Background: This study aimed to investigate the incidence and clinical significance of segmental chromosomal aberrations (SCAs) in Korean patients with neuroblastoma. Methods: Patients diagnosed with neuroblastoma from 2012 to 2018 were included for retrospective review. Fluorescence in situ hybridization (FISH) was used to analyze four SCAs (MYCN amplification, 1p deletion, 11q deletion, and 17q gain). Clinical characteristics at diagnosis, early tumor response (reduction in primary tumor volume and neuron-specific enolase level after the first three cycles of chemotherapy), and survival rates were compared according to SCAs. Results: Among 173 patients with FISH results, 92 (53.2%) had at least one of the four SCAs, while 25 (14.5%) had two co-aberrations, and eight (4.6%) had three co-aberrations. SCAs detected in our study were MYCN amplification (n = 17, 9.8%), 1p deletion (n = 26, 15.2%), 11q deletion (n = 44, 25.6%), and 17q gain (n = 46, 27.1%). Patients with MYCN amplification showed a better early response but a worse survival than those without (5-year overall survival: 46.2% ± 13.1% vs. 88.6% ± 3.4%). Furthermore, 1p deletion was associated with a better early response but a worse survival; however, it was not an independent factor for survival. We could not find any prognostic significance associated with 11q deletion or 17q gain. Conclusion This is the first study investigating SCAs in Korean neuroblastoma patients. Prognostic significance of SCAs other than MYCN amplification was different from those reported in western countries. Further study with a larger cohort and longer follow-up is needed to confirm our findings.

Xanthogranulomatous pyelonephritis (XP) is an uncommon, chronic, destructive, granulomatous disease of the renal parenchyma. The condition is generally associated with long-term urinary tract obstruction and infection, although the precise etiology of disease remains unknown. The condition often mimics neoplastic and inflammatory diseases, and XP associated with actinomycosis is extremely uncommon. A 60 year-old male was admitted complaining of abdominal pain and weight loss. His radiological findings were suggestive of metastatic kidney cancer, and we performed radical laparoscopic nephrectomy and mesenterectomy for diagnostic purposes. Pathologically, actinomycosis combined with XP was evident. We report here a rare case of XP associated with actinomycosis.
Abdominal Pain ; Actinomycosis* ; Humans ; Kidney Neoplasms* ; Kidney* ; Male ; Nephrectomy ; Pyelonephritis ; Pyelonephritis, Xanthogranulomatous* ; Urinary Tract ; Weight Loss

Although the number of studies using tandem high-dose chemotherapy and autologous stem cell transplantation (HDCT/autoSCT) for the treatment of high-risk pediatric solid tumors has been increasing, documentation of hematologic recovery after tandem HDCT/autoSCT is very limited. For this reason, we retrospectively analyzed the hematologic recovery of 236 children with high-risk solid tumors who underwent tandem HDCT/autoSCT. The median numbers of CD34+ cells transplanted during the first and second HDCT/autoSCT were 4.3 x 10(6)/kg (range 0.6-220.2) and 4.1 x 10(6)/kg (range 0.9-157.6), respectively (P = 0.664). While there was no difference in neutrophil recovery between the first and second HDCT/autoSCT, platelet and RBC recoveries were significantly delayed in the second HDCT/autoSCT (P < 0.001 and P < 0.001, respectively). Delayed recovery in the second HDCT/autoSCT was more prominent when the number of transplanted CD34+ cells was lower, especially if it was < 2 x 10(6)/kg. A lower CD34+ cell count was also associated with increased RBC transfusion requirements and a higher serum ferritin level after tandem HDCT/autoSCT. More CD34+ cells need to be transplanted during the second HDCT/autoSCT in order to achieve the same hematologic recovery as the first HDCT/autoSCT.
Adolescent ; Antigens, CD34/metabolism ; Antineoplastic Combined Chemotherapy Protocols/*therapeutic use ; Blood Cell Count ; Blood Platelets/cytology ; Child ; Child, Preschool ; Combined Modality Therapy ; Erythrocytes/cytology ; Female ; Ferritins/blood ; Humans ; Infant ; Male ; Neoplasms/*drug therapy ; Neutrophils/cytology ; Retrospective Studies ; *Stem Cell Transplantation ; Stem Cells/cytology/metabolism ; Transplantation, Autologous ; Young Adult

PURPOSE: This study compared outcomes in children with acute leukemia who underwent transplantations with umbilical cord blood (UCB), bone marrow, or peripheral blood stem cells from a human leukocyte antigen (HLA)-matched related donor (MRD) or an unrelated donor (URD). METHODS: This retrospective study included consecutive acute leukemia patients who underwent their first allogeneic hematopoietic stem cell transplantation (HSCT) at Samsung Medical Center between 2005 and 2010. Patients received stem cells from MRD (n=33), URD (n=46), or UCB (n=41). RESULTS: Neutrophil and platelet recovery were significantly longer after HSCT with UCB than with MRD or URD (P<0.01 for both). In multivariate analysis using the MRD group as a reference, the URD group had a significantly higher risk of grade III to IV acute graft-versus-host disease (GVHD; relative risk [RR], 15.2; 95% confidence interval [CI], 1.2 to 186.2; P=0.03) and extensive chronic GVHD (RR, 6.9; 95% CI, 1.9 to 25.2; P<0.01). For all 3 donor types, 5-year event-free survival (EFS) and overall survival were similar. Extensive chronic GVHD was associated with fewer relapses (RR, 0.1; 95% CI, 0.04 to 0.6; P<0.01). Multivariate analysis showed that lower EFS was associated with advanced disease at transplantation (RR, 3.2; 95% CI, 1.3 to 7.8; P<0.01) and total body irradiation (RR, 2.1; 95% CI, 1.0 to 4.3; P=0.04). CONCLUSION: Survival after UCB transplantation was similar to survival after MRD and URD transplantation. For patients lacking an HLA matched donor, the use of UCB is a suitable alternative.
Blood Platelets ; Bone Marrow ; Child ; Disease-Free Survival ; Fetal Blood ; Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation ; Hematopoietic Stem Cells ; Humans ; Leukemia ; Leukocytes ; Multivariate Analysis ; Neutrophils ; Recurrence ; Retrospective Studies ; Stem Cells ; Tissue Donors ; Transplants ; Umbilical Cord ; Unrelated Donors ; Whole-Body Irradiation

PURPOSE: This study compared outcomes in children with acute leukemia who underwent transplantations with umbilical cord blood (UCB), bone marrow, or peripheral blood stem cells from a human leukocyte antigen (HLA)-matched related donor (MRD) or an unrelated donor (URD). METHODS: This retrospective study included consecutive acute leukemia patients who underwent their first allogeneic hematopoietic stem cell transplantation (HSCT) at Samsung Medical Center between 2005 and 2010. Patients received stem cells from MRD (n=33), URD (n=46), or UCB (n=41). RESULTS: Neutrophil and platelet recovery were significantly longer after HSCT with UCB than with MRD or URD (P<0.01 for both). In multivariate analysis using the MRD group as a reference, the URD group had a significantly higher risk of grade III to IV acute graft-versus-host disease (GVHD; relative risk [RR], 15.2; 95% confidence interval [CI], 1.2 to 186.2; P=0.03) and extensive chronic GVHD (RR, 6.9; 95% CI, 1.9 to 25.2; P<0.01). For all 3 donor types, 5-year event-free survival (EFS) and overall survival were similar. Extensive chronic GVHD was associated with fewer relapses (RR, 0.1; 95% CI, 0.04 to 0.6; P<0.01). Multivariate analysis showed that lower EFS was associated with advanced disease at transplantation (RR, 3.2; 95% CI, 1.3 to 7.8; P<0.01) and total body irradiation (RR, 2.1; 95% CI, 1.0 to 4.3; P=0.04). CONCLUSION: Survival after UCB transplantation was similar to survival after MRD and URD transplantation. For patients lacking an HLA matched donor, the use of UCB is a suitable alternative.
Blood Platelets ; Bone Marrow ; Child ; Disease-Free Survival ; Fetal Blood ; Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation ; Hematopoietic Stem Cells ; Humans ; Leukemia ; Leukocytes ; Multivariate Analysis ; Neutrophils ; Recurrence ; Retrospective Studies ; Stem Cells ; Tissue Donors ; Transplants ; Umbilical Cord ; Unrelated Donors ; Whole-Body Irradiation

PURPOSE: To analyze clinical, radiological and pathological features as well as clinical outcome after surgical treatment of patients with secondary chondrosarcoma arising from osteochondroma(tosis). MATERIALS AND METHODS: We retrospectively reviewed clinical records, radiographs, pathologic slides of 14 patients. Nine patients were male and five were female. The mean age was 34 years. The mean follow-up period was 54 months. RESULTS: All patients had a history of previous mass since childhood or puberty. Preexisted osteochondroma was single in 3 patients and multiple in 10. Remaining 1 patient had multiple osteochondromatosis with enchondromatosis. MRI clearly provided thickness of cartilage cap, which was over 2 cm except in 2 cases. Chondrosarcoma was grade 1 in all except 1 case, which was grade 2. Wide excision was performed in 10 patients, marginal excision in 3 and amputation in 1. Twelve patients were doing very well without evidence of disease. Among 3 patients with marginal excision, 1 patient had local recurrence and 1 patient died of disease. CONCLUSION: Comprehensive understanding of clinical, radiological and pathological features of secondary chondrosarcoma is warranted for accurate diagnosis. The best result can be expected with early recognition of malignant change of osteohcondroma(tosis) and wide excision.
Amputation ; Cartilage ; Chondrosarcoma ; Enchondromatosis ; Female ; Follow-Up Studies ; Humans ; Male ; Osteochondroma ; Osteochondromatosis ; Puberty ; Recurrence ; Retrospective Studies

OBJECTIVES: Adoption of hospital information systems offers distinctive advantages in healthcare delivery. First, implementation of consolidated hospital information system in Seoul National University Hospital led to significant improvements in quality of healthcare and efficiency of hospital management. METHODS: The hospital information system in Seoul National University Hospital consists of component applications: clinical information systems, clinical research support systems, administrative information systems, management information systems, education support systems, and referral systems that operate to generate utmost performance when delivering healthcare services. RESULTS: Clinical information systems, which consist of such applications as electronic medical records, picture archiving and communication systems, primarily support clinical activities. Clinical research support system provides valuable resources supporting various aspects of clinical activities, ranging from management of clinical laboratory tests to establishing care-giving procedures. CONCLUSIONS: Seoul National University Hospital strives to move its hospital information system to a whole new level, which enables customized healthcare service and fulfills individual requirements. The current information strategy is being formulated as an initial step of development, promoting the establishment of next-generation hospital information system.
Adoption ; Confidentiality ; Delivery of Health Care ; Electronic Health Records ; Hospital Information Systems ; Information Systems ; Management Information Systems ; Quality of Health Care ; Radiology Information Systems ; Referral and Consultation

OBJECTIVE: To describe the radiographic findings of primary pulmonary tuberculosis (TB) in previously healthy adolescent patients. MATERIALS AND METHODS: The Institutional Review Board approved this retrospective study, with a waiver of informed consent from the patients. TB outbreaks occurred in 15 senior high schools and chest radiographs from 58 students with identical strains of TB were analyzed by restriction fragment length polymorphism analysis by two independent observers. Lesions of nodule(s), consolidation, or cavitation in the upper lung zones were classified as typical TB. Mediastinal lymph node enlargement; lesions of nodule(s), consolidation, or cavitation in lower lung zones; or pleural effusion were classified as atypical TB. Inter-observer agreement for the presence of each radiographic finding was examined by kappa statistics. RESULTS: Of 58 patients, three (5%) had normal chest radiographs. Cavitary lesions were present in 25 (45%) of 55 students. Lesions with upper lung zone predominance were observed in 27 (49%) patients, whereas lower lung zone predominance was noted in 18 (33%) patients. The remaining 10 (18%) patients had lesions in both upper and lower lung zones. Pleural effusion was not observed in any patient, nor was the mediastinal lymph node enlargement. Hilar lymph node enlargement was seen in only one (2%) patient. Overall, 37 (67%) students had the typical form of TB, whereas 18 (33%) had TB lesions of the atypical form. CONCLUSION: The most common radiographic findings in primary pulmonary TB by recent infection in previously healthy adolescents are upper lung lesions, which were thought to be radiographic findings of reactivation pulmonary TB by remote infection.
Adolescent ; *Disease Outbreaks ; Female ; Humans ; Male ; Mass Screening ; Polymorphism, Restriction Fragment Length ; Radiographic Image Interpretation, Computer-Assisted ; *Radiography, Thoracic ; Republic of Korea/epidemiology ; Retrospective Studies ; Schools ; Tuberculosis, Pulmonary/epidemiology/*radiography

BACKGROUND: To evaluate the usefulness and compliance of a hospital-based tabletop exercise in setting of pandemic influenza in hospitals. MATERIALS AND METHODS: Tabletop exercise was held in Pusan National University Hospital and forty two hospital employees were invited to participate in the exercise. The scenario for hospital-based tabletop exercise was designed. It consisted of three modules, which simulated the influx and outbreak of H5N1 influenza that was epidemic in Southeast Asia. Pre-, post-exercise surveys were completed by anonymous questions. RESULTS: Thirty-seven (88%) of 42 invited participants attended exercise. All members of the administration group and the ancillary services group participated. But, only 77% members of the clinical services group participated. In pre-exercise survey, priorities of eight goals regarding skills and knowledge during exercise were inquired., The highest priorities pointed out by the respondents were "Increase the knowledge of pandemic influenza" (22%), "Development of strategies for optimal communication among employees within specific department" (19%) and "Development of strategies for improved coordination between facilities within the health system" (19%). Twenty-one (57%) of participants completed the post-exercise surveys. At post-exercise surveys, 81% of the participants stated that the tabletop exercise was extremely or very useful, 86% of the participants also stated that it increased their knowledge of pandemic influenza. CONCLUSION: Tabletop exercise is an effective modality for increasing pandemic influenza preparedness in hospitals, and this method is useful for guiding preparedness activities within the hospital environment. Further studies to determine the appropriate method of discussion, questionnaire, duration of exercise and injection are needed.
Anonyms and Pseudonyms ; Asia, Southeastern ; Compliance ; Influenza, Human ; Pandemics ; Surveys and Questionnaires

BACKGROUND: To evaluate the usefulness and compliance of a hospital-based tabletop exercise in setting of pandemic influenza in hospitals. MATERIALS AND METHODS: Tabletop exercise was held in Pusan National University Hospital and forty two hospital employees were invited to participate in the exercise. The scenario for hospital-based tabletop exercise was designed. It consisted of three modules, which simulated the influx and outbreak of H5N1 influenza that was epidemic in Southeast Asia. Pre-, post-exercise surveys were completed by anonymous questions. RESULTS: Thirty-seven (88%) of 42 invited participants attended exercise. All members of the administration group and the ancillary services group participated. But, only 77% members of the clinical services group participated. In pre-exercise survey, priorities of eight goals regarding skills and knowledge during exercise were inquired., The highest priorities pointed out by the respondents were "Increase the knowledge of pandemic influenza" (22%), "Development of strategies for optimal communication among employees within specific department" (19%) and "Development of strategies for improved coordination between facilities within the health system" (19%). Twenty-one (57%) of participants completed the post-exercise surveys. At post-exercise surveys, 81% of the participants stated that the tabletop exercise was extremely or very useful, 86% of the participants also stated that it increased their knowledge of pandemic influenza. CONCLUSION: Tabletop exercise is an effective modality for increasing pandemic influenza preparedness in hospitals, and this method is useful for guiding preparedness activities within the hospital environment. Further studies to determine the appropriate method of discussion, questionnaire, duration of exercise and injection are needed.
Anonyms and Pseudonyms ; Asia, Southeastern ; Compliance ; Influenza, Human ; Pandemics ; Surveys and Questionnaires