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Sarcoidosis-lymphoma syndrome associated with portal hypertension is very rare. A 68-year-old female presented with a 5 kg weight loss in 6 months. Soluble interleukin-2 receptor activity was increased and total platelet count was decreased. Contrast-enhanced computed tomography showed the presence of hepatosplenomegaly and a 3 cm-sized tumor in segment 3 of the liver. The hepatic venous catheterization showed mild portal hypertension. On fluorodeoxyglucose-positron emission tomography/computed tomography, progressive malignant lymphoma was suspected. However, bone marrow biopsy showed multiple noncaseating granulomas. A laparoscopic liver biopsy revealed that the liver tumor had features of Hodgkin lymphoma. There were multiple noncaseating epithelioid granulomas in the portal tracts of the liver. Splenectomy for splenomegaly and partial hepatectomy for the liver tumor were performed. Pathological examination of the resected specimens revealed multiple noncaseating epithelioid granulomas in the liver and spleen. Histopathology of th e liver tumor confirmed classic Hodgkin lymphoma with mixed cellularity. We conclude that hepatic venous catheterization, positron emission tomography/computed tomography, and pathological examinations of bone marrow, liver, and spleen are crucial for the diagnosis of sarcoidosis-lymphoma syndrome associated with portal hypertension.

Management of splenomegaly with thrombocytopenia is important in the treatment of portal hypertension. We propose a new concept: "Splanchnic Caput Medusae" in which enlarged spleen is her face and portal collateral pathways are her snake hairs. We report 2 demonstrable cases who were treated based on this new concept. Case 1 with refractory esophageal varices and splenomegaly was treated by stepwise partial splenic embolization (PSE) and endoscopic injection sclerotherapy with ligation. Spleen/liver volume ratio changed from 0.33 to 0.10. Hepatic venous pressure gradient changed from 19 to 14 mmHg. Case 2 with mesenteric shunt and splenomegaly was treated by stepwise PSE and retrograde obliteration. Spleen/liver volume ratio changed from 0.70 to 0.05. Hepatic venous pressure gradient changed from 11 to 7 mmHg. In these 2 cases, 3D-CT reconstruction images after treatment revealed that spleen- portal system reversed almost to normal form. We conclude that splenomegaly and portal collateral pathways could be considered as "Splanchnic Caput Medusae" and have to be treated by stepwise PSE.

Flood syndrome is a rare complication of cirrhosis of liver accompanied by ascites and a sudden rupture of umbilical hernia causing drainage of ascitic fluid from abdominal cavity. We report management of a case of Flood syndrome which was caused by rupture of incisional hernia. The clinical picture was similar to well described and widely accepted Flood syndrome. A 70-year-old female with decompensated hepatitis C cirrhosis was transported to the emergency department with a sudden drainage of ascitic fluid after sudden dehiscence of pre-existing incisional hernia and diffuse abdominal tenderness. Initially, she was managed by applying ostomy bag and diuretics to reduce the ascites. On 8th day of admission, a 16 Fr. drain was percutaneously placed in the left lower abdominal quadrant to divert the fluid from the abdominal wall defect. On 13th day, 80% partial splenic embolization (PSE) was attempted to control portal hypertension to reduce the ascites volume. After PSE, the hepatic venous pressure gradient reduced from 28 to 21cm H2O. The peritoneal drain was removed on 16th day and she was discharged on 22nd day. We conclude that PSE and temporary percutaneous peritoneal drainage are useful option to manage Flood syndrome.

We report a case of hemoperitoneum and sepsis from transhepatic gallbladder perforation in an 87-year-old male with acute cholecystitis who had past history of endoscopic sphincterotomy for common bile duct stone. Contrast-enhanced computed tomography (CT) showed intrahepatic and subcapsular low density areas. A wall defect of gallbladder was seen in coronal and sagittal - sections at the liver bed. Fluids obtained through the paracentesis were hemorrhagic. Percutaneous transhepatic gallbladder drainage (PTGBD) was attempted. First cholangiography revealed an orifice of fistula. Further injection of contrast medium drained into the intrahepatic secondary abscess and intraperitoneal cavity confirming the diagnosis of transhepatic gallbladder perforation. We conclude that contrast-enhanced CT with coronal and sagittal - sections and cholangiography via PTGBD tube are useful to confirm diagnosis of transhepatic gallbladder perforation.

Platelets participate in not only thrombosis and hemostasis but also other pathophysiological processes, including tumor metastasis and inflammation. However, the putative role of platelets in the development of solid organs has not yet been described. Here, we report that platelets regulate lung development through the interaction between the platelet-activation receptor, C-type lectin-like receptor-2 (Clec-2; encoded by Clec1b), and its ligand, podoplanin, a membrane protein. Clec-2 deletion in mouse platelets led to lung malformation, which caused respiratory failure and neonatal lethality. In these embryos, α-smooth muscle actin-positive alveolar duct myofibroblasts (adMYFs) were almost absent in the primary alveolar septa, which resulted in loss of alveolar elastic fibers and lung malformation. Our data suggest that the lack of adMYFs is caused by abnormal differentiation of lung mesothelial cells (luMCs), the major progenitor of adMYFs. In the developing lung, podoplanin expression is detected in alveolar epithelial cells (AECs), luMCs, and lymphatic endothelial cells (LECs). LEC-specific podoplanin knockout mice showed neonatal lethality and Clec1b-/--like lung developmental abnormalities. Notably, these Clec1b-/--like lung abnormalities were also observed after thrombocytopenia or transforming growth factor-β depletion in fetuses. We propose that the interaction between Clec-2 on platelets and podoplanin on LECs stimulates adMYF differentiation of luMCs through transforming growth factor-β signaling, thus regulating normal lung development.

Background/aim To investigate the impact of inguinal lymph node dissection (ILND) following neoadjuvant chemoradiotherapy (NACRT) for rectal cancer patients with ILN metastasis. Patients and methods Forty-three patients with rectal cancer underwent NACRT followed by curative surgery between January 2005 and December 2016. Seven patients underwent ILND after NACRT for clinically-positive ILN metastasis (ILND (+) group), while the remaining 36 did not receive ILND for clinically negative ILN metastasis (ILND (-) group). Their outcomes were retrospectively analyzed. Results Only one patient in the ILND (+) group had a local recurrence at six years after surgery. The 5-year recurrence-free survival was 100% and 65.4% in the ILND (+) and ILND (-) groups, respectively (p=0.09), and the 5-year overall survival was 100% and 83.2%, respectively (p=0.32). Conclusion ILND following NACRT seems effective for rectal cancer patients with ILN metastasis.

Background It was recently reported that expression of prospero homeobox protein-1 (PROX1) is correlated with the prognosis of esophageal cancer and colorectal cancer. However, its correlation with gastric cancer is unclear. Materials and methods Our study analyzed the effect of PROX1 knockdown on the migration, invasion and proliferation of the MKN45 human gastric cancer cell line. The correlation between PROX1 expression levels and clinicopathological factors were also analyzed in tumor samples from 99 patients with gastric cancer. Results Migration, invasion and proliferation were significantly reduced in MKN45 cells with PROX1 knockdown. PROX1 expression was detected in gastric cancer tissues at various levels. PROX1 expression levels were positively correlated with cancer stage, N factor, lymphatic vascular invasion, and vascular invasion in patients with gastric cancer. Analysis of overall and recurrence-free survival indicated that high PROX1 expression was significantly correlated with poor prognosis. Conclusion PROX1 can be an indicator of poor prognosis and a molecular target for gastric cancer treatment.

A 27-year-old woman was diagnosed with gastric cancer complicated peritoneal dissemination and direct invasion to pancreas via staging laparoscopy. After systemic chemotherapy using regimen of S-1/CDDP for 2courses, the tumor did not increase in size and peritoneal dissemination did not progress. The patient subsequently underwent distal gastrectomy as a curative surgery. The histological diagnosis was ypT4bN1M0, ypStage III B. The patient was treated with DOC/CDDP for 6 courses after surgery as adjuvant therapy. At present 6 years after surgery, the patient is alive without tumor recurrence.

No consensus has been reached with regard to the optimal treatment strategy and the prognosis of patients with advanced rectal cancer and inguinal lymph node metastasis. We, therefore, retrospectively analyzed the outcomes of 41 patients with locally advanced rectal cancer who underwent surgery after neoadjuvant chemoradiotherapy(NACRT). Six patients, with clinical inguinal lymph node metastasis determined by pretreatment imaging, underwent inguinal lymph node dissections after NACRT. Five patients survived without a relapse. Only 1 patient, who had been diagnosed with pathological inguinal lymph node metastasis, had a relapse in the right iliac lymph node 6 years after surgery. Surgical treatment after NACRT for rectal adenocarcinoma with inguinal lymph node metastasis contributes to an improvement in outcomes. NACRT plus inguinal lymph node dissection is an effective strategy for patients with inguinal lymph node metastasis from rectal adenocarcinoma.