Tubulocystic Renal Cell Carcinoma Is Not an Indolent Tumor: A Case Report of Recurrences in the Retroperitoneum and Contralateral Kidney.

Tubulocystic renal cell carcinoma (RCC) is a rare subtype of RCC that was recently included in the 2016 World Health Organization classification of tumors of the kidney. Most of these tumors exhibit indolent behavior with low metastatic potential. However, here we report a case of recurrent tubulocystic RCC with aggressive features in the retroperitoneum and contralateral kidney treated with targeted agents and radiofrequency ablation.

Clinicopathologic analysis of intraductal papillary neoplasm of bile duct: Korean multicenter cohort study.

BACKGROUND: IPNB is very rare disease and most previous studies on IPNB were case series with a small number due to low incidence. The aim of this study is to validate previously known clinicopathologic features of intraductal papillary neoplasm of bile duct (IPNB) based on the first largest multicenter cohort. METHODS: Among 587 patients previously diagnosed with IPNB and similar diseases from each center in Korea, 387 were included in this study after central pathologic review. We also reviewed all preoperative image data. RESULTS: Of 387 patients, 176 (45.5%) had invasive carcinoma and 21 (6.0%) lymph node metastasis. The 5-year overall survival was 80.9% for all patients, 88.8% for IPNB with mucosal dysplasia, and 70.5% for IPNB with invasive carcinoma. According to the "Jang & Kim's modified anatomical classification," 265 (68.5%) were intrahepatic, 103 (26.6%) extrahepatic, and 16 (4.1%) diffuse type. Multivariate analysis revealed that tumor invasiveness was a unique predictor for survival analysis. (p = 0.047 [hazard ratio = 2.116, 95% confidence interval 1.010-4.433]). CONCLUSIONS: This is the first Korean multicenter study on IPNB through central pathologic and radiologic review process. Although IPNB showed good long-term prognosis, relatively aggressive features were also found in invasive carcinoma and extrahepatic/diffuse type.

Dermatofibroma: sonographic findings and pathologic correlation.

Background Ultrasound has been increasingly used for the evaluation of superficial soft tissue lesions. Dermatofibroma is one of the most common dermal lesions. Experiences in ultrasound examinations of dermatofibromas have been accumulated. Purpose To evaluate sonographic findings of dermatofibroma and to correlate them with pathologic findings. Material and Methods A total of 18 dermatofibromas in 15 patients were included. Sonographic findings were assessed for location, depth, size, shape, margin, spiculation, echogenicity, internal hyperechoic dots, change in echogenicity of adjacent soft tissue, and vascularity, by two musculoskeletal radiologists in consensus. Pathologic findings were evaluated by one pathologist. Ultrasonographic characteristics were correlated with the pathologic findings by discussion between one radiologist and one pathologist. Results On ultrasound, all lesions were found to be located in the dermis, and 14 lesions (78%) extended into the subcutaneous layer. Mean size was 7 mm (range = 3 to 10 mm). Characteristic findings were ill-defined margins (n = 15, 83%), spiculated margins (n = 12, 67%), change in echogenicity of adjacent soft tissue (n = 14, 78%), and no Doppler flow (n = 14, 78%). Histologically, depth, margin, and spiculation were correlated with sonographic findings. Reactive fibrosis and chronic inflammation were seen in adjacent dermis and subcutaneous fat layer of echogenicity change. Conclusion On ultrasound, dermatofibromas are usually visible as avascular dermal lesions with extension into the subcutaneous layer, and are characterized by ill-defined margins, marginal spiculation, and change in echogenicity of surrounding soft tissues.

MR imaging and ultrasonography findings of early myositis ossificans: a case report.

Myositis ossificans (MO) is a benign soft tissue lesion with non-neoplastic heterotopic bone formation. MO in the intermediate and mature stages can be easily diagnosed if characteristic imaging findings such as a peripheral zonal pattern of ossification with variable thickness is observed. However, it is difficult to correctly diagnose early MO because it can mimic malignancy clinically, radiologically, and histopathologically. We report a case of early pseudosarcomatous phase of non-traumatic MO with atypical imaging findings. A 59-year-old woman presented with pain followed by a mass in the left thigh within a week. MR imaging and ultrasonography showed an intramuscular lesion with preserved muscle fascicles in the vastus lateralis muscle. Intralesional ossification or calcification was not seen on ultrasonography. A diagnosis of myositis ossificans was made by ultrasonographically guided biopsy.

Chronic Intussusception Caused by Diffuse Large B-Cell Lymphoma in a 6-Year-Old Girl Presenting with Abdominal Pain and Constipation for 2 Months.

The classical triad of abdominal pain, vomiting, and bloody stool is absent in chronic intussusception for more than 2 weeks. Here, we report a 6-year-old female with recurrent abdominal pain for 2 months. Ultrasonography of the abdomen revealed an ileocolic-type intussusception. The lesion accompanying the tight fibrous adhesion was treated by resection and ileocolic anastomosis. It was diagnosed as intussusception with diffuse large B-cell lymphoma. A high index of suspicion for abdominal pain in children should result in the correct diagnosis and appropriate management.

Hyperuricemia-induced NLRP3 activation of macrophages contributes to the progression of diabetic nephropathy.

IL-1ß-secreting nucleotide-binding oligomerization domain protein 3 (NLRP3) inflammasomes play a pivotal role in triggering innate immune responses in metabolic disease. We investigated the role of soluble uric acid in NLRP3 inflammasome activation in macrophages to demonstrate the effect of systemic hyperuricemia on progressive kidney damage in type 2 diabetes. THP-1 cells, human acute monocytic leukemia cells, were cultured to obtain macrophages, and HK-2 cells, human renal proximal tubule cells, were cultured and stimulated with uric acid. In vivo, we designed four rat groups as follows: 1) Long-Evans Tokushima Otsuka (LETO); 2) Otsuka Long-Evans Tokushima Fatty (OLETF); 3) OLETF+high-fructose diet (HFD) for 16 wk; and 4) OLETF+HFD+allopurinol (10 mg/dl administered in the drinking water). Soluble uric acid stimulated NLRP3 inflammasomes to produce IL-1ß in macrophages. Uric acid-induced MitoSOX mediates NLRP3 activation and IL-1ß secretion. IL-1ß from macrophages activates NF-κB in cocultured proximal tubular cells. In vivo, intrarenal IL-1ß expression and macrophage infiltration increased in HFD-fed OLETF rats. Lowering the serum uric acid level resulted in improving the albuminuria, tubular injury, macrophage infiltration, and renal IL-1ß (60% of HFD-fed OLETF) independently of glycemic control. Direct activation of proximal tubular cells by uric acid resulted in (C-X-C motif) ligand 12 and high mobility group box-1 release and accelerated macrophage recruitment and the M1 phenotype. Taken together, these data support direct roles of hyperuricemia in activating NLRP3 inflammasomes in macrophages, promoting chemokine signaling in the proximal tubule and contributing to the progression of diabetic nephropathy through cross talk between macrophages and proximal tubular cells.

Cystic Duct Enhancement: A Useful CT Finding in the Diagnosis of Acute Cholecystitis Without Visible Impacted Gallstones.

OBJECTIVE: The purpose of this study was to determine the incremental value of the presence of cystic duct enhancement for diagnosing acute cholecystitis without visible impacted gallstones. MATERIALS AND METHODS: CT scans of 63 patients with acute cholecystitis and 63 control subjects were retrospectively and independently reviewed by two radiologists to determine the presence of cystic duct enhancement or impacted stones. Two additional radiologists were then asked to independently evaluate all CT images using a 5-point scoring system for diagnosing acute cholecystitis. They conducted the evaluations both before and after being informed that cystic duct enhancement could be substituted for a CT finding of impacted gallstones. RESULTS: The prevalence of either cystic duct enhancement or stone impaction was observed to be significantly more common in the patient group (86-91%) than in the control group (6-14%) (p < 0.001) with good interobserver agreement (κ = 0.79). Diagnostic sensitivities increased significantly from 60.3% to 85.7% for reviewer 1 (p = 0.001) and from 71.4% to 87.3% for reviewer 2 (p = 0.028) after the reviewers were informed of the presence of cystic duct enhancement. Diagnostic accuracy increased significantly for the less experienced radiologist, from 75.4% to 87.3% (p = 0.015). CONCLUSION: The accuracy and sensitivity of CT for the diagnosis of acute cholecystitis improved significantly when cystic duct enhancement was used as an alternative to impacted gallstones as a diagnostic criterion.

A case of intussusception of the appendix secondary to endometriosis: US and CT findings.

Intussusception of the appendix is an uncommon condition that is difficult to diagnose with radiology. Endometriosis causing appendiceal intussusception is a rare condition that has only been reported a few times in the literature. Here, we report a case of appendiceal intussusception caused by endometriosis in a 33-year-old woman who presented with intermittent right lower abdominal pain. Sonography revealed a hypoechoic mass invaginating into the cecum, which was covered by echogenic cecal wall with central dimpling indicating the appendiceal orifice. On CT, the mass was identified as an enhancing mass invaginating into the cecum at the level the appendiceal orifice.

Effect of pronase premedication on narrow-band imaging endoscopy in patients with precancerous conditions of stomach.

BACKGROUND: Narrow-band imaging (NBI) endoscopy improves the detection of intestinal metaplasia. However, strategies to improve the visibility and diagnostic performance of NBI should be sought, as endoscopic views are often obscured by the presence of mucus. AIM: To compare the visibility and diagnostic performance of NBI endoscopy according to pronase premedication in patients with precancerous conditions of the stomach. METHODS: Consecutive outpatients with a previous diagnosis of precancerous condition of the stomach were invited to undergo a surveillance NBI endoscopy between June and December 2012. Enrolled subjects were randomly assigned to pronase or control groups before NBI endoscopy. The visibility score and diagnostic performance of NBI endoscopy were compared between the two groups. RESULTS: Patients' endoscopic and histopathological characteristics were similar between the two groups. Visibility score in the proximal part of the stomach and satisfaction score of the endoscopist were significantly higher in the pronase group than in the control group (p = 0.014 and p = 0.034, respectively). The diagnostic performance of NBI endoscopy to detect intestinal metaplasia was not different in either group (both p > 0.1). However, the negative predictive value of NBI endoscopy was much improved over that of white light endoscopy only in the pronase group (p = 0.013). CONCLUSION: Pronase premedication increased the visibility of the proximal part of the stomach and the satisfaction score during NBI endoscopy. Furthermore, negative predictive value of NBI endoscopy was much improved compared with that of white light endoscopy after pronase premedication.

Imaging and Clinical Findings of Xanthogranulomatous Inflammatory Disease of Various Abdominal and Pelvic Organs: A Pictorial Essay.

Xanthogranulomatous (XG) inflammatory disease is a rare benign disease involving various organs, including the gallbladder, bile duct, pancreas, spleen, stomach, small bowel, colon, appendix, kidney, adrenal gland, urachus, urinary bladder, retroperitoneum, and female genital organs. The imaging features of XG inflammatory disease are nonspecific, usually presenting as a heterogeneous solid or cystic mass. The disease may also extend to adjacent structures. Due to its aggressive nature, it is occasionally misdiagnosed as a malignant neoplasm. Herein, we review the radiological features and clinical manifestations of XG inflammatory diseases in various organs of the abdomen and pelvis.

Synovial sarcoma of primary bone origin arising from the cervical spine.

Synovial sarcoma of primary bone origin is very rare and has not been previously reported in the cervical vertebra. We report the first case of a synovial sarcoma of the cervical vertebra, presenting as an expansile osteolytic lesion in the posterior osseous elements of the C3 vertebra characteristically with prominent intratumoral vessels.

Myxoid Liposarcoma of the Breast Mimicking Phyllodes Tumor: A Case Report.

Myxoid liposarcoma is an extremely rare malignant breast tumor. We report the case of a 44-year-old woman who had myxoid liposarcoma of the breast with a history of phyllodes tumor and describe the imaging findings on US, mammography, and MRI. Before surgery, the mass was considered to be a recurrent phyllodes tumor. However, using US, we retrospectively identified some differences between myxoid liposarcomas and phyllodes tumors.

Mesonephric-like Adenocarcinoma of the Uterine Corpus: Comprehensive Analyses of Clinicopathological, Molecular, and Prognostic Characteristics With Retrospective Review of 237 Endometrial Carcinoma Cases.

BACKGROUND/AIM: Uterine mesonephric-like adenocarcinoma (MLA) is a rare malignant tumor of the female genital tract. PATIENTS AND METHODS: We reviewed 237 endometrial carcinoma cases and investigated the clinicopathological and molecular characteristics of uterine MLA. RESULTS: We found that 3.0% (7/237) of the endometrial carcinoma cases were MLAs. Compared to endometrial endometrioid carcinoma, MLA showed larger tumor size, deeper myometrial invasion, increasingly advanced-stage disease, and more frequent lymphovascular space invasion. All MLAs exhibited architectural diversity, compactly aggregated small tubules, eosinophilic intraluminal secretions, overlapped and angulated nuclei, scant cytoplasm, and presence of spindle cells. All the MLAs expressed at least two mesonephric markers. All except one MLA harbored activating Kirsten rat sarcoma viral oncogene homolog mutations. All patients with MLA developed postoperative metastases. MLA had the lowest progression-free survival rate among different histological types of endometrial carcinoma. CONCLUSION: Uterine MLA is a highly aggressive gynecological malignancy, showing unique morphological and molecular features, frequent recurrences and metastases, as well as poor prognosis.

Bicameral Gallbladder With Chronic Cholecystitis on Hepatobiliary Scintigraphy.

Bicameral gallbladder, also known as segmental adenomyomatosis, is not a rare benign condition with the lumen divided into 2 interconnected chambers. Here we present 2 interesting cases of the bicameral gallbladder, which shows unremarkable findings on hepatobiliary scintigraphy at first appearance. However, the CT scan revealed that the fundal chamber was not visualized on the scintigraphy unlike the ductal chamber. These cases suggest that the findings of the bicameral gallbladder on hepatobiliary scintigraphy can lead to misdiagnosis without carefully correlating with anatomic imaging findings.

The antioncogenic effect of Beclin-1 and FOXP3 is associated with SKP2 expression in gastric adenocarcinoma.

ABSTRACT: An overexpression of S-phase kinase-associated protein 2 (SKP2) is frequently observed in human cancer progression and metastasis, and evidence suggests that SKP2 plays a proto-oncogenic role both in vitro and in vivo. However, the function of SKP2 in gastric adenocarcinoma remains largely obscure. We investigated SKP2 expression in human gastric carcinomas.Tissue samples were acquired from 182 cases of gastric adenocarcinoma that were surgically resected from 2006 to 2012. Immunohistochemical staining for SKP2, Beclin-1, and forkhead box protein P3 (FOXP3) was performed. Pearson chi-square test was used to evaluate the associations among clinicopathological variables. The Kaplan-Meier method, the log-rank test, and the Cox proportional-hazards model were used in the analysis of the overall survival (OS) and disease-free survival (DFS).As a result, SKP2 overexpression in gastric adenocarcinomas showed a significant correlation with several favorable clinical factors, including the tumor size, T category, N category, lymphatic invasion, vascular invasion, OS, and DFS. SKP2 expression was positively correlated with the tumoral FOXP3, Beclin-1 expression, and regulatory T cell (Treg) infiltration. The difference in DFS between the SKP2 positive and negative group was attenuated by FOXP3 high expression, Beclin-1 high expression, and Tregs infiltration. Attenuation of the difference in OS by FOXP3 high expression, Beclin-1 high expression, and Tregs infiltration was not significant. In multivariable analysis, SKP2 expression was not correlated with OS and DFS.Our study showed a complex interrelationship between SKP2 and Beclin-1 and FOXP3 expression in gastric adenocarcinoma. The antioncogenic effect of Beclin-1 and FOXP3 expression in gastric adenocarcinoma is related to SKP2 expression.

Solitary, Endobronchial Metastasis from Renal Cell Carcinoma 20 Years after Nephrectomy.

Late recurrence over 10 years after surgery and endobronchial metastasis are some of the specific biological behaviors of renal cell carcinoma (RCC). The current report describes a case of solitary endobronchial metastasis at a subsegmental bronchus that developed 20 years after curative nephrectomy for RCC. A 71-year-old male was admitted to our hospital for pneumonia. Chest radiography showed multifocal ill-defined nodular opacities in the right lower lung zone, suggesting pneumonia. Subsequent chest CT confirmed pneumonic infiltration in the right lung. However, a 4.3-cm, well-defined, elongated mass with a branching pattern was also identified in the right lower lobe, and a right nephrectomy scar was detected on the covered upper abdomen. The patient had undergone right nephrectomy 20 years ago due to clear cell RCC. After right lower lobectomy, the postoperative pathological diagnosis was endobronchial metastatic clear cell RCC. Endobronchial metastasis should be considered in a patient with a history of RCC who presents with a suspected endobronchial tumor, even decades after curative surgery.

Clinical Significance of Receptor-Interacting Protein 3 and Parkin, Essential Molecules for Necroptosis, in Breast Cancer.

PURPOSE: Receptor-interacting protein 3 (RIP3) is the main initiator of necroptosis. Parkin prevents the formation of the RIP1-RIP3 complex by promoting polyubiquitination of RIP3. However, the mechanism by which necroptosis affects the clinical features of breast cancer and prognosis is not known. Here, we aimed to study the effect of necroptosis on the clinical features and prognosis of breast cancer by assessing the expression of RIP3 and Parkin. METHODS: Tissue microarrays (TMAs) were constructed from 257 cases of breast cancer. Immunohistochemistry was performed on 4-µm tissue sections from each TMA block. The χ² test, Kaplan-Meier survival analysis with log-rank test, and Cox regression proportional hazard model were used for statistical analysis. RESULTS: Low RIP3 expression resulted in a large tumor size and high nuclear grade. Low RIP3 expression was correlated with human epidermal growth factor receptor 2 positivity, short overall survival (OS), and short disease-free survival (DFS). The triple negative breast cancer group with low RIP3 expression and lymph node (LN) positive group with low RIP3 expression had the shortest OS. High Parkin expression was associated with high histological grade, estrogen and/or progesterone receptor negativity, and lymphatic emboli, but was not correlated with OS and DFS. OS was correlated with LN metastasis and RIP3 loss and DFS with large tumor size, LN metastasis, and RIP3 loss. CONCLUSION: Low RIP3 and high Parkin expression are associated with aggressive clinical features in breast cancer. RIP3, a molecular marker of necroptosis, is an independent factor associated with survival in breast cancer. Further in-depth studies are needed to investigate the role of necroptosis in breast cancer development, metastasis, and treatment in the future.

ERG and nestin: useful markers of immature vessels and novel prognostic markers in renal cell carcinoma.

OBJECTIVES: Renal cell carcinoma (RCC) accounts for approximately 90% of all renal malignancy. Because a rich vasculature is an outstanding feature of RCC, information on the blood vessels of RCC might explain its tumor characteristics. Several researchers have noted the effects of tumor vessels on the clinicopathologic characteristics and prognosis of tumors; however, a clear association has not been established. We hypothesized that the immaturity of the neovasculature may be an important clinicopathologic characteristic forprognosis of RCC patients. ERG and nestin are new vascular markers that regulate vascular homeostasis and angiogenesis. Therefore, in the present study, we investigated how ERG and nestin were expressed with respect to tumor characteristics. MATERIALS AND METHODS: IHC staining for ERG, nestin, CD31, and CD34 was performed for 217 renal tumors, including clear-cell RCC (ccRCC; n = 184), papillary RCC (pRCC; n = 14), chromophobe RCC (chRCC; n = 14), and oncocytoma (n = 5). RESULTS: Vascular endothelial cells from normal kidney consistently showed strong nuclear expression of ERG and nestin. Conversely, a loss of ERG and nestin expression was observed in endothelial cells of some tumor blood vessels, which was associated with tumor progression. In particular, the loss of ERG expression was significantly associated with progression-free survival and overall survival (univariate analyses: P = 0.027 and P = 0.004, respectively; multivariate analyses: P = 0.030 and P = 0.046, respectively). CONCLUSION: A loss of ERG and nestin expression is associated with tumor progression, and loss of ERG is a powerful prognostic marker for ccRCC.

Adenosine A2A Receptor Agonist Polydeoxyribonucleotide Alleviates Interstitial Cystitis-Induced Voiding Dysfunction by Suppressing Inflammation and Apoptosis in Rats.

BACKGROUND: Interstitial cystitis (IC) is a chronic disorder that indicates bladder-related pain or discomfort. Patients with IC often experience urination problems, such as urinary frequency and urgency, along with pain or discomfort in the bladder area. Therefore, new treatments based on IC etiology are needed. Polydeoxyribonucleotide (PDRN) is a biologic agonist of the adenosine A2A receptor, and PDRN has anti-inflammatory effect and inhibits apoptosis. In the current study, the effect of PDRN on cyclophosphamide-induced IC animal model was investigated using rats. METHODOLOGY: To induce the IC animal model, 75 mg/kg of cyclophosphamide was injected intraperitoneally once every 3 days for 10 days. The rats in the PDRN-treated groups were intraperitoneally injected with 0.5 mL physiological saline containing 8 mg/kg PDRN, once a day for 10 days after IC induction. RESULTS: Induction of IC by cyclophosphamide injection caused voiding dysfunction, bladder edema, and histological damage. Cyclophosphamide injection increased secretion of pro-inflammatory cytokines and enhanced apoptosis. In contrast, PDRN treatment alleviated voiding dysfunction, bladder edema, and histological damage. Secretion of pro-inflammatory cytokines and expressions of apoptotic factors were suppressed by PDRN treatment. These changes indicate that treatment with PDRN improves voiding function by ultimately promoting the repair of damaged bladder tissue. CONCLUSION: The conclusion of this experiment suggests the possibility that PDRN could be used as an effective therapeutic agent for IC.

Clinical and histologic response to methylprednisolone pulse therapy in glomerulonephritis.

We retrospectively analyzed the data of the first and second renal biopsies to investigate the adverse effects as well as the clinical and histologic responses of methylprednisolone pulse therapy in patients with chronic glomerulonephritis. At the time of the second renal biopsy, the activity index had decreased significantly and the chronicity index was well preserved. The activity index and interstitial fibrosis were improved in the complete and partial remission groups, but not in the nonresponse group. These findings indicate that methylprednisolone pulse therapy is effective in patients with chronic glomerulonephritis and has an acceptably low risk of side effects.