Hepatobiliary rhabdomyosarcoma mimicking choledochal cyst: Lessons learned

INTRODUCTION

The differential diagnosis of hepatic cystic lesions is a challenging process especially in case of hepatic rhabdomyosarcoma (HRMS) presenting as hepatic cyst.

PRESENTATION OF CASE

We introduce our experience with a case of HRMS in a 3-year-old female patient who was misdiagnosed to have type IV-A choledochal cyst and definitive correct diagnosis was reached after the pathological and immunohistochemical examination of the surgically resected lesion. This case presentation is followed by important practical messages to hepatobiliary surgeons regarding HRMS.

DISCUSSION

HRMS is a rare pediatric tumor. Jaundice is the most common presentation of HRMS followed by abdominal pain and vomiting. Great effort is needed to differentiate the tumor from choledochal cyst and infectious hepatitis. Through evaluation using available imaging studies together with clinical anticipation is mandatory for establishing the correct diagnosis.

CONCLUSION

Differentiation of HRMs from choledochal cyst mandates through evaluation and clinical anticipation. HRMS should be suspected in any child with obstructive jaundice. Once diagnosis is established, multidisciplinary treatment is the best management strategy and it has proved better surgical outcome and long term survival.     

Novel 3,3a,4,5,6,7-hexahydroindazole and arylthiazolylpyrazoline derivatives as anti-inflammatory agents

A novel series of 7-benzylidene-3, 3a, 4, 5, 6, 7-hexahydro-3-phenyl-2H-indazole substituted at the 2-position were synthesized. The reaction of 2, 6-bis-benzylidenecyclohexanone (1) with thiosemicarbazide in the presence of NaOH afforded a mixture of the 3-H, 3a-H trans 2 and cis 2a diastereoisomers which have been separated by fractional recrystallization. Interaction of the first intermediate 2 with substituted phenacyl bromides, aromatic aldehydes and chloroacetic acid in presence of a mixture of acetic acid and acetic anhydride, and 2, 3-dichloroquinoxaline yielded the corresponding 7-benzylidene-3, 3a, 4, 5, 6, 7-hexahydro-3-phenyl-2H-indazole derivatives substituted at the 2-position with 4-aryl-2-thiazolyl 3a, b, 5-arylidene-4, 5-dihydro-4-oxo-2-thiazolyl 4a, b and thiazolo[4, 5-b]quinoxalin-2-yl 5, respectively. Moreover, the other intermediates 3, 5-diaryl-1-thiocarbamoyl-2-pyrazolines 7a-d were reacted with the previously-mentioned reagents and gave the corresponding 3, 5-diaryl-1-(4-aryl-2-thiazolyl)-2-pyrazolines 8a-h, 3, 5-diaryl-1-(5-arylidene-4, 5-dihydro-4-oxo-2-thiazolyl)-2-pyrazolines 9a-d and 3, 5-diaryl-1-(thiazolo[4, 5-b]quinoxalin-2-yl)-2-pyrazoline derivatives 10a, b, respectively. Some of the newly prepared compounds were subjected to evaluation for their anti-inflammatory activity. The structures of the new compounds were confirmed by elemental analyses as well as (1)H-NMR, IR, and MS data.     

Outcomes of Living Donor Liver Transplantation for Patients with Preoperative Portal Vein Problems

Background

Portal vein thrombosis (PVT) is a common complication for patients with end-stage liver disease. The presence of PVT used to be a contraindication to living donor liver transplantation (LDLT). The aim of this study is to evaluate the influence of preoperative PVT on perioperative and long-term outcomes of the recipients after LDLT.

Methods

We reviewed the data of patients who underwent LDLT during the period between 2004 till 2017.

Results

During the study period, 500 cases underwent LDLT. Patients were divided into three groups. Group I included non-PVT, 446 patients (89.2%); group II included attenuated PV, 26 patients (5.2%); and group III included PVT, 28 patients (5.6%). Higher incidence of hematemesis and encephalopathy was detected in PVT (p?=?0.001). Longer anhepatic phase was found in PVT (p?=?0.013). There were no significant differences between regarding operation time, blood loss, transfusion requirements, ICU, and hospital stay. The 1-, 3-, and 5-year overall survival (OS) rates of non-PVT were 80.5%, 77.7%, and 75%, and for attenuated PV were 84.6%, 79.6%, and 73.5%, and for PVT were 88.3%, 64.4%, and 64.4%, respectively. There was no significant difference between the groups regarding OS rates (logrank 0.793).

Conclusion

Preoperative PVT increases the complexity of LDLT operation, but it does not reduce the OS rates of such patients.

     

SPOP,ZEB-1 and E-cadherin expression in clear cell renal cell carcinoma (cc-RCC): Clinicopathological and prognostic significance

Background

Clear cell renal cell carcinoma (cc-RCC), is a serious cancer regarding; its fatality, liability for metastases and chemoresistance, so identification of recent therapeutic targets to improve the patients prognosis is needed. SPOP is a BTB/POZ domain containing speckle-type POZ protein, has been identified as an E3 ubiquitin ligase component. ZEB1 is an essential epithelial mesenchymal transition (EMT) activator; E-cadherin is a cell adhesion protein that had been detected in normal epithelial cells membrane.

Prevention of sodium valproate-induced hepatotoxicity by curcumin, rosiglitazone and N-acetylcysteine in rats

The present study was designed to examine the potential preventive effect of curcumin (CMN; CAS 458-37-7), rosiglitazone (RGN; CAS 155141-29-0), N-acetylcysteine (NAC; CAS 616-91-1), resveratrol (RSV; CAS 501-36-0), and losartan (LOS; CAS 114798-26-4) on sodium valproate-induced hepatotoxicity. Sodium valproate (SVP; CAS 1069-66-5) was given at a dose of 250 mg/kg i. p. 3 times daily for one week. The tested compounds were given simultaneously with SVP for one week. The results demonstrate that CMN, RGN and NAC treatment can confer protection from SVP-induced hepatotoxicity. The second part of the study includes an evaluation of the effect of CMN, RGN and NAC on the anticonvulsant activity of SVP against pentetrazole-induced seizures in mice. The results demonstrate that CMN, RGN and NAC do not affect the anticonvulsant activity of SVP. Combined administration of either of CMN, RGN and NAC with valproate appears to be beneficial in reducing valproate-induced hepatotoxicity.     

Hepcidin-orchestrated Hemogram and Iron Homeostatic Patterns in Two Models of Subchronic Hepatic injury

Objective This study was designed to evaluate hematological disorders and the orchestrating roles of hepcidin and IL-6 in rat models of thioacetamide(TAA) and carbon tetrachloride(CCl_4) hepatotoxicity. Methods Rats were intraperitoneally injected with TAA(10 mg/100 g rat weight dissolved in isosaline) or CCl_4(100 μL/100 g rat weight diluted as 1:4 in corn oil) twice weekly for eight consecutive weeks to induce subchronic liver fibrosis. Blood and tissue samples were collected and analyzed. Results CCl_4 but not TAA significantly decreased the RBCs, Hb, PCV, and MCV values with minimal alterations in other erythrocytic indices. Both hepatotoxins showed leukocytosis, granulocytosis, and thrombocytopenia. By the end of the experiment, the erythropoietin level increased in the CCl_4 model. The serum iron, UIBC, TIBC, transferrin saturation%, and serum transferrin concentration values significantly decreased, whereas that of ferritin increased in the CCl_4 model. TAA increased the iron parameters toward iron overload. RT-PCR analysis revealed increased expression of hepatic hepcidin and IL-6 m RNAs in the CCl_4 model and suppressed hepcidin expression without significant effect on IL-6 in the TAA model. Conclusion These data suggest differences driven by hepcidin and IL-6 expression between CCl_4 and TAA liver fibrosis models and are of clinical importance for diagnosis and therapeutics of liver diseases.     

Surgical Management of Giant Hepatic Hemangioma: Single Center’s Experience with 144 Patients

Background

Hepatic hemangioma (HH) is the most common benign solid tumor of the liver. The aim of this study is to review our experiences of surgical treatment for giant HH and to show the impact of HH size and type of surgical resection on surgical outcomes.

Patients and Methods

This is a retrospective study of the cases who underwent surgery for giant HH during the period from January 2000 to April 2017.

Results

Elective surgery was performed for 144 patients who had giant HH. The median diameter of resected HH was 10 cm (5–31 cm). Enucleation was performed for 92 (63.9%) patients and anatomical resection was required in 52 (36.1%) patients. No statistical difference between enucleation and resection as regards intraoperative and postoperative findings. The amount of intraoperative blood loss is significantly more in HH?>?10 cm (300 vs. 575 ml, P?=?0.007), the need of blood transfusion was significantly more in HH?>?10 cm (P?=?0.000), and the operation time was significantly longer in HH?>?10 cm (120 vs. 180 min, P?=?0.000). The size of HH had no significant effect as regards the development of postoperative complications.

Conclusion

Giant hemangioma can be treated surgically with low incidence of morbidity and mortality. No statistical difference between enucleation and resection as regards surgical outcomes. In left lobe HH, HH located deeper in posterior hepatic segments and in multiple HH, hepatic resection is preferred. The size of the HH had significant impact intraoperative blood loss and operative time

     

Mediation of the schistosomicidal effect of praziquantel through nitric oxide

The effect of praziquantel (CAS 55268-74-1) on serum nitrate/nitrite level (a marker for nitric oxide (NO) synthesis) in S. mansoni infected mice was studied. The effects of the NO precursor (L-arginine) and NO-synthase inhibitor (NG-nitro-L-arginine methyl ester, L-NAME) on the effect of praziquantel on nitrate/nitrite level as well as on its antischistosomal activity were also evaluated. Praziquantel increased nitrate/nitrite level in serum of infected mice in a dose dependent manner. An oral dose of 75 mg/kg praziquantel produces a significant (p < 0.05) increase in serum nitrate/nitrite level by about 3.5 fold. Administration of L-arginine (200 mg/kg orally) induced a significant (p < 0.05) increase in nitrate/nitrite level (to about 5 fold) compared to praziquantel 75 mg/kg alone. Praziquantel-induced increase in nitrate/nitrite level was significantly reduced by administration of L-NAME 100 mg/kg. The antischistosomal activity of praziquantel was evaluated using two models: hepatic shift model and reduction of worm burden. In the hepatic shift model, praziquantel increased the percentage of worms in the liver (from 5% in control to 60%). Praziquantel-induced hepatic shift was not significantly affected by concurrent L-arginine or L-NAME administration. In the second model, praziquantel induced a significant decrease of the worm burden (p < 0.05) and the action of praziquantel was significantly increased by L-arginine and reduced by L-NAME administration. In conclusion, NO is possibly involved in the antibilharzial effect of praziquantel and administration of L-arginine with praziquantel produces beneficial antibilharzial effect.     

Effect of bosentan (ETA/ETB receptor antagonist) on metabolic changes during stress and diabetes.

Elevated plasma ET-1 levels have been reported in several conditions such as stress and diabetes. ET-1 is found to cause insulin resistance and to stimulate liver glycogenolysis. The question arises whether ET-1 has a role in the metabolic changes occurring in such conditions. To test this, we studied the possible effect of the endothelin receptor antagonist, bosentan (50 and 100 mg kg(-1)) on serum glucose and insulin levels as well as on liver glycogen contents in normoglycemic stressed animals. In addition, the effect of bosentan on serum glucose and insulin levels in both mild and severely diabetic rats and its effect on insulin-induced hypoglycemia were also determined. Restraining water immersion stress was used as a model for severe stress reported to elevate plasma ET-1 level. Mild diabetes was induced in rats by intraperitoneal injection of a low dose of streptozotocin (38 mg kg(-1)) while severe diabetes was induced by intraperitoneal injection of a higher dose of streptozotocin (45 mg kg(-1)). Bosentan partially prevented stress-induced both hyperglycemia and decrease in glycogen content while it completely blocked the stress-induced decrease in insulin level in normoglycemic stressed rats. Bosentan also decreased serum glucose level without any effect on insulin secretion in mild diabetic rats and potentiated the hypoglycemic action of insulin.     

Tranilast ameliorates impaired hepatic functions in Schistosoma mansoni-infected mice

The ability of tranilast, a mast cell stabilizer and anti-transforming growth factor_β (TGF_β) to improve impaired hepatic functions in Schistosoma mansoni (S. mansoni)-infected mice, was investigated, providing the first evidence on the ability of tranilast to improve hepatic impairment due to schistosomal infection. Tranilast had significant beneficial effects against progression of hepatic fibrosis in S. mansoni-infected mice treated with praziquantel and those untreated. Different aspects of drug activity were investigated. Its effect on serum liver functions was evaluated by estimating: alanine aminotransferase, aspartate aminotransferase, total bilirubin, alkaline phosphatase and albumin. Its effect on the extent of liver fibrosis, through estimation of hepatic hydroxyproline and hepatic collagen content in liver hydrolysates, was also evaluated. Also, the expression of profibrogenic mediators, such as serum TGF_β1, was estimated. Finally, the effect on S. mansoni infection itself was studied, via histopathological examination of liver specimens stained with both hematoxylin–eosin and Masson’s trichome stains. Tranilast ameliorated the harmful effects of S. mansoni infection on the liver. Such action was manifested in its significant ability to improve impaired hepatic functions, reduce histopathological changes, lower hepatic collagen content and finally reduce serum TGF_β1 levels. The beneficial effect of tranilast may be in part due to its ability to reduce the production of profibrogenic mediators in the infected animals by improving the host immune response or by interfering with critical steps in the fibrogenic cascade.