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Background: Capicua transcriptional repressor-double homeobox 4 sarcoma (CDS) is a rare and aggressive malignant soft tissue tumor that typically arises within the soft tissues. We report an exceptionally rare case of a gastric CDS successfully resected despite its extensive invasion into surrounding organs. Case presentation: A 48-year-old male presented with a progressively enlarging abdominal mass. Upper gastrointestinal endoscopy revealed a large ulcerative tumor on the posterior gastric wall. Biopsy results initially suggested a neuroendocrine cell carcinoma. Contrast-enhanced computed tomography showed a 20 cm tumor protruding from the posterior stomach wall, directly invading the pancreas and colon. We performed a multivisceral resection (stomach, pancreatic tail, spleen, and transverse colon) achieving an R0 resection. Pathological examination of the permanent specimen revealed small round cells with high nuclear-to-cytoplasmic ratios. Immunohistochemical staining confirmed the diagnosis of CDS. The patient recovered well and was discharged on postoperative day 33. Conclusions: This case report describes the first detailed account of a surgically resected aggressive CDS originating from the stomach. [ABSTRACT FROM AUTHOR]

Refractory gastroesophageal reflux disease can develop after proximal gastrectomy and esophagogastrostomy. We introduce a new method that combines distal gastrectomy and Roux-en-Y reconstruction to treat refractory reflux esophagitis in patients who have undergone proximal gastrectomy and esophagogastric anastomosis reconstruction. This novel method may be useful not only for alleviating the symptoms of gastroesophageal reflux disease but also for preventing future esophageal malignancies arising from long-term reflux esophagitis. [ABSTRACT FROM AUTHOR]

Background: Approximately 8300 hemophiliacs are registered in Japan, but no comprehensive reports on hepatobiliary and pancreatic surgery (HBPS) have been conducted. This report investigates the current status of HPBS in hemophilia patients in Japan. Methods: The subjects were hemophiliac patients seen between January 1 2007, and December 31 2017, at facilities participating in this study among the facilities for performing high‐difficulty cases nationwide designated by the Japanese Society for HBPS. A retrospective examination of short‐term outcomes in 49 cases was conducted to assess patient background, disease, surgical procedure, and complications. Results: The types of hemophilia were A: 43 cases, B: four cases, and von Willebrand disease: two cases (hemophilia severity: mild 32, moderate seven, severe 10). The target malignant diseases for surgery were hepatocellular carcinoma (HCC) in 20 cases, intrahepatic cholangiocellular carcinoma (CCC) in four cases, combined HCC‐CCC in two cases, hilar CCC in two cases, and pancreatic cancer in four cases. As for the surgical procedure, limited resection (subsegmentectomy and partial hepatectomy) was performed in 16 cases of HCC even with normal liver function tests. Pancreaticoduodenectomy and distal pacreatectomy were performed for pancreatic cancers as in the standard procedure. Postoperative complications were postoperative bleeding in two cases after hepatectomy and one after pancreatectomy in one case. When compared with Japanese National Clinical Data base, the complication rates after hepatectomy and pancreatectomy were not conspicuous in hemophilic patients. Conclusions: As long as they are performed in qualified centers, complication rate is not increased in hemophilic patients undergoing HBPS. [ABSTRACT FROM AUTHOR]

Ovarian cancer is the second-most lethal gynecological malignancy and the seventh-commonest cause of cancer-related death in women around the world. Most of the ovarian cancer patients are diagnosed at advanced stages and suffer from recurrence after primary cytoreductive surgery and standard first-line chemotherapy. Thus, the successful management of ovarian cancer patients requires the identification of factors that contribute to progression and relapse. Interleukin-34 (IL-34) is a novel cytokine that acts as a tissue-specific ligand of colony-stimulating factor-1 receptor (CSF-1R). In cancer, IL-34 exerts pro-tumorigenic functions that promote tumor growth, metastasis, angiogenesis, immune suppression and therapeutic resistance. In this study, we evaluate the impact of IL-34 on progression and survival of ovarian cancer patients. First, IL-34 was found to be expressed in several human ovarian cancer cell lines and cancer tissues from patients. The expression of IL-34 was enhanced by cytotoxic chemotherapy in ovarian cancer cell lines and cancer tissues from chemotherapy-treated ovarian cancer patients. Importantly, high IL-34 expression correlated with worse progression-free survival (PFS) and overall survival in different cohorts. The assessment of PFS based on a combination between IL34 expression and other related genes such as CSF1R and CD163 helped further to reach more statistical significance compared with IL34 alone. Furthermore, in the murine ovarian cancer cell HM-1 in vivo model, it was suggested that IL-34-derived tumor cells was correlated with tumor progression and survival by modulating the immune environment. Collectively, these findings indicate a possible correlation between IL-34 expression and disease progression in ovarian cancer patients and the mouse model. [ABSTRACT FROM AUTHOR]

The mortality of colorectal cancer is expected to increase in some countries including the United States, which necessitates the identification of new molecules that help in prognosis assessment and survival improvement. In this brief report, we evaluated the potential of interleukin-34 (IL-34) as a prognostic factor in colorectal cancer. IL-34 was reported for the first time in 2008 as a novel cytokine that controls the biology of the myeloid cell lineage. Accumulating evidence suggests important roles for IL-34 in modifying the tumor microenvironment and enhancing therapeutic resistance of cancer. In this study, we found that IL-34 expression was detectable in various colorectal cancer cell lines in addition to primary cancer tissues from a cohort of Japanese colorectal cancer patients, ranging from high to absent. A Kaplan–Meier analysis showed that high expression of IL-34 correlated with poor survival of colorectal cancer patients. Importantly, in both univariate and multivariate analysis, high IL-34 expression correlated with unfavorable prognosis. A similar relationship between IL-34 expression and the poorer prognosis was also observed in a cohort of colorectal cancer patients registered at The Cancer Genome Atlas. Together, these findings indicate a potential role for IL-34 as a prognostic factor in colorectal cancer. [ABSTRACT FROM AUTHOR]

Congenital absence of the portal vein (CAPV) is a rare malformation in which intestinal and splenic venous blood bypasses the liver and drains into systemic veins. CAPV is classified into two types based on the absence (type I) or presence (type II) of portal venous flow into the hepatic parenchyma and is associated with multiple other anomalies such as usually benign hepatic tumors. There have been only two case reports describing hepatocellular carcinoma (HCC) in patients with CAPV type II to date. We report the third such patient. A 50‐year‐old woman was referred to our hospital for management of a giant hepatic tumor. Contrast‐enhanced computed tomography (CECT) indicated a huge mass occupying the right lobe of the liver; the radiological diagnosis was HCC. CECT also demonstrated that the superior mesenteric vein (SMV) and the splenic vein (SpV) joined to form a shunt draining into the left renal vein and that a hypoplastic portal vein branched from the confluence of the SMV and SpV and drained into the liver, indicating that the CAPV was type II. Liver resection was successfully performed to treat the HCC, and the pathological diagnosis was well‐differentiated HCC. Seven months after the operation, a recurrent small HCC was detected and treated with radiofrequency ablation without complications. The patient has been carefully followed for 6 months to date without any evidence of further recurrence. Patients with CAPV are predisposed to developing HCC and require close surveillance. [ABSTRACT FROM AUTHOR]

IL‐34 is a novel cytokine that was identified in 2008 in a comprehensive proteomic analysis as a tissue‐specific ligand of CSF‐1 receptor (CSF‐1R). IL‐34 exists in all vertebrates including fish, amphibians, birds, and mammals, showing high conservation among species. Structurally, IL‐34 belongs to the short‐chain helical hematopoietic cytokine family but shows no apparent consensus structural domains, motifs, or sequence homology with other cytokines. IL‐34 is synthesized as a secreted homodimeric glycoprotein that binds to the extracellular domains of CSF‐1R and receptor‐type protein‐tyrosine phosphatase‐zeta (PTP‐ζ) in addition to the chondroitin sulfate chains of syndecan‐1. These interactions result in activating several signaling pathways that regulate major cellular functions, including proliferation, differentiation, survival, metabolism, and cytokine/chemokine expression in addition to cellular adhesion and migration. In the steady state, IL‐34 contributes to the development and maintenance of specific myeloid cell subsets in a tissue‐specific manner: Langerhans cells in the skin and microglia in the brain. In pathological conditions, changes in IL‐34 expression—increased or decreased—are involved in disease pathogenesis and correlate with progression, severity, and chronicity. One decade after its discovery, IL‐34 has been introduced as a newcomer to the big family of interleukins with specific physiological functions, critical pathological roles, and promising clinical applications in disease diagnosis and treatment. In this review, we celebrate the 10th anniversary of IL‐34 discovery, introducing its biological characteristics, and discussing the importance of IL‐34 signaling network in health and disease. IL‐34, a newcomer to the big family of interleukins with specific physiological functions and critical pathological roles. [ABSTRACT FROM AUTHOR]

Metabolism may determine the biologically malignant behavior of pancreatic cancer. To investigate the significance and prognostic value of cancer metabolism in cancer patients, we investigated the expression of two key enzymes in anaerobic glycolysis, hexokinase 2 (HK2) and pyruvate kinase isoenzyme type M2 (PKM2), in surgical specimens obtained from 36 patients who underwent curative resection of pancreatic ductal carcinoma. The HK2-glycolysis axis is a key system in the clinical imaging of tumors via positron emission tomography. Immunohistochemical staining for HK2 and PKM2 was performed and the data were statistically analyzed to evaluate their prognostic power. The expression of HK2 and PKM2 was associated with clinicopathological variables and patient prognosis, including overall survival, local recurrence-free survival and distant metastasis-free survival. Staining for HK2 was negative and positive in 42 and 58% of the patients, respectively, whereas staining for PKM2 was negative and positive in 56 and 44%, respectively; HK2-positive staining was correlated with progressive pathological tumor stage (pT3 vs. pT1 and pT2; P=0.017). In the univariate analysis, the positive expression of HK2 and PKM2, pathological stage (pT3 vs. pT1 and pT2) and nodal metastasis were significantly correlated with poor prognosis (P<0.03). In the multivariate analysis, pathological nodal metastasis was an independent prognostic factor for overall survival, whereas the positive expression of HK2 and PKM2 exhibited borderline significance (P=0.08 and 0.12, hazard ratio = 2.57 and 2.16, respectively). In addition, the combination of high expression of HK2 as well as PKM2 was found to be significant (P<0.05). These results suggested that the expression of HK2 and PKM2, particularly their combination, in surgical specimens obtained during curative resection, may predict an unfavorable clinical outcome in patients with pancreatic cancer. [ABSTRACT FROM AUTHOR]

Pancreaticoduodenectomy is a radical treatment for pancreatic head cancer. However, it is sometimes difficult to perform pancreaticoduodenectomy, particularly in patients who have previously undergone esophagectomy with gastric conduit reconstruction. We herein describe a surgical technique for radical pancreaticoduodenectomy with microvascular reconstruction that preserves the gastric conduit. A 72-year-old male with a previous history of esophagectomy and gastric conduit reconstruction for esophageal cancer was referred to our hospital for surgical treatment of advanced pancreatic head cancer. After considering both the cancer curability and preservation of the gastric conduit, we performed a standard pancreaticoduodenectomy, and added a microvascular anastomosis of the gastroduodenal artery to the right gastroepiploic artery. In addition, we also performed reconstruction of the right gastroepiploic vein. This radical pancreaticoduodenectomy with microvascular reconstruction was safely and successfully performed, and it preserved the gastric conduit. The 6-month follow-up showed recurrence-free survival and a good quality of life. [ABSTRACT FROM AUTHOR]

Biliary tract cancer ( BTC) has a generally poor prognosis. Furthermore, it is difficult to distinguish BTC from benign biliary disease ( BBD) with commonly used modalities. Therefore, a novel biomarker to facilitate cancer detection is highly desirable. Recent studies have reported the use of circulating micro RNAs (mi RNAs) as biomarkers for cancers. The purpose of this study was to evaluate whether circulating mi RNA-21 (mi R-21) could be used as a biomarker for BTC. Plasma samples were obtained from 94 BTC patients, 50 healthy volunteers ( HVs), and 23 BBD patients. mi R-21 levels in the samples were measured by q RT- PCR. Plasma mi R-21 levels in patients with BTC were significantly higher than in HVs or in patients with BBD ( P < 0.0001 for both). Receiver-operator curve ( ROC) curve analysis in differentiating BTC patients from HVs indicated that area under the curve ( AUC), optimal sensitivity and specificity was 0.93, 85.1% and 100%, respectively, and those in differentiating BTC patients from BBD patients was 0.83, 72.3%, 91.3%, respectively. Validation of these results indicated that the negative predictive value, positive predictive value, sensitivity, specificity, and accuracy in differentiating BTC patients from HVs was 76.6%, 98.6%, 84.0%, 98.0%, and 88.9%, respectively, and those in differentiating BTC patients from BBD patients was 42.2%, 93.0%, 71.2%, 82.6%, and 72.6%, respectively. These sets of values were improved by combining mi R-21 and CA19-9 measurements. Plasma mi R-21 is a novel diagnostic biomarker for BTC, and may be useful in distinguishing between BTC and BBD patients. [ABSTRACT FROM AUTHOR]