Sarcomatoid carcinoma is rarely diagnosed as gallbladder cancer. Its aggressive nature, due to the characteristics of both sarcoma and carcinoma, results in a poor prognosis. We report a case of gallbladder sarcomatoid carcinoma in an 82-year-old male who was referred to our hospital for evaluation of gallbladder cancer observed on abdominopelvic computed tomography. The characteristics of the cancer were not confirmed after several imaging modalities. The surgically resected tumor was positive for both cytokeratin and vimentin as revealed via immunohistochemical staining, and a sarcomatoid carcinoma was finally diagnosed. The role of chemotherapy has not yet been identified. Therefore, radiation therapy is planned to reduce the risk of recurrence.

Background/Aims: We evaluated the feasibility and long-term efficacy of the combination of cytarabine, idarubicin, and all-trans retinoic acid (ATRA) for treating patients with newly diagnosed acute promyelocytic leukemia (APL). Methods: We included 87 patients with newly diagnosed acute myeloid leukemia and a t(15;17) or promyelocytic leukemia/retinoic acid receptor alpha (PML-RARα) mutation. Patients received 12 mg/m2/day idarubicin intravenously for 3 days and 100 mg/m2/day cytarabine for 7 days, plus 45 mg/m2/day ATRA. Clinical outcomes included complete remission (CR), relapse-free survival (RFS), overall survival (OS), and the secondary malignancy incidence during a 20-year follow-up. Results: The CR, 10-year RFS, and 10-year OS rates were 89.7%, 94.1%, and 73.8%, respectively, for all patients. The 10-year OS rate was 100% for patients that achieved CR. Subjects were classified according to the white blood cell (WBC) count in peripheral blood at diagnosis (low-risk, WBC < 10,000/mm3; high-risk, WBC ≥ 10,000/mm3). The low-risk group had significantly higher RFS and OS rates than the high-risk group, but the outcomes were not superior to the current standard treatment (arsenic trioxide plus ATRA). Toxicities were similar to those observed with anthracycline plus ATRA, and higher than those observed with arsenic trioxide plus ATRA. The secondary malignancy incidence after APL treatment was 2.7%, among the 75 patients that achieved CR, and 5.0% among the 40 patients that survived more than 5 years after the APL diagnosis. Conclusions Adding cytarabine to anthracycline plus ATRA was not inferior to anthracycline plus ATRA alone, but it was not comparable to arsenic trioxide plus ATRA. The probability of secondary malignancy was low.

Objective: Clinical outcomes of patients who undergo transarterial chemoembolization (TACE) for single small hepatocellular carcinoma (HCC) are not consistent, and may differ based on certain imaging findings. This retrospective study was aimed at determining the efficacy of pre-TACE CT or MR imaging findings in predicting survival outcomes in patients with small HCC upon being treated with TACE. Besides, the study proposed to build a risk prediction model for these patients. Materials and Methods: Altogether, 750 patients with functionally good hepatic reserve who received TACE as the first-line treatment for single small HCC between 2004 and 2014 were included in the study. These patients were randomly assigned into training (n = 525) and validation (n = 225) sets. Results: According to the results of a multivariable Cox analysis, three pre-TACE imaging findings (tumor margin, tumor location, enhancement pattern) and two clinical factors (age, serum albumin level) were selected and scored to create predictive models for overall, local tumor progression (LTP)-free, and progression-free survival in the training set. The median overall survival time in the validation set were 137.5 months, 76.1 months, and 44.0 months for low-, intermediate-, and high-risk groups, respectively (p < 0.001). Time-dependent receiver operating characteristic curves of the predictive models for overall, LTP-free, and progression-free survival applied to the validation cohort showed acceptable areas under the curve values (0.734, 0.802, and 0.775 for overall survival; 0.738, 0.789, and 0.791 for LTP-free survival; and 0.671, 0.733, and 0.694 for progression-free survival at 3, 5, and 10 years, respectively). Conclusion Pre-TACE CT or MR imaging findings could predict survival outcomes in patients with small HCC upon treatment with TACE. Our predictive models including three imaging predictors could be helpful in prognostication, identification, and selection of suitable candidates for TACE in patients with single small HCC.

Botryoid Wilms tumor, a very rare variant of Wilms tumor, arises from the pelvocalyceal system, and its occurrence in the fetal or neonatal period has never been reported in the literature. Herein, we report an exceedingly rare and challenging case of botryoid Wilms tumor in a neonate who initially presented with fetal hydronephrosis. Postnatal ultrasonography revealed multiple lobulating hypoechoic masses with varying degrees of intralesional vascularity within the dilated pelvocalyceal system. To our knowedge, this is a case report of botryoid Wilms tumor of the youngest child in English literature.

Background/Aims: Stereotactic body radiation therapy (SBRT) is used as an alternative ablative treatment in patients with hepatocellular carcinoma (HCC) not suitable for curative treatments. The purpose of this prospective study was to evaluate the long-term efficacy of SBRT for small (≤5 cm) HCCs. Methods: A phase II, single-arm clinical trial on SBRT for small HCCs was conducted at an academic tertiary care center. The planned SBRT dose was 45 Gy with a fraction size of 15-Gy over 3 consecutive days. The primary endpoint was 2-year local control rate. Radiologic responses were assessed according to the Response Evaluation Criteria in Solid Tumors (RECIST, version 1.1) and the modified RECIST criteria. Results: Between 2013 and 2016, 50 patients (53 lesions) were enrolled, with a median follow-up period of 47.8 months (range, 2.9–70.6). Patients’ age ranged from 41 to 74 years, and 80% were male. Median tumor size was 1.3 cm (range, 0.7–3.1). The 2- and 5-year local control rates were 100% and 97.1%, respectively. The 5-year overall survival rate was 77.6%. Six months after SBRT, radiologic responses were evident in 44 lesions (83%) according to the RECIST criteria and 49 (92.4%) according to the modified RECIST criteria. None of the patients showed grade ≥3 adverse events. Conclusions SBRT showed excellent results as an ablative treatment for patients with small HCCs while showing minimal toxicities. SBRT can be a good alternative for both curative and salvage intents in patients with HCCs that are unsuitable for curative treatments.

PURPOSE: This study was to develop evidence-based clinical practice guideline in order to prevent contrast-induced nephropathy (CIN) for patients undergoing percutaneous coronary intervention (PCI). METHODS: The guideline was developed based on the “Scottish Intercollegiate Guidelines Network (SIGN)”. The first draft of guideline was developed through 5 stages and evaluated by 10 experts.(1) Clinical questions were ensured in PICO format.(2) Two researchers conducted a systematic search through electronic database, identifying 170 studies. We selected 27 full text articles including 16 randomized clinical trials, 7 systematic reviews, and 4 guidelines. Quality of each studies were evaluated by the Cochran's Risk of Bias, AMSTAR, K-AGREEII. Among the studies, 11 studies were excluded.(3) The strength of recommendations were classified and quality of recommendations were ranked.(4) Guideline draft was finalized.(5) Content-validation was conducted by an expert group. All contents were ranked above 0.8 in CVI. RESULTS: Evidence-based clinical practice guideline to prevent CIN was dveloped.(1) The guideline for preventing CIN recommends using 0.9% saline.(2) Standardized rate of fluid therapy is 1 to 1.5ml/kg/hr.(3) Execute hydration for 6~12hrs before PCI and after PCI. CONCLUSION: This study suggests evidence-based clinical practice guideline for preventing CIN which can be more efficiently used in clinical practice.
Acute Kidney Injury ; Bias (Epidemiology) ; Contrast Media ; Evidence-Based Practice ; Fluid Therapy ; Humans ; Percutaneous Coronary Intervention

OBJECTIVE: The present study investigated the clinical characteristics of Alzheimer's disease (AD) dementia with low brain amyloid-beta (Aβ-AD) burden comparing with AD dementia with high amyloid-beta burden (Aβ+AD). We also developed a prediction model for the amyloid positivity on ¹¹C-labelled Pittsburgh Compound B (PiB) positron emission tomography (PET) with distinct clinical variables in AD dementia patients. METHODS: Fifty-nine clinically defined AD dementia individuals, who participated in the Korean Brain Aging Study for Early diagnosis and prediction of AD (KBASE) study, were included. All the subjects received comprehensive clinical evaluations and PiB-PET. Based on cerebral PiB retention, all subjects were divided into Aβ+AD (n=47) and Aβ-AD (n=12) subgroups. To develop a prediction model for amyloid positivity, stepwise multiple logistic regression analysis was conducted. RESULTS: When compared to Aβ+AD, Aβ-AD showed older age, later age-at-onset, and lower education. In regard of risk factors for dementia, Aβ-AD had higher frequency of hypertension and diabetes mellitus as well as lower frequency of apolipoprotein E (APOE) ε4 allele. Although there was no between group difference in Clinical Dementia Rating (CDR) or CDR sum-of-boxes scores, mini-mental state examination and constructional recall scores were higher for Aβ-AD than Aβ+AD. The final amyloid positivity prediction model included APOE4 genotype, hypertension, and diabetes mellitus. CONCLUSION: The findings from this study indicated that clinically diagnosed AD dementia may have high possibility of not being pathological AD if they have older age and higher vascular risks, and did not have APOE4 genotype.
Age of Onset ; Aging ; Alleles ; Alzheimer Disease* ; Amyloid* ; Apolipoprotein E4 ; Apolipoproteins ; Brain ; Dementia* ; Diabetes Mellitus ; Early Diagnosis ; Education ; Genotype ; Humans ; Hypertension ; Logistic Models ; Positron-Emission Tomography ; Risk Factors

PURPOSE: Adherence to immunosuppressants is the key to prevent organ rejection in organ transplant recipients. The purpose of this study was to investigate current interventions to improve adherence to immunosuppressants in liver transplant recipients. METHODS: A systemic literature search was done using PubMed, Embase, Cochrane Library, CINAHL and four Korean databases to identify experimental studies reported in English or Korean up to and including 2015. We identified eight intervention studies on the adherence to immunosuppressants in liver transplant recipients independently reviewed by two reviewers. The quality and risk of bias of the selected studies were assessed. RESULTS: Education, conversion of regimen, and text messaging were identified as intervention techniques to improve adherence. We found positive results in three out of four studies implementing educational strategies, but the results were not sufficient to draw a definite conclusion. Conversion from a twice-daily tacrolimus-based regimen to a once-daily tacrolimus extended-release formula was used in three adult-only studies and its effectiveness was confirmed. One study showed that improved adherence and outcomes were effected by using text messaging with pediatric patients. CONCLUSION: Future research is needed to facilitate interventions to improve adherence to immunosuppressants in various ages of patients including pediatric/adolescent liver transplant recipients.
Bias (Epidemiology) ; Education ; Humans ; Immunosuppressive Agents* ; Clinical Trial ; Liver* ; Tacrolimus ; Text Messaging ; Transplantation* ; Transplants

Riedel's thyroiditis (RT) is a rare chronic inflammatory disease of the thyroid gland. It is characterized by a fibroinflammatory process that partially destroys the gland and extends into adjacent neck structures. Its clinical manifestation can mask an accompanying thyroid neoplasm and can mimic invasive thyroid carcinoma. Therefore, diagnosis can be difficult prior to surgical removal of the thyroid, and histopathologic examination of the thyroid is necessary for a definite diagnosis. The concurrent presence of RT and other thyroid diseases has been reported. However, to our knowledge, the association of RT with acute suppurative thyroiditis and micropapillary carcinoma has not been reported. We report a rare case of concurrent RT, acute suppurative thyroiditis, and micropapillary carcinoma in a 48-year-old patient.
Anti-Bacterial Agents/therapeutic use ; Biopsy ; Carcinoma/*complications/diagnosis/therapy ; Female ; Hashimoto Disease/*complications/diagnosis/therapy ; Hormone Replacement Therapy ; Humans ; Lymph Node Excision ; Middle Aged ; Thyroid Neoplasms/*complications/diagnosis/therapy ; Thyroidectomy ; Thyroiditis/*complications/diagnosis/therapy ; Thyroiditis, Suppurative/*complications/diagnosis/therapy ; Thyroxine/therapeutic use ; Tomography, X-Ray Computed ; Treatment Outcome

Gene expression is suppressed by DNA methylation. The goal of this study was to identify genes whose CpG site methylation and mRNA expression are associated with recurrence after surgical resection for hepatocellular carcinoma (HCC). Sixty-two HCCs were examined by both whole genome DNA methylation and transcriptome analysis. The Cox model was used to select genes associated with recurrence. A validation was performed in an independent cohort of 66 HCC patients. Among fifty-nine common genes, increased CpG site methylation and decreased mRNA expression were associated with recurrence for 12 genes (Group A), whereas decreased CpG site methylation and increased mRNA expression were associated with recurrence for 25 genes (Group B). The remaining 22 genes were defined as Group C. Complement factor H (CFH) and myosin VIIA and Rab interacting protein (MYRIP) in Group A; proline/serine-rich coiled-coil 1 (PSRC1), meiotic recombination 11 homolog A (MRE11A), and myosin IE (MYO1E) in Group B; and autophagy-related protein LC3 A (MAP1LC3A), and NADH dehydrogenase 1 alpha subcomplex assembly factor 1 (NDUFAF1) in Group C were validated. In conclusion, potential tumor suppressor (CFH, MYRIP) and oncogenes (PSRC1, MRE11A, MYO1E) in HCC are reported. The regulation of individual genes by methylation in hepatocarcinogenesis needs to be validated.
Adult ; Aged ; Carcinoma, Hepatocellular/*genetics/pathology/surgery ; CpG Islands ; *DNA Methylation ; Female ; Gene Expression Profiling ; Gene Expression Regulation, Neoplastic ; Humans ; Liver/pathology ; Liver Neoplasms/*genetics/pathology/surgery ; Male ; Middle Aged ; Neoplasm Recurrence, Local/*genetics ; Oligonucleotide Array Sequence Analysis ; Proportional Hazards Models ; RNA, Messenger/biosynthesis ; Transcriptome/genetics