A case of acute kidney injury caused by dapsone-induced methemoglobinemia in a patient with chronic kidney disease

Methemoglobinemia (MetHb), which is characterized by an increased methemoglobin level, is a rare but potentially fatal disorder caused by congenital enzyme deficiencies or exposure to oxidizing agents, including dapsone. Elevation in the methemoglobin level impairs the oxygen-carrying capacity of hemoglobin, produces functional anemia, and induces tissue hypoxia. Such hypoxia results in microcirculation injury and hypoperfusion in the tissue and organs, including the kidney, and is a risk factor for acute kidney injury (AKI). This paper reports a case of AKI caused by dapsone-induced MetHb in a patient with chronic kidney disease, in which the patient ingested approximately 1,500 mg of dapsone in a suicide attempt, which was treated with aggressive management, including methylene blue, ascorbic acid, and transfusion.

Brain Tumor Classification by Methylation Profile

The goal of the methylation classifier in brain tumor classification is to accurately classify tumors based on their methylation profiles. Accurate brain tumor diagnosis is the first step for healthcare professionals to predict tumor prognosis and establish personalized treatment plans for patients. The methylation classifier can be used to perform classification on tumor samples with diagnostic difficulties due to ambiguous histology or mismatch between histopathology and molecular signatures, i.e., not otherwise specified (NOS) cases or not elsewhere classified (NEC) cases, aiding in pathological decision-making. Here, the authors elucidate upon the application of a methylation classifier as a tool to mitigate the inherent complexities associated with the pathological evaluation of brain tumors, even when pathologists are experts in histopathological diagnosis and have access to enough molecular genetic information. Also, it should be emphasized that methylome cannot classify all types of brain tumors, and it often produces erroneous matches even with high matching scores, so, excessive trust is prohibited. The primary issue is the considerable difficulty in obtaining reference data regarding the methylation profile of each type of brain tumor. This challenge is further amplified when dealing with recently identified novel types or subtypes of brain tumors, as such data are not readily accessible through open databases or authors of publications. An additional obstacle arises from the fact that methylation classifiers are primarily research-based, leading to the unavailability of charging patients. It is important to note that the application of methylation classifiers may require specialized laboratory techniques and expertise in DNA methylation analysis.

Neovascular Glaucoma Associated with Chronic Rhegmatogenous Retinal Detachment

Purpose@#To demonstrate the clinical features and natural course of chronic retinal detachment-associated neovascular glaucoma. @*Methods@#Ten patients, diagnosed with chronic retinal detachment-associated neovascular glaucoma during 2007-2016 were retrospectively investigated. Besides chronic retinal detachment, no patients had any neovascular glaucoma-predisposing conditions, such as carotid artery disease. Retinal perfusion status was evaluated from the fundus fluorescein angiography images. @*Results@#The mean age of patients was 57.5 years (range, 22-78 years). Complete retinal reattachment was achieved in three eyes, while partial or total chronic retinal detachment persisted in seven eyes. Wide-angle fundus fluorescein angiography revealed peripheral retinal capillary obstruction and severe nonperfusion. Neovascular glaucoma developed 213.4 months (range, 17-634 months) after retinal detachment. Three eyes received Ahmed valve implantation, while five eyes received intravitreal bevacizumab injection. Intraocular pressure was controlled in 10 eyes. Two eyes developed phthisis bulbi during follow-up. @*Conclusions@#In eyes with a chronic retinal detachment history, iris neovascularization and neovascular glaucoma can develop due to retinal capillary obstruction and chronic retinal ischemia, even after achieving retinal reattachment. We suggest regular follow-up examinations for patients with chronic retinal detachment, particularly for eyes with retinal nonperfusion, as detected on fundus fluorescein angiography.

Effect of BRCA1/2 Mutational Status on Survival Outcomes According to Secondary Cytoreductive Surgery and Maintenance Therapy in Platinum-Sensitive Relapsed Ovarian Cancer: A Real-World Evidence Study / Journal of the Korean Cancer Association, 대한암학회지

Purpose@#This study aimed to investigate the impact of BRCA1/2 mutational status on survival outcomes in patients with platinum-sensitive relapsed (PSR) epithelial ovarian cancer (EOC). @*Materials and Methods@#We retrospectively identified patients who received secondary treatment for PSR EOC at our institution between January 2007 and June 2021 and who underwent BRCA1/2 gene testing by either germline or somatic methods. The association between BRCA1/2 mutational status and survival outcomes was evaluated. Both secondary cytoreductive surgery (CRS) and maintenance therapy were stratified considering real-world clinical practice. @*Results@#Of 262 patients, 91 (34.7%) and 171 (65.3%) were assigned to BRCA1/2 mutation and wild-type groups, respectively. The two groups had similar proportions of patients undergoing secondary CRS (26.4% vs. 32.7%, p=0.286) and maintenance therapy (54.9% vs. 46.2%, p=0.178). Overall, no differences in progression-free survival (PFS; median, 19.7 vs. 15.1 months, p=0.120) and overall survival (OS; p=0.400) were observed between the two groups. In multivariate analyses, BRCA1/2 mutational status was not associated with PFS (adjusted hazard ratio, 0.816; 95% confidence interval, 0.596 to 1.119; p=0.207). BRCA1/2 mutational status did not affect PFS among patients who underwent secondary CRS (n=80) and among those who did not (n=182) (p=0.074 and p=0.222, respectively). PFS did not differ in the BRCA1/2 mutational status among the patients who received bevacizumab maintenance (n=90, p=0.992). @*Conclusion@#In this real-world evidence study, BRCA1/2 mutational status itself was not associated with PFS and OS in PSR EOC, which was consistent with whether secondary CRS or not and with bevacizumab maintenance.

Treatment results of carotid endarterectomy and carotid artery stenting for patients with radiation-induced carotid stenosis

Purpose@#Exposure to ionizing radiation over the head and neck accelerates atherosclerotic changes in the carotid arteries. Owing to the characteristics of radiation-induced carotid stenosis (RICS), the results regarding the optimal revascularization method for RICS vary. This study compared treatment outcomes between carotid endarterectomy (CEA) and carotid artery stenting (CAS) in RICS. @*Methods@#This was a single-center retrospective review of consecutive patients who underwent CEA or CAS for carotid stenosis. RICS was defined as carotid stenosis (>50%) with the prior neck irradiation for cancer treatment on either side.For the analyses, demographics, comorbid conditions, carotid lesion characteristics based on imaging studies, surgical complications, neurologic outcomes, and mortality during the follow-up period were reviewed. To compare CEA and CAS results in RICS, a 1:1 propensity score matching was applied. @*Results@#Between November 1994 and June 2021, 43 patients with RICS and 2,407 patients with non-RICS underwent carotid revascularization with CEA or CAS. RICS had fewer atherosclerotic risk factors and more frequent severe carotid stenosis and contralateral carotid occlusions than non-RICS. CAS was more commonly performed than CEA (22.9% vs.77.1%) for RICS due to more frequent unfavorable carotid anatomy (0 vs. 16.2%). Procedure-related complications were more common in the CEA than in the CAS. However, there was no significant difference in neurologic outcomes and restenosis rates between CEA and CAS in RICS. @*Conclusion@#Considering its lesion characteristics and cumulative incidence, RICS requires more attention than non-RICS.Although CAS has broader indications for RICS, CEA has shown acceptable results if selectively performed.

Complete response to local therapy for advanced hepatocellular carcinoma with lung metastasis: a case report

The concept of oligometastasis is widely accepted for various types of solid tumors; accordingly, better outcomes can be anticipated with aggressive local interventions. The treatment of advanced hepatocellular carcinoma (HCC) with extrahepatic metastasis is systemic therapy. However, treatment responses to systemic therapy are poor. Recently, a small number of metastatic cancers (oligometastasis) have been controlled by local therapy rather than systemic therapy. Our study reports a case of a 66-year-old male patient with advanced HCC with lung metastasis, which was treated with local therapy. There were less than four metastases in the lungs, which were treated with wedge resection, radiofrequency, and radiation therapy. He repeatedly underwent local therapy for lung oligometastasis and locoregional therapy for intrahepatic HCC rather than systemic therapy; control by local therapy was possible as his liver function was preserved with Child-Turcotte-Pugh class A.

Incidence and Risk Factors of Epiretinal Membrane after Cataract Surgery

Purpose@#To investigate the incidence and risk factors of epiretinal membrane (ERM) after cataract surgery. @*Methods@#We reviewed the medical records of patients who underwent cataract surgery between January 2016 and December 2018. Eyes with ERM, as observed by optical coherence tomography performed 1-2 months after surgery, were excluded from the study. ERM was diagnosed by fundus photography. The incidence rate was determined 2-6, 6-12, and 12-24 months after surgery. The cumulative incidence rate of ERM was calculated by Kaplan-Meier analysis. Data were analyzed using logistic regression to determine the associations between ERM development and various factors. @*Results@#The study included 218 eyes of 161 patients with a mean age of 66.6 ± 9.7 years at the time of surgery. ERM was observed in 3 of 218 eyes (1.4%) between 2 and 6 months, 15 of 176 eyes (8.3%) between 6 and 12 months, and 14 of 150 eyes (10.3%) between 12 and 24 months after surgery. The cumulative incidence rate of ERM was 11.7% over the 24-month period after surgery. In multivariate logistic regression analysis, older age (≥65 years; odds ratio [OR], 8.59, 95% confidence interval [CI], 1.43-51.49), fellow eye with ERM (OR, 3.63; 95% CI, 1.04-12.73), longer axial length (≥26 mm; OR, 8.02; 95% CI, 1.08-59.66), and complete posterior vitreous detachment development (OR, 7.48; 95% CI, 1.64-34.14) were significantly associated with ERM development. @*Conclusions@#ERM is not rare after cataract surgery. Long-term retinal examination should be required after cataract surgery.

Incidence and Risk Factors of Epiretinal Membrane after Cataract Surgery

Purpose@#To investigate the incidence and risk factors of epiretinal membrane (ERM) after cataract surgery. @*Methods@#We reviewed the medical records of patients who underwent cataract surgery between January 2016 and December 2018. Eyes with ERM, as observed by optical coherence tomography performed 1-2 months after surgery, were excluded from the study. ERM was diagnosed by fundus photography. The incidence rate was determined 2-6, 6-12, and 12-24 months after surgery. The cumulative incidence rate of ERM was calculated by Kaplan-Meier analysis. Data were analyzed using logistic regression to determine the associations between ERM development and various factors. @*Results@#The study included 218 eyes of 161 patients with a mean age of 66.6 ± 9.7 years at the time of surgery. ERM was observed in 3 of 218 eyes (1.4%) between 2 and 6 months, 15 of 176 eyes (8.3%) between 6 and 12 months, and 14 of 150 eyes (10.3%) between 12 and 24 months after surgery. The cumulative incidence rate of ERM was 11.7% over the 24-month period after surgery. In multivariate logistic regression analysis, older age (≥65 years; odds ratio [OR], 8.59, 95% confidence interval [CI], 1.43-51.49), fellow eye with ERM (OR, 3.63; 95% CI, 1.04-12.73), longer axial length (≥26 mm; OR, 8.02; 95% CI, 1.08-59.66), and complete posterior vitreous detachment development (OR, 7.48; 95% CI, 1.64-34.14) were significantly associated with ERM development. @*Conclusions@#ERM is not rare after cataract surgery. Long-term retinal examination should be required after cataract surgery.

Lymph Node Ratio Is a Strong Prognostic Factor in Patients with Early-Stage Cervical Cancer Undergoing Minimally Invasive Radical Hysterectomy

Purpose@#To determine whether the prognostic impact of lymph node ratio (LNR), defined as the ratio between the number of positive lymph nodes and removed lymph nodes, differs between open and minimally invasive surgical approaches for radical hysterectomy (RH) in node-positive, early-stage cervical cancer. @*Materials and Methods@#We retrospectively identified 2009 International Federation of Gynecology and Obstetrics stage IB1-IIA2 patients who underwent primary type C RH between 2010 and 2018. Among them, only those with pathologically proven lymph node metastases who received adjuvant radiation therapy were included. The prognostic significance of LNR was investigated according to open surgery and minimally invasive surgery (MIS). @*Results@#In total, 55 patients were included. The median LNR (%) was 9.524 (range, 2.083–62.500). Based on receiver operating characteristic curve analysis, the cut-off value for LNR (%) was determined as 8.831. Overall, patients with high LNR (≥8.831%;n=29) showed worse disease-free survival (DFS) than those with low LNR (<8.831%, n=26) (p=0.027), whereas no difference in overall survival was observed. Multivariate analyses adjusting for clinicopathologic factors revealed that DFS was adversely affected by both MIS [adjusted hazard ratio (HR), 8.132; p=0.038] and high LNR (adjusted HR, 10.837; p=0.045). In a subgroup of open surgery cases, LNR was not associated with disease recurrence. However, in a subgroup of MIS cases, high LNR was identified as an independent poor prognostic factor for DFS (adjusted HR, 14.578; p=0.034). @*Conclusion@#In patients with node-positive, early-stage cervical cancer, high LNR was associated with a significantly higher disease recurrence rate. This relationship was further consolidated among patients who received MIS RH.

The prognostic significance of p16 expression pattern in diffuse gliomas

Background@#CDKN2A is a tumor suppressor gene that encodes the cell cycle inhibitor protein p16. Homozygous deletion of the CDKN2A gene has been associated with shortened survival in isocitrate dehydrogenase (IDH)–mutant gliomas. This study aimed to analyze the prognostic value of p16 and to evaluate whether p16 immunohistochemical staining could be used as a prognostic marker to replace CDKN2A genotyping in diffuse gliomas. @*Methods@#p16 immunohistochemistry was performed on tissue microarrays of 326 diffuse gliomas with diagnoses that reflected IDH-mutations and 1p/19q codeletion status. The results were divided into three groups (negative, focal expression, overexpression) according to the presence and degree of p16 expression. Survival analysis was performed to assess the prognostic value of p16 expression. @*Results@#A loss of p16 expression predicted a significantly worse outcome in all glioma patients (n=326, p<.001), in the IDH-mutant glioma patients (n=103, p=.010), and in the IDH-mutant astrocytoma patients (n=73, p=.032). However, loss of p16 expression did not predict the outcome in the IDH-wildtype glioma patients (n=223, p=.121) or in the oligodendroglial tumor patients with the IDH-mutation and 1p/19q codeletion (n=30, p=.457). Multivariate analysis showed the association was still significant in the IDH-mutant glioma patients (p=.008; hazard ratio [HR], 2.637; 95% confidence interval [CI], 1.295 to 5.372) and in the IDH-mutant astrocytoma patients (p=.001; HR, 3.586; 95% CI, 1.649 to 7.801). Interestingly, patients who presented with tumors with p16 overexpression also had shorter survival times than did patients with tumors with p16 focal expression in the whole glioma (p< .001) and in IDH-mutant glioma groups. (p=.046). @*Conclusions@#This study suggests that detection of p16 expression by immunohistochemistry can be used as a useful surrogate test to predict prognosis, especially in IDH-mutant astrocytoma patients.

Factors associated with long-term graft patency after lower extremity arterial bypasses

Purpose@#This study was conducted to determine factors associated with long-term graft patency after lower extremity arterial bypass (LEAB). @*Methods@#Database of LEABs for patients with chronic arterial occlusive disease (CAOD) at a single institution was retrospectively reviewed. To determine the factors we compared demographic, clinical, and procedural variables between 2 patient groups; group I (graft patency < 2 years) and group II (graft patency ≥ 5 years after LEAB) using univariable and multivariable analyses. @*Results@#Among 957 LEABs, 259 limbs (group I, 125 limbs and group II, 134 limbs) in 213 patients were included for the analysis. On a univariable analysis, younger age (69 years vs. 66 years, P = 0.024), hypertension (60.8% vs. 74.6%, P = 0.017), claudication (51.2% vs. 70.9%, P = 0.001), absence of prior intervention (50.4% vs. 73.9%, P < 0.001), common femoral artery based bypass (57.6% vs. 70.1%, P = 0.035), above-the knee bypass (36.8% vs. 64.2%, P < 0.001), postoperative graft salvage procedure (3.2% vs. 14.8%, P = 0.001), and statin use (75.2% vs. 88.8, P = 0.004) were associated with long-term patency. On a multivariate analysis hypertension (odds ratio [OR], 1.91; P = 0.038), claudication (OR, 2.08; P = 0.032), no prior intervention (OR, 2.48; P = 0.001), vein graft (OR, 4.36; P = 0.001), above-the knee bypass (OR, 4.68; P < 0.001), and graft salvage procedures (OR, 7.70; P < 0.001) were identified as independent factors. @*Conclusion@#These factors can be considered in decision making before treatment of patients with CAOD.

Outcomes after Elective Open Abdominal Aortic Aneurysm Repair in Octogenarians Compared to Younger Patients in Korea

Background@#Although the first choice of treatment for abdominal aortic aneurysm (AAA) is endovascular aneurysm repair, especially in elderly patients, some patients require open surgical repair. The purpose of this study was to compare the mortality outcomes of open AAA repair between octogenarians and younger counterparts and to identify the risk factors associated with mortality. @*Methods@#All consecutive patients who underwent elective open AAA repair due to degenerative etiology at a single tertiary medical center between 1996 and June 2020 were included in this retrospective review. Medical records and imaging studies were reviewed to collect the following information: demographics, comorbid medical conditions, clinical presentations, radiologic findings, surgical details, and morbidity and mortality rates. For analysis, patients were divided into two groups: older and younger than 80 years of age. Multivariate analysis was performed to identify factors associated with mortality after elective open AAA repair. @*Results@#Among a total of 650 patients who underwent elective open AAA repair due to degenerative AAA during the study period, 58 (8.9%) were octogenarians and 595 (91.1%) were non-octogenarians. Patients in the octogenarian group were predominantly female and more likely to have lower body weight and body mass index (BMI), hypertension, chronic kidney disease, and lower preoperative serum hemoglobin and albumin compared with patients in the non-octogenarian group. Maximal aneurysm diameter was larger in octogenarians. During the median follow-up duration of 34.4 months for 650 patients, the median length of total hospital and intensive care unit stay was longer in octogenarians. The 30-day (1.7% vs. 0.7%, P= 0.374) and 1-year (6.9% vs. 2.9%, P = 0.108) mortality rates were not statistically significantly different between the two groups. Multivariate analysis showed that low BMI was associated with increased 30-day (odds ratio [OR], 16.339; 95% confidence interval [CI], 1.192–224.052; P= 0.037) and 1-year (OR, 8.236; CI, 2.301–29.477; P= 0.001) mortality in all patients. @*Conclusion@#Because the mortality rate of octogenarians after elective open AAA repair was not significantly different compared with their younger counterparts, being elderly is not a contraindication for open AAA repair. Low BMI might be associated with increased postoperative mortality.

Comparisons of survival outcomes between bevacizumab and olaparib in BRCA-mutated, platinum-sensitive relapsed ovarian cancer: a Korean Gynecologic Oncology Group study (KGOG 3052) / 부인종양

Objective@#To compare survival outcomes between bevacizumab (BEV) and olaparib (OLA) maintenance therapy in BRCA-mutated, platinum-sensitive relapsed (PSR) high-grade serous ovarian carcinoma (HGSOC). @*Methods@#From 10 institutions, we identified HGSOC patients with germline and/or somatic BRCA1/2 mutations, who experienced platinum-sensitive recurrence between 2013 and 2019, and received second-line platinum-based chemotherapy. Patients were divided into BEV (n=29), OLA (n=83), and non-BEVon-OLA users (n=36). The OLA and non-BEVon-OLA users were grouped as the OLA intent group. We conducted 1:2 nearest neighbor-matching between the BEV and OLA intent groups, setting the proportion of OLA users in the OLA intent group from 65% to 100% at 5% intervals, and compared survival outcomes among the matched groups. @*Results@#Overall, OLA users showed significantly better progression-free survival (PFS) than BEV users (median, 23.8 vs. 17.4 months; p=0.004). Before matching, PFS improved in the OLA intent group but marginal statistical significance (p=0.057). After matching, multivariate analyses adjusting confounders identified intention-to-treat OLA as an independent favorable prognostic factor for PFS in the OLA 65P (adjusted hazard ratio [aHR]=0.505; 95% confidence interval [CI]=0.280−0.911; p=0.023) to OLA 100P (aHR=0.348; 95% CI=0.184−0.658; p=0.001) datasets. The aHR of intention-to-treat OLA for recurrence decreased with increasing proportions of OLA users. No differences in overall survival were observed between the BEV and OLA intent groups, and between the BEV and OLA users. @*Conclusion@#Compared to BEV, intention-to-treat OLA and actual use of OLA maintenance therapy were significantly associated with decreased disease recurrence risk in patients with BRCA-mutated, PSR HGSOC.

Pathologic discrepancies between colposcopy-directed biopsy and loop electrosurgical excision procedure of the uterine cervix in women with cytologic high-grade squamous intraepithelial lesions / 부인종양

OBJECTIVE: To investigate pathologic discrepancies between colposcopy-directed biopsy (CDB) of the cervix and loop electrosurgical excision procedure (LEEP) in women with cytologic high-grade squamous intraepithelial lesions (HSILs).METHODS: We retrospectively identified 297 patients who underwent both CDB and LEEP for HSILs in cervical cytology between 2015 and 2018, and compared their pathologic results. Considering the LEEP to be the gold standard, we evaluated the diagnostic performance of CDB for identifying cervical intraepithelial neoplasia (CIN) grades 2 and 3, adenocarcinoma in situ, and cancer (HSIL+). We also performed age subgroup analyses.RESULTS: Among the study population, 90.9% (270/297) had pathologic HSIL+ using the LEEP. The diagnostic performance of CDB for identifying HSIL+ was as follows: sensitivity, 87.8%; specificity, 59.3%; balanced accuracy, 73.6%; positive predictive value, 95.6%; and negative predictive value, 32.7%. Thirty-three false negative cases of CDB included CIN2,3 (n=29) and cervical cancer (n=4). The pathologic HSIL+ rate in patients with HSIL− by CDB was 67.3% (33/49). CDB exhibited a significant difference in the diagnosis of HSIL+ compared to LEEP in all patients (p<0.001). In age subgroup analyses, age groups <35 years and 35–50 years showed good agreement with the entire data set (p=0.496 and p=0.406, respectively), while age group ≥50 years did not (p=0.036).CONCLUSION: A significant pathologic discrepancy was observed between CDB and LEEP results in women with cytologic HSILs. The diagnostic inaccuracy of CDB increased in those ≥50 years of age.

Pathologic discrepancies between colposcopy-directed biopsy and loop electrosurgical excision procedure of the uterine cervix in women with cytologic high-grade squamous intraepithelial lesions / 부인종양

OBJECTIVE@#To investigate pathologic discrepancies between colposcopy-directed biopsy (CDB) of the cervix and loop electrosurgical excision procedure (LEEP) in women with cytologic high-grade squamous intraepithelial lesions (HSILs).@*METHODS@#We retrospectively identified 297 patients who underwent both CDB and LEEP for HSILs in cervical cytology between 2015 and 2018, and compared their pathologic results. Considering the LEEP to be the gold standard, we evaluated the diagnostic performance of CDB for identifying cervical intraepithelial neoplasia (CIN) grades 2 and 3, adenocarcinoma in situ, and cancer (HSIL+). We also performed age subgroup analyses.@*RESULTS@#Among the study population, 90.9% (270/297) had pathologic HSIL+ using the LEEP. The diagnostic performance of CDB for identifying HSIL+ was as follows: sensitivity, 87.8%; specificity, 59.3%; balanced accuracy, 73.6%; positive predictive value, 95.6%; and negative predictive value, 32.7%. Thirty-three false negative cases of CDB included CIN2,3 (n=29) and cervical cancer (n=4). The pathologic HSIL+ rate in patients with HSIL− by CDB was 67.3% (33/49). CDB exhibited a significant difference in the diagnosis of HSIL+ compared to LEEP in all patients (p<0.001). In age subgroup analyses, age groups <35 years and 35–50 years showed good agreement with the entire data set (p=0.496 and p=0.406, respectively), while age group ≥50 years did not (p=0.036).@*CONCLUSION@#A significant pathologic discrepancy was observed between CDB and LEEP results in women with cytologic HSILs. The diagnostic inaccuracy of CDB increased in those ≥50 years of age.

Germline and Somatic BRCA1/2 Gene Mutational Status and Clinical Outcomes in Epithelial Peritoneal, Ovarian, and Fallopian Tube Cancer: Over a Decade of Experience in a Single Institution in Korea / Journal of the Korean Cancer Association, 대한암학회지

Purpose@#This study aimed to present a single institutional experience with BRCA1/2 gene tests and the effects of pathogenic mutations in epithelial peritoneal, ovarian, and fallopian tube cancer (POFTC) on survival outcomes. @*Materials and Methods@#We identified patients with epithelial POFTCs who underwent BRCA1/2 gene testing by either germline or somatic methods between March 2007 and March 2020. Based on the BRCA1/2 test results, patients were divided into BRCA mutation and wild-type groups, followed by comparisons of clinicopathologic characteristics and survival outcomes after primary treatment. @*Results@#The annual number of POFTC patients who received BRCA1/2 gene tests increased gradually. In total, 511 patients were included and BRCA1/2 mutations were observed in 143 (28.0%). Among 57 patients who received both germline and somatic tests, three (5.3%) showed discordant results from the two tests. Overall, no differences in progression-free survival (PFS; p=0.467) and overall survival (p=0.641) were observed between the BRCA mutation and wild-type groups; however, multivariate analyses identified BRCA1/2 mutation as an independent favorable prognostic factor for PFS (adjusted hazard ratio [aHR], 0.765; 95% confidence interval [CI], 0.593 to 0.987; p=0.040). In 389 patients with International Federation of Gynecology and Obstetrics stage III-IV, different results were shown depending on primary treatment strategy: while BRCA1/2 mutation significantly improved PFS in the subgroup of neoadjuvant chemotherapy (aHR, 0.619; 95% CI, 0.385 to 0.995; p=0.048), it did not affect patient PFS in the subgroup of primary debulking surgery (aHR, 0.759; 95% CI, 0.530 to 1.089; p=0.135). @*Conclusion@#BRCA1/2 mutations are frequently observed in patients with epithelial POFTCs, and such patients showed better PFS than did those harboring wild-type BRCA1/2.

Strategy to avoid open surgical conversion after endovascular aortic aneurysm repair for patients with infrarenal abdominal aortic aneurysm

Purpose@#Open surgical conversion (OSC) is the last treatment option for patients with endovascular aneurysm repair (EVAR) failure. We investigated the underlying causes of EVAR failure requiring OSC and attempted to determine strategies to avoid OSC after EVAR. @*Methods@#We retrospectively reviewed the database of patients who underwent OSC after EVAR from 2005 to 2018 in a single institution. Twenty-six OSCs were performed in 24 patients (median age, 74.5 years; 79.2% of males) who had undergone standard EVAR. We investigated pre-, intra-, and postoperative computed tomography or angiographic images and outcomes of the OSCs. @*Results@#Two main indications for OSC were persistent endoleak (50.0%) and endograft infection (EI) (38.5%). All 13 patients who underwent OSC due to endoleaks received EVAR outside of indications for use. Among 10 patients who underwent OSC due to EI, we found overlooked infection sources in 7 (70.0%) at the time of EVAR or during the surveillance period.OSC was performed at a median of 31.8 months (interquartile range, 9.4–69.8) after EVAR as an emergency (15.4%) or elective (84.6%) surgery. Aortic endograft was removed in 84.6% of cases (totally, 57.7%; partially, 26.9%), whereas it was preserved in 4 cases (15.4%). After 26 OSCs, 2 early deaths (7.7%) and 2 aortoenteric fistulae (7.7%) developed as major complications. @*Conclusion@#OSC after EVAR was associated with relatively higher perioperative morbidity and mortality. To avoid OSC after EVAR, we recommend careful assessment of coexisting infection sources and avoidance of EVAR for patients with especially unfavorable anatomy for EVAR, particularly the in proximal neck.

Differential Diagnosis and Treatment Outcomes of Tumors at the Carotid Bifurcation

Purpose@#Primary tumor at the carotid bifurcation is uncommon, which includes paraganglioma, schwannoma, and lymphoma. Due to their rarity, characteristics of these tumors and problems related to their surgical treatment have not been well known. We tried to elucidate different clinical characteristics and surgical complications of these tumors. @*Materials and Methods@#We retrospectively reviewed 21 patients with carotid bifurcation tumor from the Vascular Surgery division of a Korean tertiary institution from 1995 to 2018. We investigated patients’ demographics and clinical features, image characteristics, treatment details, and surgical outcomes. @*Results@#During the period from January 1995 to January 2018, we experienced surgical resections of 21 carotid bifurcation tumors which included 16 (76.2%) paragangliomas and 5 (23.8%) schwannomas. The most common clinical feature was a non-tender cervical mass. According to Shamblin classification, paragangliomas were classified into class II in 68.8% and class III in 25.0%. On the preoperative computed tomography images, all the paragangliomas showed characteristic splaying of the carotid bifurcation and hypervascularity of the tumors. On the contrary, all the schwannomas showed hypovascularity and splaying sign in 60%.Surgical complications related to cervical nerve injury developed in 50% and 60% of patients with Shamblin class III paraganglioma and schwannoma, respectively.During the mean follow-up period of 25 months (range, 1 to 163 months), distant metastases developed in 2 (12.5%) of paraganglioma patients. @*Conclusion@#Neurologic complications were more common after surgical resection of Shamblin class III paraganglioma and schwannoma. For patients with paraganglioma, postoperative periodic follow-up examination is advised to detect distant metastasis.

An Autopsy Proven Case of CSF1R-mutant Adult-onset Leukoencephalopathy with Axonal Spheroids and Pigmented Glia (ALSP) with Premature Ovarian Failure

Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) is a progressive degenerative white matter disorder caused by mutations in the tyrosine kinase domain of the CSF1R gene. ALSP is often misdiagnosed as other diseases due to its rarity and various clinical presentations such as Parkinsonism, pyramidal signs, cognitive impairment and/or psychiatric symptoms. We describe an autopsy case of ALSP with a CSF1R mutation. A 61-year-old woman presented insidious-onset gait difficulty for 12 years since her age of 49, and premature ovarian failure since her age of 35. At initial hospital visit, brain magnetic resonance imaging revealed hydrocephalus. Initially, Parkinson's syndrome was diagnosed, and she was prescribed L-dopa/carbidopa because of spasticity and rigidity of extremities, which had worsened. Subsequently, severe neuropsychiatric symptoms and cognitive impairment developed and radiologically, features of leukoencephalopathy or leukodystrophy were detected. She showed a down-hill course and died, 12 years after initial diagnosis. At autopsy, the brain showed severe symmetric atrophy of bilateral white matter, paper-thin corpus callosum, thin internal capsule, and marked hydrocephalus. Microscopically, diffuse loss of white matter, relatively preserved subcortical U-fibers, and many eosinophilic bulbous neuroaxonal spheroids were noted, but there was no calcification. Pigmented glia with brown cytoplasmic pigmentation were readily found in the white matter, which were positive for Periodic acid-Schiff, p62, and CD163 stains, but almost negative for CD68. Whole-exome and Sanger sequencing revealed a CSF1R mutation (c.2539G>A, p.Glu847Lys) which was reported in prior one ALSP case. This example demonstrates that ALSP could be associated with premature ovarian failure.

Combination Therapy of Angiotherapy, Radiation Therapy, and Radiofrequency Ablation for Pulmonary Metastasis of Hepatocellular Carcinoma Accompanied by Nontuberculous Mycobacteria

With the advances in hepatocellular carcinoma (HCC) treatment, the lung metastasis of HCC is becoming increasingly important. In treating the lung metastasis of HCC, a multidisciplinary approach can lead to better results than systemic chemotherapy alone. Here, we report on a patient who presented with pulmonary masses, while the HCC was being controlled in the abdominal cavity. The presence of nontuberculous mycobacteria was identified during the diagnosis of the pulmonary masses. The pulmonary metastases of HCC were treated with a combination of angiotherapy, radiation therapy, and radiofrequency ablation. The patient showed a satisfactory progress with this multidisciplinary localized treatment. We report the clinical progress and review the recent literature regarding the treatment of pulmonary metastasis without intrahepatic HCC herein.