Purpose: Outcome analysis of urachal cancer (UraC) is limited due to the scarcity of cases and different staging methods compared to urothelial bladder cancer (UroBC). We attempted to assess survival outcomes of UraC and compare to UroBC after stage-matched analyses. Materials and Methods: Total 203 UraC patients from a multicenter database and 373 UroBC patients in single institution from 2000 to 2018 were enrolled (median follow-up, 32 months). Sheldon stage conversion to corresponding TNM staging for UraC was conducted for head-to-head comparison to UroBC. Perioperative clinical variables and pathological results were recorded. Stage-matched analyses for survival by stage were conducted. Results: UraC patients were younger (mean age, 54 vs. 67 years; p < 0.001), with 163 patients (80.3%) receiving partial cystectomy and 23 patients (11.3%) radical cystectomy. UraC was more likely to harbor ≥ pT3a tumors (78.8% vs. 41.8%). While 5-year recurrence-free survival, cancer-specific survival (CSS) and overall survival were comparable between two groups (63.4%, 67%, and 62.1% in UraC and 61.5%, 75.9%, and 67.8% in UroBC, respectively), generally favorable prognosis for UraC in lower stages (pT1-2) but unfavorable outcomes in higher stages (pT4) compared to UroBC was observed, although only 5-year CSS in ≥ pT4 showed statistical significance (p=0.028). Body mass index (hazard ratio [HR], 0.929), diabetes mellitus (HR, 1.921), pathologic T category (HR, 3.846), and lymphovascular invasion (HR, 1.993) were predictors of CSS for all patients. Conclusion Despite differing histology, UraC has comparable prognosis to UroBC with relatively favorable outcome in low stages but worse prognosis in higher stages. The presented system may be useful for future grading and risk stratification of UraC.

Purpose: We aimed to investigate the risk factors and patterns of locoregional recurrence (LRR) after radical nephrectomy (RN) in patients with locally advanced renal cell carcinoma (RCC). Materials and Methods: We retrospectively analyzed 245 patients who underwent RN for non-metastatic pT3-4 RCC from January 2006 to January 2016. We analyzed the risk factors associated with poor locoregional control using Cox regression. Anatomical mapping was performed on reference computed tomography scans showing intact kidneys. Results: The median follow-up duration was 56 months (range, 1 to 128 months). Tumor extension to renal vessels or the inferior vena cava (IVC) and Fuhrman’s nuclear grade IV were identified as independent risk factors of LRR. The 5-year actuarial LRR rates in groups with no risk factor, one risk factor, and two risk factors were 2.3%, 19.8%, and 30.8%, respectively (p < 0.001). The locations of LRR were distributed as follows: aortocaval area (n=2), paraaortic area (n=4), retrocaval area (n=5), and tumor bed (n=11). No LRR was observed above the celiac axis (CA) or under the inferior mesenteric artery (IMA). Conclusion Tumor extension to renal vessels or the IVC and Fuhrman’s nuclear grade IV were the independent risk factors associated with LRR after RN for pT3-4 RCC. The locations of LRR after RN for RCC were distributed in the tumor bed and regional lymphatic area from the bifurcation of the CA to that of the IMA.

PURPOSE: To compare biopsy performance of 2 approaches for multiparametric magnetic resonance imaging (MRI) guided biopsy and transrectal ultrasonography (TRUS)-guided biopsy with 2nd and 3rd repeat biopsy patients in prostate cancer detection. MATERIALS AND METHODS: This retrospective study reviewed 2,868 patients who was performed prostate biopsy between September 2013 to March 2017 at Samsung Medical Center, Seoul, Korea with TRUS-guided random biopsy and MRI fusion, MRI cognitive, and MRI-guided biopsy as 2nd and 3rd repeat biopsy and propensity matching was applied to reduce bias. Detection rate of each study was compared with 1:1 matching. RESULTS: Among 265 patients who performed TRUS 2nd biopsy, positivity rate for prostate cancer (PCa) was 18.49% (n=49/265) while 54.72% (n=145/265) for MRI-guided biopsy. In 3rd biopsy, positivity rate for PCa of TRUS biopsy was 17.74% (n=11/62) while 56.45% (n=35/62) for MRI guided biopsy. There was no significant difference in the detection rate for the patient with Gleason score 8 or more. CONCLUSIONS: MRI-guided biopsy was associated with a higher detection rate of prostate cancer with especially in patients with prior negative biopsy.
Bias (Epidemiology) ; Biopsy ; Cohort Studies ; Humans ; Korea ; Magnetic Resonance Imaging ; Neoplasm Grading ; Passive Cutaneous Anaphylaxis ; Propensity Score ; Prostate ; Prostatic Neoplasms ; Retrospective Studies ; Seoul ; Ultrasonography

PURPOSE: We compared biopsy results and surgical outcomes of magnetic resonance imaging (MRI)-guided biopsy with transrectal ultrasonography (TRUS)-guided biopsy to demonstrate efficacy of MRI-guided biopsy on previous biopsy negative patients. MATERIALS AND METHODS: We retrospectively reviewed data of 120 patients who were categorized into MRI-guided biopsy groups (n=20) and TRUS-guided biopsy groups (n=100). All patients were diagnosed with prostate cancer (PCa) and had undergone radical prostatectomy (RP) after MRI-guided or TRUS-guided repeat biopsy between January 2010 and March 2016. Detection rate of significant cancer and Gleason score upgrading and downgrading were examined, in addition to biopsy results and subsequent RP outcomes. RESULTS: Median values for prostate-specific antigen level of the TRUS-guided biopsy group and the MRI-guided biopsy group were 6.67 and 5.86 ng/mL (p=0.303), respectively. Median prostate volume of each group (34.1 mL vs. 23.5 mL, p=0.007), number of positive cores (2.0 vs. 3.0, p=0.001) and maximum cancer/core rate (30.0% vs. 60.0%, p<0.001) were statistically different. Positive core rates of each group were 21.9% and 87.1%, respectively. Pathologic T stage was the only variable that showed difference in surgical outcomes (p=0.002). Most of PCa was confirmed as clinically significant PCa after RP in MRI-guided biopsy group (95%). CONCLUSIONS: MRI-guided biopsy showed higher positive core rate and detection rate of clinically significant PCa than TRUS-guided biopsy in repeat biopsy setting. Prospective multicenter large-scale study and accumulation of data is expected to further define superiority of the MRI-guided biopsy.
Biopsy* ; Humans ; Magnetic Resonance Imaging ; Neoplasm Grading ; Passive Cutaneous Anaphylaxis ; Prospective Studies ; Prostate ; Prostate-Specific Antigen ; Prostatectomy ; Prostatic Neoplasms ; Retrospective Studies ; Ultrasonography

PURPOSE: To determine the negative predictive value (NPV) of multiparametric magnetic resonance imaging (mp-MRI) for clinically significant cancer (CSC) based on the Prostate Imaging-Reporting and Data System (PI-RADS) version 2 in very low-risk or low-risk prostate cancer patients. MATERIALS AND METHODS: We retrospectively analyzed 380 patients with low risk of prostate cancer who underwent mp-MRI before radical prostatectomy (RP) from 2011 to 2013. Of the 380 patients, 142 patients were in the very low risk group. CSC at RP was defined as follows: any T3−4, G3+4 with tumor volume>15%, G4+3 or higher. In the very low risk and low risk groups, we analyzed the rate of CSC according to PI-RADS score and calculated the NPV of mp-MRI for detection of CSC. RESULTS: In the low risk group, 20.8% (n=79) of patients had PI-RADS version 2 score 1–2 and 17.4% (n=66) of patients had PI-RADS version 2 score 3. In the very low risk group, 26.8% (n=38) of patients had PI-RADS version 2 score 1–2 and 17.6% (n=25) of patients had PI-RADS version 2 score 3 in the very low risk group. Rates of CSC were 33.7% (n=128) and 16.9% (n=24) in the low risk and very low risk groups, respectively. The NPV of MRI was 93.7% in the very low risk group and 78.6% in the low risk group. CONCLUSIONS: The NPV of PI-RADS for CSC is high in the very low risk group, but not in the low risk group. Further multicenter studies are needed to investigate the utility of PI-RADS version 2 for NPV.
Humans ; Information Systems* ; Magnetic Resonance Imaging* ; Prostate* ; Prostatectomy* ; Prostatic Neoplasms* ; Retrospective Studies

This study represents the first epidemiological study based on the national registry of primary immunodeficiencies (PID) in Korea. Patient data were collected from 23 major hospitals. A total of 152 patients with PID (under 19 yr of age), who were observed from 2001 to 2005, have been entered in this registry. The period prevalence of PID in Korea in 2005 is 11.25 per million children. The following frequencies were found: antibody deficiencies, 53.3% (n = 81), phagocytic disorders, 28.9% (n = 44); combined immunodeficiencies, 13.2% (n = 20); and T cell deficiencies, 4.6% (n = 7). Congenital agammaglobulinemia (n = 21) and selective IgA deficiency (n = 21) were the most frequently reported antibody deficiency. Other reported deficiencies were common variable immunodeficiencies (n = 16), X-linked agammaglobulinemia (n = 15), IgG subclass deficiency (n = 4). Phagocytic disorder was mostly chronic granulomatous disease. A small number of patients with Wiskott-Aldrich syndrome, hyper-IgE syndrome, and severe combined immunodeficiency were also registered. Overall, the most common first manifestation was pneumonia. This study provides data that permit a more accurate estimation PID patients in Korea.
Adolescent ; Agammaglobulinemia/congenital/epidemiology ; Age Distribution ; Child ; Child, Preschool ; Common Variable Immunodeficiency/epidemiology ; Female ; Genetic Diseases, X-Linked/epidemiology ; Humans ; IgA Deficiency/epidemiology ; IgG Deficiency/epidemiology ; Immunologic Deficiency Syndromes/*epidemiology ; Infant ; Infant, Newborn ; Job's Syndrome/epidemiology ; Male ; Prevalence ; Questionnaires ; Registries ; Republic of Korea/epidemiology ; Severe Combined Immunodeficiency/epidemiology ; Sex Distribution ; Wiskott-Aldrich Syndrome/epidemiology ; Young Adult

Human tails or caudal cutaneous appendages are rare congenital anomalies with lesions usually arising from the midline of the lumbosacrococcygeal area. There have been only around 70 cases reported in literature. A 4-month-old male infant born as the 1st child of a dizygotic twin was presented with a penis-shaped mass of 4-cm in the coccygeal area. The mass was entirely covered by skin without any skin defect and was connected to the midline skin of the posterior coccygeal area by a stalk. Magnetic resonance images showed fat density of the mass without any demonstrable communication with the spinal canal nor any underlying central nervous system anomalies. The patient underwent a simple surgical excision of the mass without residual effects and complications. Histopathologic examination of the mass revealed lobules of mature adipose tissue, fibromuscular soft tissue, small vessels and nerves with normal hair follicles on the skin.
Adipose Tissue ; Central Nervous System ; Child ; Hair Follicle ; Humans ; Infant ; Magnetic Resonance Spectroscopy ; Male ; Skin ; Spinal Canal ; Twins, Dizygotic

PURPOSE: Despite the increasing prevalence of allergic rhinitis, there are difficulties in early diagnosis and proper treatment due to lack of education and misunderstanding of the disease. Development of efficient education materials based on the level of awareness of allergic rhinitis is required. Therefore, this study was aimed to develop a questionnaire for investigating the level of awareness of allergic rhinitis. METHODS: Questions related to the level of awareness of allergic rhinitis were prepared on the basis of the literature, professional advice and inquiries from patients and caregivers. These items were categorized into 4 areas: (1) basic knowledge, (2) symptoms, (3) diagnosis and environmental management and (4) treatment. Through the eight preliminary survey on 38 adults, a questionnaire of 60 items was developed. The results were analyzed by using SPSS 12.0. RESULTS: A tool for investigating the level of awareness was completed, which was composed of 20 items with an internal reliability of 0.67. According to the results of the survey with 60 items, the areas of basic knowledge, symptoms and environmental management of allergic rhinitis showed high awareness. The items regarding the association between allergic rhinitis and asthma, necessity of regular checkup, awareness of maintenance treatment, importance of treatment with intranasal steroids, and the influence of treatment on immunity, growth and development of patient showed low awareness. CONCLUSION: A reliable tool was developed for investigating the level of awareness of allergic rhinitis. It may be useful in developing educational materials to optimize its educational effect on allergic rhinitis.
Adult ; Asthma ; Caregivers ; Early Diagnosis ; Growth and Development ; Humans ; Prevalence ; Surveys and Questionnaires ; Rhinitis ; Rhinitis, Allergic, Perennial ; Steroids

Intracranial dermoid cyst is a rare congenital disease originating in the embryo. Intracranial dermoid cysts are usually detected before five years of age; however, asymptomatic cysts have been discovered after the third decade of life through various complications. Sometimes, there are neurologic symptoms caused by a cystic mass effect and if there is a dermoid sinus, purulent infections can occur. Early diagnosis is important and as such, CT or MR imaging for recognition of the location, size, and characteristics of the cysts are recommended before surgical resection. Extradural dermoid cysts with an intact occipital dermoid sinus are a rare event. We report a case of intracranial dermoid cyst associated with scalp abscess. In this case, a three-year-old boy was presented with scalp abscess and we performed MR imaging, which revealed a cystic region. The prognosis after resection operation was good without any complication.
Abscess ; Dermoid Cyst ; Early Diagnosis ; Embryonic Structures ; Neurologic Manifestations ; Prognosis ; Scalp ; Spina Bifida Occulta

Miller-Fisher syndrome is an acute poly-neuropathy typically characterized by the triad of ophthalmoplegia, ataxia, and areflexia. Other poly-neuropathy diseases include Guillain-Barre syndrome and Bickerstaff brainstem encephalitis. However, not all cases of Miller-Fisher syndrome have the typical three symptoms. These diseases have been differentiated by clinical manifestations and anti-GQ1b IgG antibody testing. In Miller-Fisher syndrome, anti-GQ1b IgG antibody is present in over 95% of patients; however, the frequency is not so high in normal subjects or in those with other poly-neuropathy diseases. Infectious illnesses, such as respiratory diseases and gastrointestinal diseases, usually precede Miller-Fisher syndrome. In general, Miller-Fisher syndrome is a self-limiting illness; however, a few cases of Miller-Fisher syndrome progressing to respiratory failure have been reported, particularly in children. In this case, we report abnormal angiographic findings. We report a case of atypical Miller-Fisher syndrome with internal carotid artery stenosis treated with prednisolone.
Ataxia ; Brain Stem ; Carotid Artery, Internal ; Carotid Stenosis ; Child ; Encephalitis ; Gastrointestinal Diseases ; Guillain-Barre Syndrome ; Humans ; Immunoglobulin G ; Miller Fisher Syndrome ; Ophthalmoplegia ; Polyneuropathies ; Prednisolone ; Respiratory Insufficiency