Background: The association between renal dysfunction and cardiovascular outcomes has yet to be determined in patients with hypertrophic cardiomyopathy (HCM). We aimed to investigate whether mildly reduced renal function is associated with the prognosis in patients with HCM. Methods: Patients with HCM were enrolled at two tertiary HCM centers. Patients who were on dialysis, or had a previous history of heart failure (HF) or stroke were excluded. Patients were categorized into 3 groups by estimated glomerular filtration rate (eGFR): stage I (eGFR ≥ 90 mL/min/1.73 m2 , n = 538), stage II (eGFR 60–89 mL/min/1.73 m2 , n = 953), and stage III–V (eGFR < 60 mL/min/1.73 m2 , n = 265). Major adverse cardiovascular events (MACEs) were defined as a composite of cardiovascular death, hospitalization for HF (HHF), or stroke during median 4.0-year follow-up. Multivariable Cox regression model was used to adjust for covariates. Results: Among 1,756 HCM patients (mean 61.0 ± 13.4 years; 68.1% men), patients with stage III–V renal function had a significantly higher risk of MACEs (adjusted hazard ratio [aHR], 2.71; 95% confidence interval [CI], 1.39–5.27; P = 0.003), which was largely driven by increased incidence of cardiovascular death and HHF compared to those with stage I renal function. Even in patients with stage II renal function, the risk of MACE (vs. stage I: aHR, 2.21’ 95% CI, 1.23–3.96; P = 0.008) and HHF (vs. stage I: aHR, 2.62; 95% CI, 1.23–5.58; P = 0.012) was significantly increased. Conclusion This real-world observation showed that even mildly reduced renal function (i.e., eGFR 60–89 mL/min/1.73 m2 ) in patients with HCM was associated with an increased risk of MACEs, especially for HHF.

Purpose: Lymph node metastasis (LNM) is the most significant prognostic factor in cervical cancerthat was recently incorporated into the International Federation of Gynecology and Obstetrics(FIGO) staging system. This study was performed to evaluate whether the prognosticsignificance of LNM differs according to disease status. Materials and Methods: Patients with FIGO stage IB or higher cervical cancer who had pretreatment computedtomography and/or magnetic resonance imaging studies as well as long-term follow-upwere enrolled in this retrospective study. The hazard ratio (HR) of Cox regression was usedto determine the prognostic significance of LNM. The HRs were compared between the differenttumor groups (based on stage, histology, tumor size, primary treatment, age, parametriuminvolvement, and lymphovascular space invasion). Results: A total of 970 patients treated between January 1999 and December 2007 were included.The pretreatment LNM had prognostic significance in patients with stage IB1/IIA (HR forprogression-free survival 2.10, p=0.001; HR for overall survival 1.99, p=0.005). However,the significance gradually decreased or disappeared with advancing stages. Similarly, theprognostic significance of the pretreatment LNM decreased with advancing disease status,including old age, parametrial involvement or lymphovascular space involvement. In contrast,the tumor size was associated with the prognostic significance of LNM with advancingstatus. The significance of the clinical LNM did not reflect the significance of the clinicalstage. In contrast, the tumor size, parametrial involvement, and significance of the pathologicLNM reflected the clinical stage. Conclusion In patients with cervical cancer, pretreatment LNM on imaging has different clinical significancedepending on the tumor status.

OBJECTIVE: BRCA mutational status is important in the management of ovarian cancer, but there is a lack of evidence supporting genetic testing in Asian populations. This study was performed to investigate the prevalence and prognostic outcomes of BRCA1/2 mutation and variant of unknown significance (VUS) in Korean patients diagnosed with epithelial ovarian cancer (EOC). METHODS: Among patients newly diagnosed with EOC between January 2007 and January 2017, those tested for germline BRCA1/2 mutation were studied, regardless of family history. Overall survival (OS) and progression-free survival (PFS) were compared between the patients with and without BRCA1/2 mutation and VUS. RESULTS: A total of 313 patients underwent BRCA testing: 88 patients had a BRCA1/2 mutation and 48 patients had a BRCA1/2 VUS (28.1% and 15.3%, respectively). There were no significant associations between BRCA1/2 mutation, BRCA1/2 wild-type, or BRCA1/2 VUS with age at diagnosis, histologic distribution, or residual disease status after primary cytoreductive surgery. BRCA1 mutation, including BRCA1 VUS, showed no difference in PFS or OS compared to BRCA1 wild-type. In contrast, BRCA2 mutation showed longer PFS than that of BRCA2 wild-type (P=0.04) or BRCA2 VUS (P=0.02). BRCA2 mutation, including BRCA2 VUS, did not show any difference in OS compared to BRCA2 wild-type. CONCLUSION: BRCA mutation and BRCA VUS had similar clinical characteristics and survival outcomes, except that BRCA2 mutation showed better PFS. The results of this study will help to understand the prognostic significance of BRCA mutation and VUS in Korean patients.
Asian Continental Ancestry Group ; Diagnosis ; Disease-Free Survival ; Genes, BRCA1 ; Genes, BRCA2 ; Genetic Testing ; Humans ; Korea ; Ovarian Neoplasms ; Prevalence

PURPOSE: This study was conducted to evaluate the prognostic significance of pre-treatment complete blood cell count (CBC), including white blood cell (WBC) differential, in epithelial ovarian cancer (EOC) patients with primary debulking surgery (PDS) and to develop nomograms for platinum sensitivity, progression-free survival (PFS), and overall survival (OS). MATERIALS AND METHODS: We retrospectively reviewed the records of 757 patients with EOC whose primary treatment consisted of surgical debulking and chemotherapy at Samsung Medical Center from 2002 to 2012. We subsequently created nomograms for platinum sensitivity, 3-year PFS, and 5-year OS as prediction models for prognostic variables including age, stage, grade, cancer antigen 125 level, residual disease after PDS, and pre-treatment WBC differential counts. The models were then validated by 10-fold cross-validation (CV). RESULTS: In addition to stage and residual disease after PDS, which are known predictors, lymphocyte and monocyte count were found to be significant prognostic factors for platinum-sensitivity, platelet count for PFS, and neutrophil count for OS on multivariate analysis. The area under the curves of platinum sensitivity, 3-year PFS, and 5-year OS calculated by the 10-fold CV procedure were 0.7405, 0.8159, and 0.815, respectively. CONCLUSION: Prognostic factors including pre-treatment CBC were used to develop nomograms for platinum sensitivity, 3-year PFS, and 5-year OS of patients with EOC. These nomograms can be used to better estimate individual outcomes.
Blood Cell Count* ; Blood Cells* ; Disease-Free Survival* ; Drug Therapy ; Humans ; Leukocytes ; Lymphocytes ; Monocytes ; Multivariate Analysis ; Neutrophils ; Nomograms* ; Ovarian Neoplasms* ; Platelet Count ; Platinum* ; Prognosis ; Retrospective Studies

OBJECTIVE: The aim of this study was to compare survival outcomes in two groups of patients with recurrent epithelial ovarian cancer (EOC) with initial recurrence detection by cancer antigen 125 (CA-125) elevation or imaging, and underwent secondary cytoreductive surgery (SCS). METHODS: A retrospective review of the medical records was performed on 99 recurrent EOC patients who underwent SCS at the Samsung Medical Center between January 2002 and December 2013. For follow-up after primary treatment, patients were routinely assessed by CA-125 levels every 3 months and computed tomography (CT) scan (or magnetic resonance imaging [MRI]) every 6 months for first 3 years, and by CA-125 every 6 months and CT scan (or MRI) every 12 months thereafter. RESULTS: The first recurrence was initially identified by either CA-125 elevation (n=41, 41.4%) or by imaging study (n=58, 58.6%). None of the patients showed the symptoms as initial sign of recurrence. There were higher percentages of extra-pelvic recurrence (87.8%) and multiple recurrences (78.0%) in the group diagnosed by CA-125 elevation. The proportion of no residual disease after SCS was comparably lower in the CA-125 group (22.0% vs. 72.4%). There were 19 cancer-associated deaths (19.2%) within a median follow-up period of 67 months. The group diagnosed by imaging had better overall survival from initial diagnosis (OS1), overall survival after SCS (OS2), progression-free survival after the initial treatment (PFS1) and progression-free survival after SCS compared to those of the CA-125 group (PFS2). CONCLUSION: EOC patients with recurrence initially detected by imaging study showed better survival outcomes than patients diagnosed by CA-125 elevation.
Adult ; Aged ; CA-125 Antigen/*blood ; Female ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Neoplasm Recurrence, Local/blood/diagnostic imaging/*mortality ; Neoplasms, Glandular and Epithelial/blood/diagnostic imaging/*mortality ; Ovarian Neoplasms/blood/diagnostic imaging/*mortality ; Retrospective Studies ; Tomography, X-Ray Computed

OBJECTIVE: To assess the feasibility of laparoscopic cytoreduction in patients with localized recurrent epithelial ovarian cancer (EOC) by comparing its outcomes to those of laparotomy. METHODS: We performed retrospective analysis in 79 EOC patients who had a localized single recurrent site, as demonstrated by computed tomography (CT) scan, magnetic resonance imaging, or positron emission tomography/CT scan; had no ascites; were disease-free for 12 or more months prior; and who had undergone secondary cytoreduction (laparoscopy in 31 patients, laparotomy in 48 patients) at Samsung Medical Center between 2002 and 2013. By reviewing the electronic medical records, we investigated the patients' baseline characteristics, surgical characteristics, and surgical outcomes. RESULTS: There were no statistically significant differences between laparoscopy and laparotomy patients in terms of age, body mass index, cancer antigen 125 level, tumor type, initial stage, grade, recurrence site, type of procedures used in the secondary cytoreduction, adjuvant chemotherapy, and disease-free interval from the previous treatment. With regards to surgical outcomes, reduced operating time, shorter hospital stay, and less estimated blood loss were achieved in the laparoscopy group. Complete debulking was achieved in all cases in the laparoscopy group. CONCLUSION: The laparoscopic approach is feasible without compromising morbidity and survival in selected groups of patients with recurrent EOC. The laparoscopic approach can be a possible treatment option for recurrent EOC.
Adolescent ; Adult ; Aged ; Cytoreduction Surgical Procedures/*methods ; Feasibility Studies ; Female ; Humans ; Laparoscopy/methods ; Magnetic Resonance Imaging ; Middle Aged ; Neoplasm Recurrence, Local/diagnostic imaging/*surgery ; Neoplasms, Glandular and Epithelial/diagnostic imaging/*surgery ; Ovarian Neoplasms/diagnostic imaging/*surgery ; Ovary/diagnostic imaging/surgery ; Positron-Emission Tomography ; Retrospective Studies ; Tomography, X-Ray Computed ; Young Adult

OBJECTIVE: The aim of this study was to investigate outcomes in uterine cancer patients undergoing pulmonary metastasectomy and prognostic factors associated with survival after the procedure. METHODS: A retrospective study was performed in 29 uterine cancer patients who underwent surgical resection of pulmonary metastatic lesions at Samsung Medical Center between June 1995 and December 2011. RESULTS: Histopathology showed carcinoma in 17 patients (58.6%) and sarcoma in 12 patients (41.4%). Of the 29 patients, 17 (58.6%) had less than three pulmonary metastatic lesions. Eight (27.6%) had symptoms related to lung metastasis. The 5-year survival rate after pulmonary metastasectomy for the entire cohort was 48.2%. On univariate and multivariate analysis, the presence of pulmonary symptoms and more than three lesions of metastasis were associated with poor survival after pulmonary metastasectomy. CONCLUSION: Pulmonary metastasectomy for uterine cancer is an acceptable treatment in selected patients. Patients with more than three pulmonary metastatic lesions and pulmonary symptoms related to lung metastasis could expect to have worse prognosis after pulmonary metastasectomy.
Adenocarcinoma/secondary/surgery ; Adult ; Aged ; Combined Modality Therapy ; Female ; Humans ; Hysterectomy/methods ; Leiomyosarcoma/*secondary/*surgery ; Lung Neoplasms/*secondary/*surgery ; Metastasectomy/*methods ; Middle Aged ; Prognosis ; Retrospective Studies ; Treatment Outcome ; *Uterine Neoplasms

OBJECTIVE: To analyze the prognostic role of revised version of International Federation of Gynecology and Obstetrics (FIGO) stage (2013) in epithelial ovarian cancer and compare with previous version staging classification METHODS: We retrospectively enrolled patients with epithelial ovarian cancer treated at Samsung Medical Center from 2002 to 2012. We reclassified the patients based on the revised FIGO staging classification. RESULTS: Eight hundred seventy-eight patients were enrolled (stage I, 22.8%; stage II, 10.4%; stage III, 56.2%; stage IV, 10.7%). Previous stage IC (98, 11.1%) was subdivided into IC1 (9, 1.0%), IC2 (57, 6.4%), and IC3 (32, 4.1%). In addition, previous stage IV (94, 1.7%) was categorized into IVA (37, 4.2%) and IVB (57, 6.5%) in new staging classification. Stage IIC (66, 7.5%) has been eliminated and integrated into IIA (36, 4.1%) and IIB (55, 6.2%) in revised classification. Revised FIGO stage IC3 had significant prognostic impact on PFS (hazard ratio [HR], 3.840; 95% confidence interval [CI], 1.361 to 10.83; P=0.011) and revised FIGO stage IIIC appears to be an independent, significant poor prognostic factor for PFS (HR, 2.541; 95% CI, 1.242 to 5.200; P=0.011) but not in the case of previous version of FIGO stage IIIC (HR, 1.070; 95% CI, 0.502 to 2.281; P=0.860). However, any sub-stages of both previous and revised version in stage II and IV, there was no significant prognostic role. CONCLUSION: Revised FIGO stage has more progressed utility for informing prognosis than previous version, especially in stage I and III. For stage II and IV, further validation should be needed in large population based study in the future.
Classification* ; Gynecology ; Humans ; Neoplasm Staging ; Obstetrics ; Ovarian Neoplasms* ; Prognosis ; Retrospective Studies ; Survival Analysis*

Invasive pulmonary aspergillosis (IPA) is the most frequent form of invasive fungal diseases in immunocompromised patients. However, there are only a few studies on IPA in immunocompromised children in Korea. This study was designed to characterize IPA in Korean children with hematologic/oncologic diseases. Medical records of children with hematologic/oncologic diseases receiving antifungal therapy were reviewed. The enrolled children were divided into the IPA group (proven and probable IPA) and non-IPA group, and the clinical characteristics and prognosis were compared between the two groups. During the study period, 265 courses of antifungal therapy were administered to 166 children. Among them, two (0.8%) episodes of proven IPA, 35 (13.2%) of probable IPA, and 52 (19.6%) of possible IPA were diagnosed. More children in the IPA group suffered from neutropenia lasting for more than two weeks (51.4% vs. 21.9%, P<0.001) and showed halo signs on the chest computed tomography (78.4% vs. 40.7%, P<0.001) than in the non-IPA group. No other clinical factors showed significant differences between the two groups. Amphotericin B deoxycholate was administered as a first line antifungal agent in 33 (89.2%) IPA group episodes, and eventually voriconazole was administered in 27 (73.0%) episodes. Ten (27.0%) children in the IPA group died within 12 weeks of antifungal therapy. In conclusion, early use of chest computed tomography to identify halo signs in immunocompromised children who are expected to have prolonged neutropenia can be helpful for early diagnosis of IPA and improving prognosis of children with IPA.
Antifungal Agents/*therapeutic use ; Child ; Child Health/statistics & numerical data ; Comorbidity ; Female ; Hematologic Diseases/*mortality ; Humans ; Incidence ; Invasive Pulmonary Aspergillosis/*diagnosis/drug therapy/*mortality ; Male ; Neoplasms/*mortality ; Prognosis ; Republic of Korea/epidemiology ; Risk Factors ; Survival Rate ; Tomography, X-Ray Computed/statistics & numerical data ; Treatment Outcome

Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS), a very rare disease that is caused by the presence of antifactor II antibodies, is usually counterbalanced by the prothrombotic effect of lupus anticoagulant (LAC). Patients with LAHPS are treated using fresh frozen plasma, steroids, immunosuppressive agents, and immunoglobulins for managing the disease and controlling hemorrhages. Notably, steroids are the important treatment for treating hypoprothrombinemia and controlling the bleeding. However, some patients suffer from severe, life-threatening hemorrhages, when factor II levels remain very low in spite of treatment with steroids. Here, we report a case of LAHPS in a 15-year-old girl who experienced pulmonary hemorrhage with rapid progression. She was referred to our hospital owing to easy bruising and prolonged bleeding. She was diagnosed with LAHPS that presented with pancytopenia, positive antinuclear antibody, proloned prothrombin time, activated partial thromboplastin time, positive LAC antibody, and factor II deficiency. Her treatment included massive blood transfusion, high-dose methylprednisolone, vitamin K, and immunoglobulin. However, she died due to uncontrolled pulmonary hemorrhage.
Adolescent ; Antibodies ; Antibodies, Antinuclear ; Blood Transfusion ; Female ; Hemorrhage* ; Humans ; Hypoprothrombinemias* ; Immunoglobulins ; Immunosuppressive Agents ; Lupus Coagulation Inhibitor* ; Methylprednisolone ; Pancytopenia ; Partial Thromboplastin Time ; Plasma ; Prothrombin ; Prothrombin Time ; Rare Diseases ; Steroids ; Vitamin K