a formal hearing. Conclusions The extrahepatic guideline committee believed that this guideline could be helpful in the treatment of patients.

Background/Aims: This study evaluated the long-term cardiovascular complications among Korean patients with hypertension and compared them with that of controls without hypertension. Methods: The Korean Hypertension Cohort (KHC) enrolled 11,043 patients with hypertension and followed them for more than 10 years. Age- and sex-matched controls without hypertension were enrolled at a 1:10 ratio. We compared the incidence of cardiovascular events and death among patients and controls without hypertension. Results: The mean age was 59 years, and 34.8% and 16.5% of the patients belonged to the high and moderate cardiovascular risk groups, respectively. During the 10-year follow-up, 1,591 cardiovascular events (14.4%) with 588 deaths (5.3%) occurred among patients with hypertension and 7,635 cardiovascular events (6.9%) with 4,826 deaths (4.4%) occurred among controls. Even the low-risk population with hypertension showed a higher cardiovascular event rate than the population without hypertension. Although blood pressure measurements in the clinic showed remarkable inaccuracy compared with those measured in the national health examinations, systolic blood pressure (SBP) ≥ 150 mmHg was significantly associated with a higher risk of cardiovascular events. Conclusions This long-term follow-up study confirmed the cardiovascular event rates among Korean hypertensive patients were substantial, reaching 15% in 10 years. SBP levels ≥ 150 mmHg were highly associated with occurrence of cardiovascular event rates.

Background: and Objective Endoscopic airway dilation is the primary treatment for pediatric subglottic stenosis (SGS) due to its feasibility and non-invasiveness. The aim of this study is to evaluate the risk factors for the failure of endoscopic airway dilation in pediatric patients with SGS.
Materials and Methods: This study reviewed medical records of 38 pediatric patients had endoscopic dilation from a single and tertiary referral center, retrospectively. The success of the endoscopic dilation procedure was defined as no dyspneic symptom without tracheostomy or laryngotracheal reconstruction. Demographic profiles, underlying disease, and Myer-Cotton SGS severity grade were recorded. Success rates and risk factors for the failure of treatment were analyzed.
Results: The SGS patients with severity grade I was most common. After mean 1.8 numbers of procedures, there were 23 patients (60.5%) in the success group and 15 patients (39.5%) in the failure group. Age, sex, underlying diseases, and SGS severity grade were not significantly different between two groups. In patients who had multiple endoscopic procedures, the failure group showed SGS deteriorated after procedures in 66.7%, compared to 11.1% of the success group. In multivariable analysis, a long-term intubation (≥1 month) was identified as an independent risk factor for failure of endoscopic dilation procedure.
Conclusion Although endoscopic dilation procedure is safe and effective for the management, repetitive endoscopic dilation may not give clinical benefit in patient with long-term intubation. Other airway procedures must be considered in those group of patients.

PURPOSE: Papillary thyroid carcinomas (PTCs) frequently involve genetic alterations. The objective of this study was to investigate genetic alterations and further explore the relationships between these genetic alterations and clinicopathological characteristics in a high-recurrence risk (node positive, N1) PTC group. MATERIALS AND METHODS: Tumor tissue blocks were obtained from 240 surgically resected patients with histologically confirmed stage III/IV (pT3/4 or N1) PTCs. We screened gene fusions using NanoString’s nCounter technology and mutational analysis was performed by direct DNA sequencing. Data describing the clinicopathological characteristics and clinical courses were retrospectively collected. RESULTS: Of the 240 PTC patients, 207 (86.3%) had at least one genetic alteration, including BRAF mutation in 190 patients (79.2%), PIK3CA mutation in 25 patients (10.4%), NTRK1/3 fusion in six patients (2.5%), and RET fusion in 24 patients (10.0%). Concomitant presence of more than two genetic alterations was seen in 36 patients (15%). PTCs harboring BRAF mutation were associated with RET wild-type expression (p=0.001). RET fusion genes have been found to occur with significantly higher frequency in N1b stage patients (p=0.003) or groups of patients aged 45 years or older (p=0.031); however, no significant correlation was found between other genetic alterations. There was no trend toward favorable recurrence-free survival or overall survival among patients lacking genetic alterations. CONCLUSION: In the selected high-recurrence risk PTC group, most patients had more than one genetic alteration. However, these known alterations could not entirely account for clinicopathological features of high-recurrence risk PTC.
Gene Fusion ; Humans ; Retrospective Studies ; Sequence Analysis, DNA ; Thyroid Gland* ; Thyroid Neoplasms*

PURPOSE: Oropharyngeal squamous cell carcinoma (OSCC) has been recognized as an immunosuppressive disease. Various mechanisms have been proposed for immune escape, including dysregulation of immune checkpoints such as the PD-1:PD-L1 pathway. We investigated the expression of programmed cell death-ligand 1 (PD-L1) in HPV-negative and HPV-positive OSCC to determine its prevalence and prognostic relevance. MATERIALS AND METHODS: Using immunohistochemistry, 133 cases of OSCC were evaluated for expression of PD-L1. Formalin-fixed paraffin-embedded tumor samples were stained with monoclonal antibody (clone 5H1) to PD-L1. PD-L1 positivity was defined as membrane staining in ≥20% of tumor cells. Correlations between PD-L1 expression and HPV status and survival parameters were analyzed. RESULTS: Of the 133 patients, 68% showed PD-L1 expression, and 67% of patients were positive for p16 expression by immunohistochemistry. No significant difference in PD-L1 expression was observed between HPV(-) and HPV(+) tumors (61% vs. 71%, p=0.274). No significant difference in age, gender, smoking history, location of tumor origin, or stage was observed according to PD-L1 status. With a median follow-up period of 44 months, older age (≥65) (p=0.017) and T3-4 stage (p<0.001) were associated with poor overall survival (OS), whereas PD-L1 expression did not affect OS in univariate and multivariate analysis. CONCLUSION: PD-L1 expression was observed in the majority of OSCC patients regardless of HPV status. Further large prospective studies are required to determine the role of PD-L1 expression as a prognostic or predictive biomarker, and clinical studies of immune checkpoint inhibitors in OCSS are warranted regardless of HPV status.
Carcinoma, Squamous Cell* ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Membranes ; Multivariate Analysis ; Oropharyngeal Neoplasms ; Prevalence ; Prospective Studies ; Smoke ; Smoking ; United Nations

BACKGROUND: Azacitidine (AZA) is standard care for patients with myelodysplastic syndrome (MDS) who have not had allogeneic stem cell transplantation. Chromosomal abnormalities (CA) including complex karyotype (CK) or monosomal karyotype (MK) are associated with clinical outcome in patients with MDS. METHODS: We investigated which prognostic factors including CAs would predict clinical outcomes in patients with International Prognostic Scoring System (IPSS) higher risk MDS treated with AZA, retrospectively. CK was defined as the presence of three or more numerical or structural CAs. MK was defined as the presence of two or more distinct autosomal monosomies or single autosomal monosomy with at least one additional structural CA. RESULTS: A total of 243 patients who treated with AZA, were enrolled. CK was present in 124 patients and MK was present in 90 patients. Bone marrow blasts > or =15% and CK were associated with poorer response (P=0.038, P=0.007) and overall survival (OS) (P<0.001, P<0.001) independently. Although MK in CK group was not associated with prognosis, non-MK status in non-CK group reflected favorable OS (P=0.005). The group including >3 CAs was associated with poorer OS (group including <3 CAs vs. only three CAs, P=0.001; group with >3 CAs vs. only three CAs, P=0.001). CONCLUSION: CK was an important prognostic parameter associated with worse outcome. MK may predict poor survival in only non-CK status. The higher number of CAs was associated with poorer survival.
Azacitidine* ; Bone Marrow ; Chromosome Aberrations ; Humans ; Karyotype* ; Monosomy ; Myelodysplastic Syndromes* ; Prognosis ; Retrospective Studies ; Stem Cell Transplantation

Adrenocortical carcinoma is a rare neoplasm and it has an invariably lethal prognosis. We report here on the fine needle aspiration cytologic findings of a solitary metastatic pulmonary adrenocortical carcinoma in a 24-year-old woman. The aspirate smears were very cellular and they were composed of a monomorphic population of large polyhedral cells with abundant granular or vacuolated cytoplasm, and the cells were predominantly singly scattered in a necrotic background. Multinucleated pleomorphic tumor cells were also found. Pleomorphic nuclei with thickened nuclear membranes were impinging on the cell membranes. Mitotic activity was occasionally seen. The cytologic findings of pleomorphic cells with microvacuolated cytoplasm and the presence of vague gland-like sheets, as well as the patient's history of undergoing adrenalectomy for primary adrenocortical carcinoma helped the pathologist reach the diagnosis of metastatic adrenocortical carcinoma. Here, we focus on the cytologic differential points of metastastic pulmonary adrenocortical carcinoma and primary pulmonary carcinoma, especially large cell carcinoma.
Adrenalectomy ; Adrenocortical Carcinoma ; Biopsy, Fine-Needle ; Carcinoma, Large Cell ; Cell Membrane ; Cytoplasm ; Female ; Humans ; Lung ; Lung Neoplasms ; Neoplasm Metastasis ; Nuclear Envelope ; Prognosis ; Young Adult

Adrenocortical carcinoma is a rare neoplasm and it has an invariably lethal prognosis. We report here on the fine needle aspiration cytologic findings of a solitary metastatic pulmonary adrenocortical carcinoma in a 24-year-old woman. The aspirate smears were very cellular and they were composed of a monomorphic population of large polyhedral cells with abundant granular or vacuolated cytoplasm, and the cells were predominantly singly scattered in a necrotic background. Multinucleated pleomorphic tumor cells were also found. Pleomorphic nuclei with thickened nuclear membranes were impinging on the cell membranes. Mitotic activity was occasionally seen. The cytologic findings of pleomorphic cells with microvacuolated cytoplasm and the presence of vague gland-like sheets, as well as the patient's history of undergoing adrenalectomy for primary adrenocortical carcinoma helped the pathologist reach the diagnosis of metastatic adrenocortical carcinoma. Here, we focus on the cytologic differential points of metastastic pulmonary adrenocortical carcinoma and primary pulmonary carcinoma, especially large cell carcinoma.
Adrenalectomy ; Adrenocortical Carcinoma ; Biopsy, Fine-Needle ; Carcinoma, Large Cell ; Cell Membrane ; Cytoplasm ; Female ; Humans ; Lung ; Lung Neoplasms ; Neoplasm Metastasis ; Nuclear Envelope ; Prognosis ; Young Adult

OBJECTIVES : The aims of this study are to estimate the prevalence of the DSM-IV psychiatric disorders in the Korean population using the Korean version of Composite International Diagnostic Interview (K-CIDI), and to compare those with previous studies. METHODS : The Korean Epidemiologic Catchment Area study Replication (KECA-R) was conducted between August 2006 and April 2007. The sampling of the subjects was carried out across 12 catchment areas. A multistage, cluster sampling design was adopted. The target population included all eligible residents aged 18 to 64 years. Face-to-face interviews were conducted with the Korean version of Composite International Diagnostic Interview (K-CIDI) based on the DSM-IV (N=6,510, response rate=81.7%). RESULTS : A total of 6,510 participants completed the interview. The lifetime and 12-month prevalence rates for all types of DSM-IV disorders were 30.0% and 17.3%, respectively. Those of specific disorders were as follows : 1) alcohol use disorder, 16.2% and 5.6% ; 2) nictotine use disorder, 9.0% and 6.0%;3) specific phobia, 3.8% and 3.4%;4) major depressive disorder, 5.6% and 2.5% ; and 5) generalized anxiety disorder, 1.6% and 0.8%. Data relating to nicotine and alcohol use disorder revealed a very high male/female ratio. Mood disorder and anxiety disorder were more prevalent among females than males. CONCLUSION : The prevalence of psychiatric disorders was high. In comparison with other studies, remarkable differences in the distribution of psychiatric disorders across the country and times were observed.
Adult ; Aged ; Anxiety Disorders ; Depressive Disorder, Major ; Diagnostic and Statistical Manual of Mental Disorders ; Female ; Health Services Needs and Demand ; Humans ; Male ; Mental Disorders ; Mood Disorders ; Nicotine ; Phobic Disorders ; Prevalence

Desmoid tumor (fibromatosis) is a histologically benign fibrous neoplasm showing locally infiltrating growth. This type of tumor commonly occurs in the abdomen, but intrathoracic desmoid tumor is uncommon. To date, 12 cases of intrathoracic desmoid tumor protruding into the pleural cavity, radiologically mimicking pleural masses, have been reported. Here, we report on a case of intrathoracic desmoid tumor protruding into the pleural cavity, and partially covered by parietal pleura. The main preoperative differential diagnoses included pleural solitary fibrous tumor, inflammatory pseudotumor or malignant mesothelioma. A near-total mass excision was performed. Pathologically, the tumor was composed of a paucicellular arrangement of spindle-shaped cells with fibromyxoid stroma. The resection margin was partially involved with spindle cells present. On histochemical staining, the spindle cells were strongly positive for vimentin and negative for CD34, consistent with a desmoid tumor. The patient was stable without further adjuvant treatment during 6-years of follow-up.
Abdomen ; Diagnosis, Differential ; Fibromatosis, Abdominal ; Fibromatosis, Aggressive ; Follow-Up Studies ; Granuloma, Plasma Cell ; Humans ; Mesothelioma ; Pleura ; Pleural Cavity ; Solitary Fibrous Tumors ; Vimentin