Loss of aquaporin-1 expression is associated with worse clinical outcomes in clear cell renal cell carcinoma: an immunohistochemical study

Background@#Aquaporin (AQP) expression has been investigated in various malignant neoplasms, and the overexpression of AQP is related to poor prognosis in some malignancies. However, the expression of AQP protein in clear cell renal cell carcinoma (ccRCC) has not been extensively investigated by immunohistochemistry with large sample size. @*Methods@#We evaluated the AQP expression in 827 ccRCC with immunohistochemical staining in tissue microarray blocks and classified the cases into two categories, high and low expression. @*Results@#High expression of aquaporin-1 (AQP1) was found in 320 cases (38.7%), but aquaporin-3 was not expressed in ccRCC. High AQP1 expression was significantly related to younger age, low TNM stage, low World Health Organization/International Society of Urologic Pathology nuclear grade, and absence of distant metastasis. Furthermore, high AQP1 expression was also significantly associated with longer overall survival (OS; p<.001) and progression-specific survival (PFS; p<.001) and was an independent predictor of OS and PFS in ccRCC. @*Conclusions@#Our study revealed the prognostic significance of AQP1 protein expression in ccRCC. These findings could be applied to predict the prognosis of ccRCC.

A dual-plane approach for surgical treatment of pseudoaneurysm with arteriovenous fistula in hemodialysis patients

Background@#We report the efficacy of a dual-plane approach using a Dufourmentel skin flap with a purse-string suture of the de-epithelized dermis to manage pseudoaneurysm at the vascular access site for hemodialysis. @*Methods@#A retrospective analysis was conducted of 61 patients from 2013 to 2018 with pseudoaneurysms at the arteriovenous fistula or graft who were treated with rhomboid excision, vessel repair with a purse-string suture, and a full-thickness Dufourmentel skin flap. The success rate was defined as the probability of complete wound closure and intact vascular access patency without infection or other complications. @*Results@#The success rate was 93.4% at 6 months postoperatively. Complications included newly occurring pseudoaneurysms (n=2), wound dehiscence (n=1) and bleeding (n=1). There were no complications such as stenosis or thrombosis from the procedure. @*Conclusions@#A dual-plane approach using a Dufourmentel skin flap with a purse-string suture for vessel repair was shown to be a favorable option for managing stable, small (diameter <2 cm) pseudoaneurysms without infection, rapid expansion, or patency issues of the vascular access.

Real-World Clinical Outcomes and Prognostic Factors for Patients with Advanced Angiosarcoma who Received Systemic Treatment / Journal of the Korean Cancer Association, 대한암학회지

Purpose@#Angiosarcoma is a highly aggressive mesenchymal tumor. Although systemic chemotherapy is often considered for the inoperable or metastatic angiosarcoma, the outcome of such treatment is unsatisfactory and poorly delineated. @*Materials and Methods@#We reviewed electronic medical records of 75 patients with angiosarcoma who were treated with systemic chemotherapy for inoperable or metastatic disease. Patients were classified as having liver involvement if they had either primary or metastatic hepatic lesions. @*Results@#Among the patients evaluated, 51 patients were male (68%) and 24 patients (32%) had primary cutaneous angiosarcoma. Liver involvement was present in 28 patients (37.3%). A total of 59 patients received first-line weekly paclitaxel (wPac) and showed an objective response rate (ORR) of 23.7% (n=14), a median progression free survival (mPFS) of 4.0 months (95% confidence interval [CI] 3.0–6.1), and a median overall survival (mOS) of 10.2 months (95% CI 7.0–14.6). Among patients without liver involvement, patients receiving wPac (n=35) had significantly prolonged mPFS (5.8 vs. 3.2 months, respectively, p=0.014) with a tendency for prolonged mOS (13.8 vs. 11.6 months, respectively, p=0.13) than those receiving other regimens (n=12). A total of 24 patients received second- or later-line pazopanib monotherapy and showed an ORR of 16.7% (n=4), a mPFS of 2.4 months (95% CI 1.8–4.3) and a mOS of 5.4 months (95% CI 3.5–NA). @*Conclusion@#Treatment with first-line wPac and later-line pazopanib seems to provide survival benefit, especially for patients with advanced angiosarcoma without liver involvement.

Histopathologic and clinicopathologic classifications of antineutrophil cytoplasmic antibody-associated glomerulonephritis: a validation study in a Korean cohort

Background@#Antineutrophil cytoplasmic antibodies (ANCA)-associated glomerulonephritis (AAGN) is a common cause of rapidly progressive glomerulonephritis and requires prompt and proper immunosuppressive therapy to improve renal prognosis. This study aimed to evaluate the predictive value of two different classifications for renal outcomes in Korean AAGN patients. @*Methods@#Ninety-two patients who were diagnosed with AAGN at two tertiary hospitals between 2004 and 2018 were retrospectively analyzed retrospectively. The histopathologic classification according to glomerular pathology and the clinicopathologic classification according to normal glomeruli ratio, degree of interstitial fibrosis/tubular atrophy, and baseline renal function were evaluated using the Cox proportional hazards model. @*Results@#Forty-five patients (48.9%) progressed to end-stage kidney disease (ESKD) during the observation period. The mean age was 61.0 ± 15.3 years, and most patients had myeloperoxidase-ANCA (93.5%). In the histopathologic classification, the best renal survival occurred in the focal class, whereas the sclerotic class had the worst renal survival (sclerotic class vs. focal class; adjusted hazard ratio [aHR], 5.05; 95% confidence interval [CI], 1.32–19.31; p = 0.018). The mixed class had intermediate renal outcomes (mixed class vs. focal class; aHR, 4.23; 95% CI, 1.23–14.58; p = 0.022). In the clinicopathologic classification, the high-risk group had poor renal outcomes compared with the low-risk group (aHR, 6.56; 95% CI, 1.25–34.26; p = 0.026), but renal outcomes did not differ between the low- and medium-risk groups. @*Conclusion@#In Korean AAGN patients, histopathologic and clinicopathologic classifications had predictive value for renal outcomes, especially in the sclerotic class or the high-risk group with higher risk of progression to ESRD despite treatment.

A dual-plane approach for surgical treatment of pseudoaneurysm with arteriovenous fistula in hemodialysis patients

Background@#We report the efficacy of a dual-plane approach using a Dufourmentel skin flap with a purse-string suture of the de-epithelized dermis to manage pseudoaneurysm at the vascular access site for hemodialysis. @*Methods@#A retrospective analysis was conducted of 61 patients from 2013 to 2018 with pseudoaneurysms at the arteriovenous fistula or graft who were treated with rhomboid excision, vessel repair with a purse-string suture, and a full-thickness Dufourmentel skin flap. The success rate was defined as the probability of complete wound closure and intact vascular access patency without infection or other complications. @*Results@#The success rate was 93.4% at 6 months postoperatively. Complications included newly occurring pseudoaneurysms (n=2), wound dehiscence (n=1) and bleeding (n=1). There were no complications such as stenosis or thrombosis from the procedure. @*Conclusions@#A dual-plane approach using a Dufourmentel skin flap with a purse-string suture for vessel repair was shown to be a favorable option for managing stable, small (diameter <2 cm) pseudoaneurysms without infection, rapid expansion, or patency issues of the vascular access.

Histopathologic and clinicopathologic classifications of antineutrophil cytoplasmic antibody-associated glomerulonephritis: a validation study in a Korean cohort

Background@#Antineutrophil cytoplasmic antibodies (ANCA)-associated glomerulonephritis (AAGN) is a common cause of rapidly progressive glomerulonephritis and requires prompt and proper immunosuppressive therapy to improve renal prognosis. This study aimed to evaluate the predictive value of two different classifications for renal outcomes in Korean AAGN patients. @*Methods@#Ninety-two patients who were diagnosed with AAGN at two tertiary hospitals between 2004 and 2018 were retrospectively analyzed retrospectively. The histopathologic classification according to glomerular pathology and the clinicopathologic classification according to normal glomeruli ratio, degree of interstitial fibrosis/tubular atrophy, and baseline renal function were evaluated using the Cox proportional hazards model. @*Results@#Forty-five patients (48.9%) progressed to end-stage kidney disease (ESKD) during the observation period. The mean age was 61.0 ± 15.3 years, and most patients had myeloperoxidase-ANCA (93.5%). In the histopathologic classification, the best renal survival occurred in the focal class, whereas the sclerotic class had the worst renal survival (sclerotic class vs. focal class; adjusted hazard ratio [aHR], 5.05; 95% confidence interval [CI], 1.32–19.31; p = 0.018). The mixed class had intermediate renal outcomes (mixed class vs. focal class; aHR, 4.23; 95% CI, 1.23–14.58; p = 0.022). In the clinicopathologic classification, the high-risk group had poor renal outcomes compared with the low-risk group (aHR, 6.56; 95% CI, 1.25–34.26; p = 0.026), but renal outcomes did not differ between the low- and medium-risk groups. @*Conclusion@#In Korean AAGN patients, histopathologic and clinicopathologic classifications had predictive value for renal outcomes, especially in the sclerotic class or the high-risk group with higher risk of progression to ESRD despite treatment.

Endoscopic Full-Thickness Resection for Gastric Subepithelial Lesions Arising from the Muscularis Propria

Most cases of gastric subepithelial lesions follow a good clinical course; however, some lesions progress to malignant tumors, and treatment of tumors with a high risk of malignancy is essential. Surgical excision has been the primary treatment for tumors originating from the propria muscle layer, but it has the disadvantages of being invasive and causing postoperative functional abnormalities. With the development of endoscopic techniques and instruments, the role of endoscopic resection, which is a less invasive method for the removal of gastric subepithelial lesions, has been attracting attention. We performed an endoscopic full-thickness resection for 8 patients with gastric subepithelial lesions originating from the muscularis propria. No fatal complications occurred. Our findings suggest the need to develop various devices for resection and closure and to accumulate further experience through additional studies to prevent complications and specimen loss.

Consensus regarding diagnosis and management of atypical hemolytic uremic syndrome

Thrombotic microangiopathy (TMA) is defined by specific clinical characteristics, including microangiopathic hemolytic anemia, thrombocytopenia, and pathologic evidence of endothelial cell damage, as well as the resulting ischemic end-organ injuries. A variety of clinical scenarios have features of TMA, including infection, pregnancy, malignancy, autoimmune disease, and medications. These overlapping manifestations hamper differential diagnosis of the underlying pathogenesis, despite recent advances in understanding the mechanisms of several types of TMA syndrome. Atypical hemolytic uremic syndrome (aHUS) is caused by a genetic or acquired defect in regulation of the alternative complement pathway. It is important to consider the possibility of aHUS in all patients who exhibit TMA with triggering conditions because of the incomplete genetic penetrance of aHUS. Therapeutic strategies for aHUS are based on functional restoration of the complement system. Eculizumab, a monoclonal antibody against the terminal complement component 5 inhibitor, yields good outcomes that include prevention of organ damage and premature death. However, there remain unresolved challenges in terms of treatment duration, cost, and infectious complications. A consensus regarding diagnosis and management of TMA syndrome would enhance understanding of the disease and enable treatment decision-making.

Eccrine Porocarcinoma: A Multicenter Retrospective Study with Review of the Literatures Reported in Korea

Background@#Eccrine porocarcinoma (EPC) is a rare malignant cutaneous adnexal tumor. Other than several scattered case reports, no comprehensive review on EPC has been conducted in Korea. @*Objective@#To clinicopathologically review all EPC cases from our institutions as well as those reported in Korea. @*Methods@#Medical records and histopathological slides of EPC cases in the skin biopsy registries of our institutions were retrospectively reviewed. Additionally, EPC cases reported in Korea before June 2019 were retrieved by searching the PubMed, KoMCI, KoreaMed, and KMbase databases. @*Results@#Nine EPC cases from our institutions were included in the study. In addition, 27 reports of 28 patients with EPC were reported in Korea. A total of 37 patients with EPC were identified, consisting of 19 males (male:female ratio, 1.06:1; mean age at diagnosis, 65.6 years). The most common site of primary tumor was the head and neck (29.7%). Wide excision was the most common (78.4%) treatment method. Initial metastasis work-up imaging studies were performed in 18 patients (48.6%), and metastasis was confirmed in eight patients (21.6%). @*Conclusion@#EPC is a rare cutaneous carcinoma in Korea. EPC usually affects elderly patients, with no sexual predilection. Due to possible metastasis, careful diagnosis and appropriate metastasis workups are warranted in EPC.

Gorham-Stout Syndrome with Focal Segmental Glomerulosclerosis: A Case Report

Gorham-Stout syndrome is a rare bone disorder characterized by progressive massive osteolysis and proliferation of vascular and lymphatic vessels. A 15-year-old boy was initially diagnosed with Gorham-Stout at the age of 8 years based on clinical and radiological findings. Following diagnosis, he was treated with pamidronate, interferon alfa, propranolol, oral corticosteroids, and sirolimus. He developed proteinuria at the age of 15 and progressed into the nephrotic range 2 years later. A renal biopsy revealed focal segmental glomerulosclerosis, not otherwise specified variant. The sequential increase in proteinuria associated with medications suggested that the focal segmental glomerulosclerosis may be caused by pamidronate and sirolimus, but cannot completely rule out the possibility of kidney involvement of GSS itself.

A Pediatric Case of a D-Penicillamine Induced ANCA-associated Vasculitis Manifesting a Pulmonary-Renal Syndrome

D-penicillamine has been reported to cause antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis presenting as rapidly progressive glomerulonephritis or pulmonary-renal syndrome mostly in adults. We report a pediatric case of D-penicillamine induced ANCA-associated vasculitis that manifests as a pulmonary-renal syndrome with a mild renal manifestation. A 13-year-old girl who has been taking D-penicillamine for five years under the diagnosis of Wilson disease visited the emergency room because of hemoptysis and dyspnea. She had diffuse pulmonary hemorrhage, microscopic hematuria, and proteinuria. Myeloperoxidase ANCA was positive, and a renal biopsy revealed pauci-immune crescentic glomerulonephritis. Under the diagnosis of D-penicillamine-induced ANCA-associated vasculitis, D-penicillamine was switched to trientine, and the patient was treated with plasmapheresis, glucocorticoid, cyclophosphamide, and mycophenolate mofetil. Pulmonary hemorrhage improved rapidly followed by the disappearance of the hematuria and proteinuria five months later.

Possibility of Injecting Adipose-Derived Stromal Vascular Fraction Cells to Accelerate Microcirculation in Ischemic Diabetic Feet: A Pilot Study

BACKGROUND AND OBJECTIVES: Beneficial effects of human adipose-derived stromal vascular fraction (SVF) cell injection on microcirculation have been recently reported in in vitro and in vivo studies. However, no clinical studies have reported its effect in diabetic patients who commonly experience compromised tissue perfusion, regardless of the status of intravascular blood flow. The present piloting study was designed to clinically examine the possibility of SVF cell injection to accelerate microcirculation, particularly in ischemic diabetic feet. METHODS: Ten diabetic feet were included to receive subcutaneous injection of SVF cells around wounds. Transcutaneous partial oxygen pressure (TcPO2) and cutaneous microvascular blood flow were measured before and every four weeks after cell injection until the 12th week visit. RESULTS: TcPO2 values increased from 31.3±7.4 before injection to 46.4±8.2 mmHg at 12 weeks after SVF injection (1.5-fold, p<0.05). Cutaneous microvascular blood flow levels increased from 34.0±21.1 before injection to 76.1±32.5 perfusion unit at 12 weeks after SVF injection (2.2-fold, p<0.05). There were no adverse events related to SVF cell injection. CONCLUSIONS: Results of this study demonstrate that adipose-derived SVF cell injection have the possibility to provide beneficial effects on microcirculation in ischemic diabetic feet.

Choice of recipient vessels in muscle-sparing transverse rectus abdominis myocutaneous flap breast reconstruction: A comparative study

BACKGROUND: Thoracodorsal vessels (TDVs) and internal mammary vessels (IMVs) have both been widely employed as recipient vessels for use in free muscle-sparing transverse rectus abdominis myocutaneous (MS-TRAM) flaps. However, whether TDVs or IMVs are preferable as recipient vessels for autologous breast reconstruction with a free MS-TRAM flap remains controversial. The purpose of this study was to compare the clinical outcomes when TDVs were used as recipient vessels to those obtained when IMVs were used as recipient vessels for autologous breast reconstruction with a free MS-TRAM flap. METHODS: A retrospective matched-cohort study was performed. We retrospectively reviewed data collected from patients who underwent a free MS-TRAM flap for autologous breast reconstructions after mastectomy between March 2003 and June 2013. After a one-to-one matching using age, 100 autologous breast reconstructions were selected in this study. Of the 100 breast reconstructions, 50 flaps were anastomosed to TDVs and 50 to IMVs. Patient demographics and clinical outcomes including operation time, length of hospital stay, postoperative complications, and aesthetic score were compared between the two groups. RESULTS: No statistically significant differences were found between the two groups in patient demographics and clinical outcomes, including the complication rates and aesthetic scores. There were no major complications such as total or partial flap loss in either group. CONCLUSIONS: The results of our study demonstrate that both TDVs and IMVs were safe and efficient as recipient vessels in terms of the complication rates and aesthetic outcomes.

Survival outcomes after adjuvant radiotherapy for aggressive fibromatosis depend on time frame and nuclear β-catenin

PURPOSE: To identify prognostic factors influencing progression-free survival (PFS) of aggressive fibromatosis (AF) after postoperative radiotherapy (PORT) and assess correlations between immunohistochemistry (IHC) features of β-catenin/smooth muscle actin (SMA) and PFS. MATERIALS AND METHODS: Records of 37 patients with AF treated by PORT from 1984 to 2015 were retrospectively reviewed. Fifteen patients underwent wide excision for AF and 22 patients received debulking operation. The median total dose of PORT was 59.4 Gy. IHC staining results of β-catenin and SMA were available for 11 and 12 patients, respectively. RESULTS: The median follow-up duration was 105.9 months. Five-year PFS rate was 70.9%. Tumor size or margin status was not related to PFS in univariate analysis (p = 0.197 and p = 0.716, respectively). Multivariate analysis showed that increased interval from surgery to PORT (>5.7 weeks) was a marginal risk factor for PFS (p = 0.054). Administration of PORT at the initial diagnosis resulted in significantly improved PFS compared to deferring PORT after recurrence (p = 0.045). Patient with both risk factors of deferring PORT after recurrence and interval from surgery to PORT >5.7 weeks had significantly lower 5-year PFS than patients without risk factor (34.1% vs. 100.0%; p = 0.012). Nuclear β-catenin intensity tended to inversely correlate with 5-year PFS, although it did not reach statistical significance (62.5% at low vs. 100.0% at high; p = 0.260). SMA intensity was not related to PFS (p = 0.700). CONCLUSION: PORT should be performed immediately after surgery irrespective of margin status or tumor size especially in recurrent case. Nuclear β-catenin staining intensity of IHC might correlate with local recurrence.

Reninoma: a rare cause of curable hypertension / 소아과

The most common type of refractory hypertension found in children is secondary hypertension, which is a potentially curable disease. Reninoma, a renin-secreting juxtaglomerular cell tumor, is a rare cause of severe hypertension that is usually diagnosed in adolescents and young adults. Surgical resection of the tumor completely cures the hypertension of patients with reninoma. The typical clinical presentation of reninoma includes hypokalemia, metabolic alkalosis, and features secondary to the increased activation of the renin-angiotensin system without renal artery stenosis. We report a case of reninoma in a female adolescent with a typical clinical presentation, in which surgical removal of the tumor completely cured hypertension. We discuss here the clinical features, imaging studies, and immunohistochemical examination of the tumor used to establish the diagnosis of reninoma and for the management of the condition.

A Pediatric Case of Inflammatory Bowel Disease with Renal Amyloidosis

Amyloidosis is a rare disease that results from the deposition of extracellular protein in various body tissues, causing progressive organ dysfunction. Secondary renal amyloidosis is a rare but serious complication of chronic inflammatory bowel disease, particularly in patients with Crohn's disease or ulcerative colitis. We report a case of secondary renal amyloidosis in a pediatric patient who reported a 16-year history of “very early onset inflammatory bowel disease”. Intensive treatment including repeated infliximab infusions improved clinical parameters of inflammatory bowel disease, although renal dysfunction showed progression. Amyloidosis should be considered in patients with IBD, particularly if they suffered disease progression.

Effects of Banxia Xiexin Decoction () on Cisplatin-Induced Apoptosis of Human A549 Lung Cancer Cells / 中国结合医学杂志

<p><b>OBJECTIVE</b>To examinie the synergistic effects of Banxia Xiexin Decoction (, Known as Banhasasim-tang in Korean) extract (BXDE) on cisplatin-induced cytotoxicity in the A549 human lung cancer cell lines.</p><p><b>METHODS</b>A549 cells were treated with varying concentrations (50-200 μg/mL) of cisplatin and BXDE alone or in combination for 96 h. We used 1-(4,5-dimethylthiazol-2-yl)-3,5-diphenylformazan assay and flow cytometry to analyze cell viability and apoptosis, respectively.</p><p><b>RESULTS</b>The exposure of cells to cisplatin and BXDE alone or in combination decreased cell viability dose- and time-dependently (P<0.05), which was found to be mediated by the apoptotic pathway as confirmed by the increase in the annexin V/propidium iodide- stained cell population and a ladder pattern of discontinuous DNA fragments. Furthermore, the apoptosis was inhibited by the pan-caspase inhibitor, benzyloxycarbonyl-Val-Ala-Asp (OMe) fluoromethylketone (z-VAD-FMK).</p><p><b>CONCLUSIONS</b>BXDE significantly potentiated apoptotic effects of cisplatin in A549 cells. Moreover, apoptosis induced by BXDE might be the pivotal mechanism mediating its chemopreventative action against cancer.</p>

Perivascular Epithelioid Cell Tumor in the Stomach

Perivascular epithelioid cell tumors or PEComas can arise in any location in the body. However, a limited number of cases of gastric PEComa have been reported. We present two cases of gastric PEComas. The first case involved a 62-year-old woman who presented with a 4.2 cm gastric subepithelial mass in the prepyloric antrum, and the second case involved a 67-year-old man with a 5.0 cm mass slightly below the gastroesophageal junction. Microscopic examination revealed that both tumors were composed of perivascular epithelioid cells that were immunoreactive for melanocytic and smooth muscle markers. Prior to surgery, the clinical impression of both tumors was gastrointestinal stromal tumor (GIST), and the second case was erroneously diagnosed as GIST even after microscopic examination. Although gastric PEComa is a very rare neoplasm, it should be considered in the differential diagnosis of gastric submucosal lesions.

Xp11.2 translocation renal cell carcinoma in the autosomal dominant polycystic kidney disease patient with preserved renal function

No abstract available.

Intraosseous Hibernoma: A Rare and Unique Intraosseous Lesion

BACKGROUND: Hibernoma is a rare benign tumor of adults that is composed of multivacuolated adipocytes resembling brown fat cells. Hibernoma typically occurs in soft tissue, and intraosseous examples are very rare. Intraosseous hibernomas can radiologically mimic metastatic carcinoma and other tumorous conditions. METHODS: To collect the intraosseous hibernomas, we searched the pathologic database and reviewed the hematoxylin and eosin (H&E)–stained slides of bone biopsy samples performed to differentiate radiologically abnormal bone lesions from 2006 to 2016. A total of six intraosseous hibernoma cases were collected, and clinical and radiological information was verified from electronic medical records. H&E slide review and immunohistochemical staining for CD68, pan-cytokeratin, and S-100 protein were performed. RESULTS: Magnetic resonance imaging of intraosseous hibernomas showed low signal intensity with slightly hyperintense foci on T1 and intermediate to high signal intensity on T2 weighted images. Intraosseous hibernomas appeared as heterogeneous sclerotic lesions with trabecular thickening on computed tomography scans and revealed mild hypermetabolism on positron emission tomography scans. Histopathologically, the bone marrow space was replaced by sheets of multivacuolated, foamy adipocytes resembling brown fat cells, without destruction of bone trabeculae. In immunohistochemical analysis, the tumor cells were negative for CD68 and pan-cytokeratin and positive for S-100 protein. CONCLUSIONS: Intraosseous hibernoma is very rare. This tumor can be overlooked due to its rarity and resemblance to bone marrow fat. Pathologists need to be aware of this entity to avoid misdiagnosis of this rare lesion.