Purpose: To compare the embolization effects of a non-fibered pushable coil with a conventional fibered pushable coil in an in vitro bench-top experiment. Materials and Methods: A simplified vascular phantom with 4 channels (1 for the non-fibered coil, 1 for the fibered coil, and 2 for continuous circuit flow) was used. A single coil of the longest length was inserted to evaluate the effect of single-coil embolization, and 3 consecutive coils were inserted to assess the effect of multiple-coil embolization. Post-embolization angiography was performed to obtain flow variables (time to peak [TTP], relative peak intensity [rPI], and angiographic flow reduction score [AFRS]) from time density curves. The packing densities of the two coil types were calculated, and the AFRS of each channel was determined by dividing the TTP by the rPI. Results: When inserting a single coil, the conventional fibered coil demonstrated better flow reduction, as indicated by a higher AFRS (25.6 vs. 17.4, P=0.034). However, the non-fibered coil exhibited a significantly higher packing density (12.9 vs. 2.4, P=0.001). Similar trends were observed with multiple coils. Conclusion The conventional fibered pushable coil showed better flow reduction efficiency, while the non-fibered pushable coil had a higher packing density, likely due to the flexibility of the coil loops. A better understanding of the distinct characteristics of different pushable coils can enhance the outcomes of various vascular embolization.

Purpose: NUT carcinoma (NC) is a solid tumor caused by the rearrangement of NUTM1 that usually develops in midline structures, such as the thorax. No standard treatment has been established despite high lethality. Thus, we investigated whether targeting the junction region of NUTM1 fusion breakpoints could serve as a potential treatment option for NC. Materials and Methods: We designed and evaluated a series of small interfering RNAs (siRNAs) targeting the junction region of BRD4-NUTM1 fusion (B4N), the most common form of NUTM1 fusion. Droplet digital polymerase chain reaction using the blood of patients was also tested to evaluate the treatment responses by the junction sequence of the B4N fusion transcripts. Results: As expected, the majority of NC fusion types were B4N (12 of 18, 67%). B4N fusion-specific siRNA treatment on NC cells showed specific inhibitory effects on the B4N fusion transcript and fusion protein without affecting the endogenous expression of the parent genes, resulting in decreased relative cell growth and attenuation of tumor size. In addition, the fusion transcript levels in platelet-rich-plasma samples of the NC patients with systemic metastasis showed a negative correlation with therapeutic effect, suggesting its potential as a measure of treatment responsiveness. Conclusion This study suggests that tumor-specific sequences could be used to treat patients with fusion genes as part of precision medicine for a rare but deadly disease.

Neurofibromatosis type 1 is the most common tumor predisposition syndrome inherited in an autosomal dominant (100% penetrance) fashion with a wide variety of expressivity. From the perspective of plastic surgery, the most significant clinical symptoms, including disfiguration, peripheral neurologic symptoms, and skeletal abnormalities, are caused by various tumors originating from the affected nerves. Surgical removal is the standard of care for these tumors. However, the outcome is frequently unsatisfactory, facilitating the search for additional therapeutic adjuvants. Current trials of molecularly targeted therapies are promising.Abbreviations: CALMs, café-au-lait macules; CNs, cutaneous neurofibromas; FDG, 18F-fluoro-deoxy-glucose; MAPK, mitogen-activated protein kinase; MPNSTs, malignant peripheral nerve sheath tumors; MRI, magnetic resonance imaging; NF1, neurofibromatosis type 1; NIH, National Institutes of Health; PET, positron emission tomography; PN, plexiform neurofibromas; TME, tumor microenvironment

Background: Surgical ciliated cysts, also known as postoperative maxillary cysts or implantation cysts, occur mainly in the posterior maxilla after radical maxillary sinus surgery; they rarely develop in the mandible. They are thought to occur when the sinonasal epithelium is infiltrated by a surgical instrument during surgery or as a result of transplantation of bone or cartilage with respiratory epithelium attached.Case presentationWe report a case in which a surgical ciliated cyst developed in the anterior part of the mandible, presumably as a result of bimaxillary orthognathic surgery and genioplasty performed 24 years earlier. We then review the few similar cases reported in the literature. Conclusion Surgical ciliated cysts in the mandible are extremely rare, but they could occur after simultaneous surgery on the maxilla and mandible, even decades later. To prevent surgical ciliated cysts in the mandible, we recommend that the surgical instruments, especially the saw blade used during bimaxillary surgery, be new or cleaned and that previously placed plates and screws be removed at an appropriate time.

Purpose: To find biomarkers for disease, there have been constant attempts to investigate the genes that differ from those in the disease groups. However, the values that lie outside the overall pattern of a distribution, the outliers, are frequently excluded in traditional analytical methods as they are considered to be ‘some sort of problem.’ Such outliers may have a biologic role in the disease group. Thus, this study explored new biomarker using outlier analysis, and verified the suitability of therapeutic potential of two genes (TM4SF4 and LRRK2). Materials and Methods: Modified Tukey’s fences outlier analysis was carried out to identify new biomarkers using the public gene expression datasets. And we verified the presence of the selected biomarkers in other clinical samples via customized gene expression panels and tissue microarrays. Moreover, a siRNA-based knockdown test was performed to evaluate the impact of the biomarkers on oncogenic phenotypes. Results: TM4SF4 in lung cancer and LRRK2 in breast cancer were chosen as candidates among the genes derived from the analysis. TM4SF4 and LRRK2 were overexpressed in the small number of samples with lung cancer (4.20%) and breast cancer (2.42%), respectively. Knockdown of TM4SF4 and LRRK2 suppressed the growth of lung and breast cancer cell lines. The LRRK2 overexpressing cell lines were more sensitive to LRRK2-IN-1 than the LRRK2 under-expressing cell lines Conclusion Our modified outlier-based analysis method has proved to rescue biomarkers previously missed or unnoticed by traditional analysis showing TM4SF4 and LRRK2 are novel target candidates for lung and breast cancer, respectively.

Gorham-Stout disease (GSD) was first described by Gorham and colleagues in 1954, but its precise mechanism and cause remain to be elucidated. In this condition, voluminous and potentially fatal chylous effusions into the thorax can occur. Herein, we describe a case of GSD in which the patient presented with massive pleural effusions and mottled osteolytic bone lesions. We performed multiple operations, including thoracic duct ligation using video-assisted thoracoscopic surgery and thoracotomic decortication, but these procedures did not succeed in preventing recurrent pleural effusion and chest wall lymphedema. After administering sirolimus (0.8 mg/m2, twice a day) and propranolol (40 mg, twice a day), the process of GSD in this patient has been controlled for more than 2 years.
Chylothorax ; Humans ; Ligation ; Lymphedema ; Osteolysis, Essential ; Pleural Effusion ; Propranolol ; Sirolimus ; Thoracic Duct ; Thoracic Surgery, Video-Assisted ; Thoracic Wall ; Thorax

BACKGROUND: The primary goal of this study was to characterize the clinical outcomes of adult patients with hematologic malignancies (HM) who were treated with extracorporeal membrane oxygenation (ECMO) support when conventional treatments failed. METHODS: In this retrospective, observational study at a tertiary medical center, we reviewed the clinical course of 23 consecutive patients with HM requiring ECMO who were admitted to the intensive care unit at Asan Medical Center from March 2010 to April 2015. RESULTS: A total of 23 patients (8 female; median age, 44 years; range, 29–51 years) with HM and severe acute circulatory and/or respiratory failure received ECMO therapy during the study period. Fourteen patients received veno-arterial ECMO, while 9 patients received veno-venous ECMO. The median ECMO duration was 104.7 hours (range, 37.1–221 hours). Nine patients were successfully weaned from ECMO. The in-hospital mortality rate was 91.1% (21 of 23). There were complications in 3 patients (cannulation site bleeding, limb ischemia, and gastrointestinal bleeding). CONCLUSION: ECMO is a useful treatment for patients with circulatory and/or pulmonary failure. However, in patients with HM, the outcomes of ECMO treatment results were very poor, so it is advisable to carefully decide whether to apply ECMO to these patients.
Adult ; Chungcheongnam-do ; Extracorporeal Membrane Oxygenation ; Extremities ; Female ; Hematologic Neoplasms ; Hemorrhage ; Hospital Mortality ; Humans ; Intensive Care Units ; Ischemia ; Mortality ; Observational Study ; Respiratory Insufficiency ; Retrospective Studies

Gorham-Stout disease (GSD) was first described by Gorham and colleagues in 1954, but its precise mechanism and cause remain to be elucidated. In this condition, voluminous and potentially fatal chylous effusions into the thorax can occur. Herein, we describe a case of GSD in which the patient presented with massive pleural effusions and mottled osteolytic bone lesions. We performed multiple operations, including thoracic duct ligation using video-assisted thoracoscopic surgery and thoracotomic decortication, but these procedures did not succeed in preventing recurrent pleural effusion and chest wall lymphedema. After administering sirolimus (0.8 mg/m2, twice a day) and propranolol (40 mg, twice a day), the process of GSD in this patient has been controlled for more than 2 years.

BACKGROUND: The primary goal of this study was to characterize the clinical outcomes of adult patients with hematologic malignancies (HM) who were treated with extracorporeal membrane oxygenation (ECMO) support when conventional treatments failed. METHODS: In this retrospective, observational study at a tertiary medical center, we reviewed the clinical course of 23 consecutive patients with HM requiring ECMO who were admitted to the intensive care unit at Asan Medical Center from March 2010 to April 2015. RESULTS: A total of 23 patients (8 female; median age, 44 years; range, 29–51 years) with HM and severe acute circulatory and/or respiratory failure received ECMO therapy during the study period. Fourteen patients received veno-arterial ECMO, while 9 patients received veno-venous ECMO. The median ECMO duration was 104.7 hours (range, 37.1–221 hours). Nine patients were successfully weaned from ECMO. The in-hospital mortality rate was 91.1% (21 of 23). There were complications in 3 patients (cannulation site bleeding, limb ischemia, and gastrointestinal bleeding). CONCLUSION ECMO is a useful treatment for patients with circulatory and/or pulmonary failure. However, in patients with HM, the outcomes of ECMO treatment results were very poor, so it is advisable to carefully decide whether to apply ECMO to these patients.