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Purpose To investigate the incidence and survival outcomes of primary ovarian sarcoma compared to those of epithelial ovarian cancer. Methods Data on primary ovarian sarcoma patients (n = 1361) and epithelial ovarian cancer patients (n = 30,366) between 1999 and 2017 were obtained from the Korea Central Cancer Registry, and their respective age-standardized incidence rate (ASR) and relative survival rate were calculated and compared. Results Based on the ASR, the incidence of epithelial ovarian cancer was 4.75 per 100,000 women, while that of primary ovarian sarcoma was 0.22 per 100,000 women. The ASR ratio was 21.94 without significant change of ASR during the study period. Primary ovarian sarcoma had a better survival curve compared with epithelial ovarian cancer, though the difference was not statistically significant (5 yr overall survival 64.0% vs. 61.5%; p = 0.6030). In addition, among the pure sarcomas, the fibrosarcoma histologic subtype showed the best overall survival, and that of liposarcomas and stromal cell sarcoma were behind that (5 yr overall survival 85.0%, 76.7%, and 72.7%; p Conclusions The incidence of primary ovarian sarcoma is quite low, with an ASR of 0.22/100,000 during the last 20 years. There were no significant differences between survival rates of primary ovarian sarcoma and epithelial ovarian cancer.

Malignant ovarian germ cell tumor (MOGCT) is a rare ovarian malignancy accounting for less than 5% of all ovarian cancers. We aimed to evaluate the incidence, survival, and subsequent malignancies after the diagnosis of MOGCT.Data from the Korea Central Cancer Registry were used to identify MOGCTs between 1999 and 2017. The age-standardized rates (ASRs), 5-year relative survival rates (RSR) and standardized incidence ratio (SIR) for subsequent cancer after diagnosis of MOGCT were estimated.Of 2125 cases of newly diagnosed MOGCTs, 596 (28.0%) were diagnosed with dysgerminoma and 1529 (72.0%) with non-dysgerminoma. The ASR per 100,000 women-years was 0.539; ASR slightly increased over the study period (annual percent change [APC] = 1.01%; p = 0.02). There was an increase and decrease in the incidence of MOGCTs in the age groups 0-19 years (APC = 1.96%; p0.01) and ≥ 50 years (APC = -6.51%; p0.01), respectively. Patients with dysgerminoma showed significantly higher RSR than patients with non-dysgerminoma (98.0% vs. 94.9%, p0.01). Patients aged ≥50 years showed worst 5-year RSR (68.7%) than patients aged 0-19 years (97.8%) and 20-34 years (96.4%) (p0.01). The overall SIR for a subsequent cancer occurrence was 2.07, with the most frequent site of subsequent primary cancer being the thyroid (SIR = 2.78).Our data demonstrated an excellent prognosis of MOGCTs among Korean women. There was a slight increase in MOGCT prevalence, which was more pronounced among those aged19 years. After MOGCT diagnosis, the risk of developing a subsequent malignancy was doubled compared with the general population.

Objective The three major gynecologic cancers are cervical, endometrial, and ovarian cancer. This study aimed to describe the 19-year trends and survival rates in cervical, endometrial, and ovarian cancer in a Korean female population.Methods We searched the Korea Central Cancer Registry to identify patients with gynecologic cancer between 1999 and 2017. Age-standardized rates and annual percent changes were calculated. The relative survival rate (RSR) was reported by histology, age, and stage for each gynecological cancer.Results The total number of cervical, endometrial, primary peritoneal, ovarian epithelial, fallopian tube (POFT) cancer was 134,863, with the number of cases increasing every year: 6,077 in 1999 to 8,011 in 2017. The incidence of cervical cancer has decreased; however, that of POFT and endometrial cancer has increased. The 5-year RSR of cervical, POFT, and endometrial cancer was reported to be 80.8%, 61.4%, and 88.1%, respectively. In the case of cervical cancer, squamous cell carcinoma showed better survival than other histology (82.8% vs. 73.5%). Furthermore, in the case of endometrial cancer, endometrioid histology had substantially better 5-year RSR than the others (93.2% vs. 76.5%). Contrastingly, in the case of ovarian cancer, serous carcinoma had worse 5-year RSR than other types of histology.Conclusion The incidence rates for gynecologic cancers increased from 2005 to 2017, with an annual increase of 2.76 per year until 2017. Endometrial cancer had the highest RSR, while ovarian cancer had the lowest. Active cancer screening and the introduction of effective treatments might have contributed to the improved RSRs of gynecologic cancers.

Background South Korea has the highest incidence of thyroid cancer in the world. Our study examined the trends in thyroid cancer incidence by the histologic type, cancer stage, and age group and explored possible factors that affected thyroid cancer trends. Methods We conducted a descriptive epidemiological study using the national cancer registry data and cause of death data from 1999 to 2016 in South Korea. Age‐standardized rates were calculated using Segi's world standard population. Joinpoint regression analysis was applied to determine the changing point of thyroid cancer trends according to histologic type; Surveillance, Epidemiology, and End Results (SEER) summary stage; and age groups by sex. Results The age‐standardized incidence of thyroid cancer in both men and women increased from 6.3 per 100,000 people in 1999 to 63.4 per 100,000 in 2012 but declined from 2012 to 2016, before the debates for over diagnosis of thyroid cancer began in 2014. The age‐standardized mortality rate of thyroid cancer, incidence of distant thyroid cancer, and incidence of regional and localized thyroid cancer started to decline since early 2000, 2010, and 2012, respectively. In addition, thyroid cancer prevalence in thyroid nodules showed decreasing trends from 1999–2000 to 2013–2014. Conclusions The incidence of thyroid cancer began declining from 2012, before the debates for over diagnosis of thyroid cancer began in 2014. Changes in guidelines for thyroid nodule examinations may have affected this inflection point. Moreover, the debates for over diagnosis of thyroid cancer may have accelerated the decline in thyroid cancer., The incidence of thyroid cancer in South Korea has peaked in 2012 and started to decline thereafter. The reduction in thyroid cancer incidence was accelerated by debates for over diagnosis of thyroid cancer in 2014, but changes in the trends of thyroid cancer by stage have already been observed before 2013.

Purpose Hospital-based clinical studies have limitations in holistic assessment of cancer treatment and prognosis, as they omit out-of-hospital patients including elderly individuals. This study aimed to investigate trends in initial treatment and corresponding prognosis of patients with exocrine pancreatic cancer (EPC) in Korea. Materials and Methods The Korea Central Cancer Registry data of patients with EPC from 2006 to 2017 were retrospectively reviewed. We defined the first course of treatment (FT) as the cancer-directed treatment administered within four months after cancer diagnosis according to Surveillance, Epidemiology, and End results (SEER) program. Results Among 62,209 patients with EPC, localized and regional (LR) SEER stage; patients over 70 years old; and ductal adenocarcinoma excluding cystic or mucinous (DAC) accounted for 40.6%, 50.1%, and 95.9%, respectively. "No active treatment" (NT, 46.5%) was the most frequent, followed by non-surgical FT (28.7%) and surgical FT (22.0%). Among 25,198 patients with LR EPC, surgical FT increased (35.9% to 46.3%) and NT decreased (45.0% to 29.5%) from 2006 to 2017. The rate of surgical FT was inversely related to age (55.1% [

B cells in the germinal center (GC) are programmed to form plasma cells (PCs) or memory B cells according to signals received by receptors that are translated to carry out appropriate activities of transcription factors. However, the precise mechanism underlying this process to complete the GC reaction is unclear. In this study, we show that both genetic ablation and pharmacological inhibition of glycogen synthase kinase 3 (GSK3) in GC B cells of mice facilitate the cell fate decision toward PC formation, accompanied by acquisition of dark zone B cell properties. Mechanistically, under stimulation with CD40L and IL-21, GSK3 inactivation synergistically induced the transcription factors Foxo1 and c-Myc, leading to increased levels of key transcription factors required for PC differentiation, including IRF4. This GSK3-mediated alteration of transcriptional factors in turn facilitated the dark zone transition and consequent PC fate commitment. Our study thus reveals the upstream master regulator responsible for interpreting external cues in GC B cells to form PCs mediated by key transcription factors.

BACKGROUND: This study investigates the effects of a neuropeptide, secretoneurin (SN), on bone regeneration in an experimental mouse model. METHODS: The effects of SN on cell proliferation, osteoblast marker genes expression, and mineralization were evaluated using the CCK-8 assay, quantitative reverse transcriptase polymerase chain reaction (RT-PCR), and alizarin red S staining, respectively. To examine the effects of SN on bone regeneration in vivo, bone defects were created in the calvaria of ICR mice, and 0.5 or 1 µg/ml SN was applied. New bone formation was analyzed by micro-computed tomography (micro-CT) and histology. New blood vessel formation was assessed by CD34 immunohistochemistry. RESULTS: SN had no significant effect on proliferation and mineralization of MC3T3-E1 cells. However, SN partially induced the gene expression of osteoblast differentiation markers such as runt-related transcription factor 2, alkaline phosphatase, collagen type I alpha 1, and osteopontin. A significant increase of bone regeneration was observed in SN treated calvarial defects. The bone volume (BV), BV/tissue volume, trabecular thickness and trabecular number values were significantly increased in the collagen sponge plus 0.5 or 1 µg/ml SN group (p

Purpose A prior history of breast cancer is a risk factor for the subsequent development of primary peritoneal, epithelial ovarian, and fallopian tubal (POFT) cancers. This study aimed to estimate the incidence of secondary POFT malignancy in breast cancer patients and the clinical outcomes of primary and secondary POFT cancer.Materials and Methods We searched the Korea Central Cancer Registry to find patients with primary and secondary POFT cancer who had breast cancer in 1999-2017. The incidence rate and standardized incidence ratio were calculated. Additionally, we compared the overall survival of patients with primary and secondary POFT cancer.Results Based on the age-standardized rate, the incidence of second primary POFT cancer after breast cancer was 0.0763 per 100,000 women, which increased in Korea between 1999 and 2017. Among the 30,366 POFT cancer patients, 25,721 were primary POFT cancer only, and 493 had secondary POFT cancer after a breast cancer diagnosis. Second primary POFT cancer patients were older at the time of diagnosis (55 vs. 53, p < 0.001) and had a larger proportion of serous histology (68.4% vs. 51.2%, p < 0.001) than patients with primary POFT. There were no differences between the two groups in tumor stage at diagnosis. The 5-year overall survival rates were 60.2% and 56.3% for primary and secondary POFT cancer, respectively (p=0.216).Conclusion The incidence of second primary POFT cancer after breast cancer increased in Korea between 1999 and 2017. Besides, second primary POFT cancer patients were diagnosed at older ages and had more serous histology.

PurposeThis study investigated the incidence and relative survival rates (RSRs) for cancers among adolescents and young adults (AYAs) aged 15-39 years between 1993 and 2016 in KoreaMaterials and MethodsData from the Korea Central Cancer Registry were used to calculate percent distributions, age-specific incidence rates, age-standardized incidence rates (ASRs) per million, annual percent changes (APCs), average APCs, and RSRs for cancers diagnosed in AYAs.Results ASR of all cancers among AYAs was 654.5 per million. The largest diagnosed group of cancers was carcinomas (almost 80%). Crude incidence increased with age, from 170.4 per million for those aged 15-19 years to 1,639.8 per million for those aged 35-39 years. ASR increased from 414.8 per million to 820.4 per million, with an APC of 9.0%. The incidence of thyroid carcinoma showed the most rapid increment (APC, 14.0%), followed by non-Hodgkin lymphoma (APC, 13.4%). The 5-year RSR among AYAs significantly improved from 62.1% to 90.8%. Survival improvement in AYAs was higher than that in children but lower than that in older adults (APC, 2.1% vs. 1.9% vs. 3.1%). The most marked survival improvement was found for leukemia and lymphoma. Astrocytoma, rhabdomyosarcoma, and carcinoma of the trachea, bronchus, and lung had a 5-year RSR of < 50%.ConclusionThere was an improvement in cancer survivals in AYAs, comparable to that achieved in children. However, survivals in several cancer types do not appear to be improving. Further research focusing on the epidemiology and therapeutic strategies for cancers in AYAs is needed.

Background/AimsLymphomas are the most frequent neoplasm of the orbit. However, the epidemiology of orbital lymphomas is not well reported. This study aimed to provide a population-based report on the epidemiology of orbital lymphomas and measure the trends in the incidence of orbital lymphoma cancer in South Korea.MethodsNationwide cancer incidence data from 1999 to 2016 were obtained from the Korea Central Cancer Registry. Age-standardised incidence rates and annual percent changes were calculated according to sex and histological types. The analysis according to the Surveillance, Epidemiology, and End Results summary stage classifications was performed from 2006 to 2016. Survival rates were estimated for cases diagnosed from 1999 to 2016.ResultsA total of 630 patients (median age: 54 years) with orbital lymphoma in the orbital soft tissue were included in this study. The age-standardised incidence rates increased from 0.03 to 0.08 per 100 000 individuals between 1999 and 2016, with an annual percent change of 6.61%. The most common histopathological type of orbital lymphoma was extra marginal zone B cell lymphoma, accounting for 82.2% of all orbital lymphomas during 1999–2016, followed by diffuse large B cell lymphoma (9.2%). Five-year, 10-year and 15-year overall survival (OS) of orbital lymphoma was 90.8%, 83.8% and 75.8%, respectively. OS showed a significant decrease as age increased and no significant differences between men and women.ConclusionThe incidence rate of orbital lymphoma is very low in South Korea. However, the incidence rate has increased over the past years. Orbital lymphomas have a worse prognosis as age increases.

Tooth root development occurs through the interaction of multiple growth factors and transcription factors expressed in Hertwig's epithelial root sheath (HERS) and dental mesenchyme. Previously, we demonstrated that bobby sox homolog (Bbx) regulates odontoblast differentiation of human dental pulp stem cells. Here, we generated Bbx knockout (Bbx-/- ) mice to address the functional role of Bbx in tooth formation. During tooth development, Bbx was expressed in both dental epithelium and mesenchyme. However, molar and incisor morphology in Bbx-/- mice at postnatal Day 0 (P0) exhibited no prominent abnormalities compared with their wild-type (Bbx+/+ ) littermates. Until P28, the crown morphology in Bbx-/- mice was not distinctively different from Bbx+/+ littermates. Meanwhile, the length of the mandibular base in Bbx-/- mice was notably less at P28. Compared with Bbx+/+ mice, the mesial and distal root lengths of the first molar were reduced by 21.33% and 16.28% at P14 and 16.28% and 16.24% at P28, respectively, in Bbx-/- mice. The second molar of Bbx-/- mice also showed 10.16% and 6.4% reductions at P28 in the mesial and distal lengths, compared with Bbx+/+ mice, respectively. The gene expression analysis during early tooth root formation (P13) showed that the expression of dentin sialophosphoprotein (Dspp) was significantly decreased in Bbx-/- mice. Collectively, our data suggest that Bbx participates in tooth root formation and might be associated with the regulation of Dspp expression.

BACKGROUND: The neural regulation of bone regeneration has emerged recently. Spexin (SPX) is a novel neuropeptide and regulates multiple biological functions. However, the effects of SPX on osteogenic differentiation need to be further investigated. Therefore, the aim of this study is to investigate the effects of SPX on osteogenic differentiation, possible underlying mechanisms, and bone regeneration. METHODS: In this study, MC3T3-E1 cells were treated with various concentrations of SPX. Cell proliferation, osteogenic differentiation marker expressions, alkaline phosphatase (ALP) activity, and mineralization were evaluated using the CCK-8 assay, reverse transcriptase-quantitative polymerase chain reaction (RT-qPCR), ALP staining, and alizarin red S staining, respectively. To determine the underlying molecular mechanism of SPX, the phosphorylation levels of signaling molecules were examined via western blot analysis. Moreover, in vivo bone regeneration by SPX (0.5 and 1 µg/µl) was evaluated in a calvarial defect model. New bone formation was analyzed using micro-computed tomography (micro-CT) and histology. RESULTS: The results indicated that cell proliferation was not affected by SPX. However, SPX significantly increased ALP activity, mineralization, and the expression of genes for osteogenic differentiation markers, including runt-related transcription factor 2 (Runx2), Alp, collagen alpha-1(I) chain (Col1a1), osteocalcin (Oc), and bone sialoprotein (Bsp). In contrast, SPX downregulated the expression of ectonucleotide pyrophosphatase/phosphodiesterase 1 (Enpp1). Moreover, SPX upregulated phosphorylated mitogen-activated protein kinase kinase (MEK1/2) and extracellular signal-regulated kinase (ERK1/2). In vivo studies, micro-CT and histologic analysis revealed that SPX markedly increased a new bone formation. CONCLUSION: Overall, these results demonstrated that SPX stimulated osteogenic differentiation in vitro and increased in vivo bone regeneration via the MEK/ERK pathway. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s13770-021-00408-2.

Biliary tract cancer (BTC) has been inconsistently identified according to its location in epidemiological and clinical studies. This study retrospectively reviewed the treatment pattern and prognosis of BTC according to tumor location using the Korea Central Cancer Registry data of 97,676 patients with BTC from 2006 to 2017. The proportion of localized and regional Surveillance, Epidemiology, and End Results (SEER) stage was the highest in ampulla of Vater (AoV, 78.2%) cancer, followed by extrahepatic bile duct (BD, 68.3%), gallbladder (GB, 52.6%), and intrahepatic BD (49.5%) cancers. Overall, the “no active anti-cancer treatment” rate was the highest in intrahepatic BD (52.8%), followed by extrahepatic BD (49.5%), GB (39.6%), and AoV cancers (28.9%). The 5-year relative survival rate was the highest in AoV (48.5%), followed by GB (28.5%), extrahepatic BD (19.9%), and intrahepatic BD (10.8%) cancers, which significantly improved over time, except for intrahepatic BD cancer. In the localized and regional stage, older patients had a higher risk of receiving no active anti-cancer treatment in each tumor location after adjusting for period and sex. BTC statistics should be reported separately according to tumor location due to its distinct SEER stage distribution, treatment pattern, and prognosis. Care should be taken in elderly patients to reduce the rate of no active anti-cancer treatment.

Background: Second primary cancer has become an important issue among cancer survivors. This study sought to determine the differences in clinicopathologic outcomes between second primary breast cancer (SPBC) after ovarian cancer and primary breast cancer (PBC) in Korea.Methods: We searched the Korea Central Cancer Registry and identified 251,244 breast cancer cases that were diagnosed between 1999 and 2017. Demographic and clinical characteristics and overall survival (OS) rates were estimated according to age, histological type, and cancer stage.Results: Among the 228,329 patients included, 228,148 were patients with PBC and 181 were had SPBC, diagnosed after ovarian cancer (OC). The most common ages at diagnosis were 50‒59 years for SPBC and 40–49 years for PBC. Patients with SPBC were significantly less likely than patients with PBC to receive adjuvant radiotherapy (14.92% vs. 21.92%, p = 0.0228) or adjuvant chemotherapy (44.75% vs. 55.69%, p = 0.0031). Based on the age-standardized rate (ASR), the incidence of SPBC after OC was 293.58 per 100,000 ovarian patients and the incidence of PBC was 39.13 per 100,000 women. The 5-year OS rates were 72.88% and 89.37% for SPBC and PBC (p < 0.0001). The OS rate in SPBC decreased significantly with advanced stage and older age.Conclusion: The incidence of breast cancer is about 1.27 times higher in ovarian cancer patients than in healthy people. The survival outcomes were worse for SPBC than for PBC and were related to older age and advanced stage. Active screening for breast cancer is necessary in ovarian cancer patients.

Emphysema is an important feature of chronic obstructive pulmonary disease (COPD). Genetic factors likely affect emphysema pathogenesis, but this question has predominantly been studied in those of European ancestry. In this study, we sought to determine genetic components of emphysema severity and characterize the potential function of the associated loci in Korean population. We performed a genome-wide association study (GWAS) on quantitative emphysema in subjects with or without COPD from two Korean COPD cohorts. We investigated the functional consequences of the loci using epigenetic annotation and gene expression data. We also compared our GWAS results with an epigenome-wide association study and previous differential gene expression analysis. In total, 548 subjects (476 [86.9%] male) including 514 COPD patients were evaluated. We identified one genome-wide significant SNP (P –8), rs117084279, near PIBF1. We identified an additional 57 SNPs (P –6) associated with emphysema in all subjects, and 106 SNPs (P –6) in COPD patients. Of these candidate SNPs, 2 (rs12459249, rs11667314) near CYP2A6 were expression quantitative trait loci in lung tissue and a SNP (rs11214944) near NNMT was an expression quantitative trait locus in whole blood. Of note, rs11214944 was in linkage disequilibrium with variants in enhancer histone marks in lung tissue. Several genes near additional SNPs were identified in our previous EWAS study with nominal level of significance. We identified a novel SNP associated with quantitative emphysema on CT. Including the novel SNP, several candidate SNPs in our study may provide clues to the genetic etiology of emphysema in Asian populations. Further research and validation of the loci will help determine the genetic factors for the development of emphysema.

The epidemiology of osteosarcoma in adolescents and young adults (AYA) remains unclear. We aimed to assess and compare the clinical features of osteosarcoma between AYA and other age groups. We retrieved osteosarcoma cases diagnosed between 1999 and 2017 from the Korea Central Cancer Registry. We compared survival trends and clinical characteristics between AYA and other age groups. AYA comprised 43.3% (1309/3022) of the osteosarcoma cases. Compared to other age groups, the male-to-female ratio was highest in AYA (1.61:1). The proportion of tumors located in an extremity was 80.3% in AYA, which was lower than in young children (92.5%) or pubertal children (93.8%) but higher than in adults (55.7%) or the elderly (47.5%). As for treatments, 71.2% of AYA received local treatment and systemic chemotherapy, and 28.8% received only local treatment (surgery: 261, radiotherapy: 9, surgery and radiotherapy: 5). The 5-year overall survival (OS) was lower in AYA (68%) than in young children (78%) or pubertal children (73%) but higher than in adults (47%) or the elderly (25%). When AYA were divided into five subgroups by age, patients aged 15–19 years constituted the largest proportion (45.4%, n = 594). Additionally, the proportion of patients with a non-extremity tumor increased in an age-dependent manner, from 10.3% in AYA aged 15–19 years to 35.3% in AYA aged 35–39 years. OS did not significantly differ among the different age subgroups of AYA. The clinical characteristics and OS of the AYA were more similar to those of children than to those of adults. There is a need for cooperation between pediatric and adult oncologists for effective osteosarcoma treatment in AYA.

The improved survival of patients with oral cavity cancer (OCC) has generated interest in factors affecting survivorship, particularly among second primary cancer (SPC) patients. This study aimed to assess the incidence, patterns, and risk factors for SPC after OCC treatment in the Korean population.Data from 15,261 patients with OCC (ICD-O: C01-C06) identified between 1993 and 2014 were extracted from the Korean Central Cancer Registry. The standardized incidence ratio (SIR) for SPC after index OCC was calculated, and Poisson regression analysis was performed to evaluate the risk factors for SPC among survivors.The overall SIR for SPC among OCC survivors was 1.47 (95% confidence interval [CI] 1.39-1.56). SIR differed by sex (male: 1.51 vs. female: 1.37), age at diagnosis (45 years: 2.47 vs. 45-64 years: 1.68 vs. ≥ 65 years: 1.10), index OCC subsite (floor of mouth: 1.95 vs. gum: 1.30), follow-up duration (6-23 months: 1.64 vs. 24-59 months: 1.51 vs. 60-119 months: 1.48 vs. ≥ 120 months: 1.24), histological OCC type (salivary gland malignancy: 1.77 vs. squamous cell carcinoma: 1.44 vs. others: 1.47), and radiation history (any: 1.94 vs. no radiation: 1.37). The risk factors for SPC development among OCC survivors included younger age at diagnosis and history of radiation therapy.OCC survivors have significantly increased risks of SPCs, exhibiting distinctive site distributions and chronological patterns. These patients would benefit from an SPC surveillance protocol.

Background Among sarcomas, which are rare cancers, many types are exceedingly rare; however, a definition of ultra-rare cancers has not been established. The problem of ultra-rare sarcomas is particularly relevant because they represent unique diseases, and their rarity poses major challenges for diagnosis, understanding disease biology, generating clinical evidence to support new drug development, and achieving formal authorization for novel therapies. Methods The Connective Tissue Oncology Society promoted a consensus effort in November 2019 to establish how to define ultra-rare sarcomas through expert consensus and epidemiologic data and to work out a comprehensive list of these diseases. The list of ultra-rare sarcomas was based on the 2020 World Health Organization classification, The incidence rates were estimated using the Information Network on Rare Cancers (RARECARENet) database and NETSARC (the French Sarcoma Network's clinical-pathologic registry). Incidence rates were further validated in collaboration with the Asian cancer registries of Japan, Korea, and Taiwan. Results It was agreed that the best criterion for a definition of ultra-rare sarcomas would be incidence. Ultra-rare sarcomas were defined as those with an incidence of approximately ≤1 per 1,000,000, to include those entities whose rarity renders them extremely difficult to conduct well powered, prospective clinical studies. On the basis of this threshold, a list of ultra-rare sarcomas was defined, which comprised 56 soft tissue sarcoma types and 21 bone sarcoma types. Conclusions Altogether, the incidence of ultra-rare sarcomas accounts for roughly 20% of all soft tissue and bone sarcomas. This confirms that the challenges inherent in ultra-rare sarcomas affect large numbers of patients.

Purpose Due to low incidence, epidemiologic data of Ewing sarcoma in the Asian population are scarce. We aimed to examine the incidence pattern and outcome of patients with Ewing sarcoma in the Republic of Korea. Materials and Methods Data of patients with Ewing sarcoma diagnosed between 1999 and 2017 were obtained from the Korea Central Cancer Registry (KCCR). Incidence, clinical characteristics, and survival rates were analyzed and compared between different age groups. Results There were 788 cases (459 male, 329 female), with a median age at diagnosis of 20 years. The age-standardized rate of Ewing sarcoma was 1.01. The number of cases and incidence rates in each age group were as follows: children, 1.6; adolescents and young adults, 0.93; adults, 0.44; and elderly, 0.53. There were more male cases in children and the adolescents and young adults (AYA) group (p

Background Cancers of unknown primary (CUPs) are tumors found after metastasizing from unidentified primary sites; these tumors generally have unknown treatment strategies, expected treatment results, and prognosis. We assessed the epidemiological characteristics of CUPs in Korea. Methods We extracted records for 1999 through 2017 from the Korea Central Cancer Registry using the International Statistical Classification of Diseases and Related Health Problems (10th revision) codes for CUP as defined by the International Agency for Research on Cancer. Age-standardized rates and relative survival rates were calculated. Results The CUPs constituted 2.1 % of the total number of cancer registrations in 1999, declining to 0.7 % in 2017. The incidence rate decreased for both sexes (5.35 to 2.20 for men, 3.15 to 1.77 for women). Patients aged 80 years and older had the highest incidence rate at 40.2, and 86.3 % of CUPs occurred in those 50 years of age or older. The cases of retroperitoneum and peritoneum sites increased over time. Cases diagnosed by microscopic methods and death certification only were 62.3 % and 7.9 %, respectively. The malignant neoplasm of the retroperitoneum and peritoneum and unknown primary site had the highest and lowest survival rates, respectively. The 5-year relative survival rate increased over time from 14.2 % (1999–2002) to 27.3 % (2013–2017). Conclusions Our analysis of data from the Korea Central Cancer Registry found decreasing rates of CUP, although with consistent disparities by patient age and sex. Advancements in diagnostic technology may be decreasing the number of CUP diagnoses. Expanding the amount of information recorded in the registry may further improve diagnostic accuracy.

BACKGROUND Misclassification of Klatskin tumor as intrahepatic rather than extrahepatic bile duct cancer (eBDC) may overestimate the incidence of intrahepatic BDC (iBDC). This study aimed to investigate the impact of misclassified Klatskin tumor on iBDC and eBDC incidences in Korea, where BDC is highly endemic. METHODS The data of incidence, topography and morphology code of BDC from 1999 to 2017 were obtained from the Korea Central Cancer Registry, which covers the entire 51.6 million Korean population. For misclassification analysis, all Klatskin tumors were reclassified as eBDC. RESULTS Klatskin tumors accounted for 13.5% of all 81 414 BDC cases. In the registry, an average of 59.7% of Klatskin tumors were classified as iBDC, gradually decreasing from 95.5% to 16.9%. Misclassification led to a 17.3% overestimation of iBDC cases and a 15.0% underestimation of eBDC cases on average. After reclassification, age standardized rate (ASR) of incidence per 100 000 population decreased in iBDC from 3.4 to 2.9 and increased in eBDC from 2.8 to 3.2. Average annual percentage change of iBDC and eBDC incidences were 2.0% and 1.2%, respectively, but ASR of iBDC significantly decreased since 2012 (P

This study provides a population-based report on eyelid skin cancer epidemiology showing trends in its incidence and survival in South Korea.For this population-based epidemiological study, nationwide cancer incidence data were obtained from the Korea Central Cancer Registry, covering the entire population. Age-standardised incidence rates (ASRs) and annual per cent changes (APCs) were calculated according to sex, histological types, stage, and date of diagnosis. The 5-year relative survival rates (RSRs) were estimated for patients diagnosed between 1993 and 2016.The ASR increased from 0.27 per 100,000 population in 1999 to 0.61 in 2016, with an APC of 4.94%. The most common histopathological type was basal cell carcinoma (BCC), accounting for 67.5% of all eyelid skin cancers during 1999-2016, followed by sebaceous gland carcinoma (SGC, 10.7%), and squamous cell carcinoma (SCC, 10.6%). The number of localised cancer diagnoses tended to increase, compared to that of distant cancer. The 5-year RSR was nearly 100% when considering all eyelid skin cancers together. When considering them separately, the 5-year RSR of BCC was nearly 100% throughout, whereas for SGC and SCC, it gradually increased from 94.2% and 87.7% (1993-1995), respectively, to nearly 100% (2011-2016) for both. Melanoma had the lowest survival, although its survival has increased markedly since 1993.Nationwide data used in this study showed an increase in incidence and survival rates of eyelid skin cancers in Korea. BCC was the most predominant type, and its proportion among all eyelid skin cancer cases has increased.

Objective Primary peritoneal cancer (PPC), ovarian cancer (OC), and fallopian tube cancer (FTC) are considered as a single disease group. As knowledge of the pathogenesis and clinical presentation of peritoneal, ovarian, and fallopian tube (POFT) cancer grows, the tendencies in OC diagnosis are changing. We investigate the incidence and clinical characteristics of epithelial POFT based on cancer site and histologic type. Methods Data from the Korea Central Cancer Registry for the period between 1999 and 2016 were analyzed. The incidence rates and annual percent changes (APCs) for each tumor site were reported. Results Among 27,768 women with cancer, 1,086 (3.91%) had PPC, 25,847 (93.08%) had OC, and 835 (3.01%) had FTC. Age-standardized rates increased from 0.05 to 0.24, 3.51 to 5.48, and 0.04 to 0.28 in PPC, OC, and FTC, respectively. The proportion of PPC and FTC among all the POFT cases increased consistently during the study period (from, respectively, 1.48 and 1.06 in 1999 to 4.52 and 4.76 in 2016). The APC of PPC, OC, and FTC during 1999-2016 was 9.3%, 2.7%, and 8.6%, respectively. The incidence of PPC, OC, and FTC was highest among patients in the 65-69, 50-54, and 55-59 years age group, respectively. Conclusion The overall incidence of PPC, OC, and FTC cancer has steadily increased. The relative increase of PPC and FTC has been significant. In this study, OC incidence had a relatively young peak age, in contrast to FTC and PPC, which had an older peak age.

Postmenopausal osteoporosis is closely associated with excessive osteoclast formation and function, resulting in the loss of bone mass. Osteoclast-targeting agents have been developed to manage this disease. We examined the effects of ciclopirox on osteoclast differentiation and bone resorption in vitro and in vivo. Ciclopirox significantly inhibited osteoclast formation from primary murine bone marrow macrophages (BMMs) in response to receptor activator of nuclear factor kappa B ligand (RANKL), and the expression of genes associated with osteoclastogenesis and function was decreased. The formation of actin rings and resorption pits was suppressed by ciclopirox. Analysis of RANKL-mediated early signaling events in BMMs revealed that ciclopirox attenuates IκBα phosphorylation without affecting mitogen-activated protein kinase activation. Furthermore, the administration of ciclopirox suppressed osteoclast formation and bone loss in ovariectomy-induced osteoporosis in mice and reduced serum levels of osteocalcin and C-terminal telopeptide fragment of type I collagen C-terminus. These results indicate that ciclopirox exhibits antiosteoclastogenic activity both in vitro and in vivo and represents a new candidate compound for protection against osteoporosis and other osteoclast-related bone diseases.