Retroperitoneal liposarcoma and craniosynostosis: possible genomic relationship, case report, and literature review.

Based on a case report, this review explores the genomic landscape for patients with liposarcomas and possible relationships with gene mutations related to craniosynostosis. We describe the case of a 40-year-old man, known for a surgical correction of craniosynostosis before the age of 1 year, who underwent a radical resection of a voluminous retroperitoneal liposarcoma; histopathological analysis revealed a low-grade well-differentiated, mostly sclerosing, liposarcoma. A genetic analysis searching for mutations in blood DNA was performed and did not detect any specific mutation. A literature review was also conducted. Several tumors related to syndromic and non-syndromic craniosynostosis are mentioned in the literature; no specific link with retroperitoneal liposarcoma is established but the FGFR3 mutation is detected in dedifferentiated liposarcomas. To date, no case has been reported in the literature demonstrating a genetic relationship between craniosynostosis and low-grade differentiated retroperitoneal liposarcoma. We conclude that further studies for gene complex mutations should be conducted to show a possible genetic relationship between retroperitoneal liposarcoma and craniosynostosis.

Split liver technique with middle hepatic vein reconstruction on livers from transplant hepatectomies: a useful tool for surgical improvement.

Livers removed during transplant hepatectomies could represent a useful anatomic ex vivo resource for surgical training, since they are intact and not altered by post-mortem changes yet. The aim of this study is to investigate the effectiveness of such kind of surgical training applied on some hepatic surgery techniques. In the present paper, we focused on split liver operation and middle hepatic vein (MHV) bipartition/reconstruction, since these procedures have a quite long learning curve. Seven native livers were submitted to split liver procedure by a senior resident assisted by a fully trained hepatic surgeon. Pre-splitting ultrasound mapping was compared to pre-operative CT scan. The whole graft was divided into two hemi-livers and the MHV into two hemi-confluents and reconstructed by venous or arterial patches obtained by deceased donor iliac homograft. Water tightness of the anastomosis was confirmed by hydro-pneumatic test and bench portal perfusion. Reduction in operating time was considered an indirect indicator of surgical skill improvement. In all cases, the US confirmed the anatomical distribution of MHV tributaries observed by pre-transplant CT scan. The "ex situ" splitting procedures and MHV bipartition and reconstruction were performed in all native livers in the usual time required for liver transplantation bench surgery (range 50-75 min). Liver grafts removed during hepatectomy could represent a useful resource of intact organs to perform surgical training and boost surgical confidence. In our initial experience, the study of venous drainage of the MHV and application of liver splitting technique and MHV reconstruction resulted technically feasible.

Liver-allocation policies for patients affected by HCC in Europe.

The main goal of organ allocation systems is to guarantee an equal access to the limited resource of liver grafts for every patients on the waiting list, balancing between the ethical principles of equity, utility, benefit, need, and fairness. The European heath care scenario is very complex, as it is essentially decentralized and each Nation and Regions inside the nation, operate on a significant degree of autonomy. Furthermore the epidemiology of liver diseases and HCC, which is different among European countries, clearly inpacts on indications and priorities. The aims of this review are to analyze liver allocation policies for hepatocellular carcinoma, among different European. The European area considered for this analysis included 5 macro-areas or countries, which have similar policies for liver sharing and allocation: Centro Nazionale Trapianti (CNT) in Italy; Eurotransplant (Germany, the Netherlands, Belgium, Luxembourg, Austria, Hungary, Slovenia, and Croatia); Organizacion Nacional de Transplantes (ONT) in Spain; Etablissement français des Greffes (EfG) in France; NHS Blood & Transplant (NHSBT) in the United Kingdom and Ireland; Scandiatransplant (Sweden, Norway, Finland, Denmark, and Iceland). Each identified area, as network for organ sharing in Europe, adopts an allocation system based either on a policy center oriented or on a policy patient oriented. Priorization of patients affected by HCC in the waiting list for deceased donors liver transplant worldwide is dominated by 2 main principles: urgency and utility. Despite the absence of a common organs allocation policy over the Eurpean countries, long-term survival patients listed for transplant due to HCC are comparable to the long-term survival reported in the UNOS register. However, as the principles of allocation are being re-discussed and new proposals emerge, and the epidemiology of liver disease changes, an effort toward a common system is highly advisable.

Residual right portal branch flow after first-step ALPPS: artifact or homeostatic response?

BACKGROUND/AIMS: Mutual interactions between portal vein and hepatic artery can be documented during hepatobiliary surgery. Associating Liver Partition and Portal Vein Ligation for Staged Hepatectomy (ALPPS) is a recently introduced surgical technique which can also represent a unique living human model to investigate intrahepatic blood circulation. We report three consecutive cases in which a residual right portal branch flow was clearly detectable after first-step ALPPS, and try to further investigate this unexpected finding with intraoperative clamping tests. METHODOLOGY: Every patient was evaluated with CT scan 7 days after first-step ALPPS and Intraoperative Doppler Ultrasonography (IOUS) at both steps of the procedure. RESULTS: In every patient, CT scan and second-step IOUS demonstrated a clear hepatopetal flow distally to the divided right portal branch. The flow was present after right biliary duct clamping and stopped after right total hilar clamping as well as after right hepatic artery occlusion. CONCLUSIONS: Neither cross-portal circulation between the two hemilivers nor trans-sinusoidal backflow from the hepatic veins can explain these findings, which are rather consistent with a refilling of the occluded portal branch through the opening of intrahepatic arterioportal shunts (APS). APS could represent the simplest homeostatic mechanism that regulate intrahepatic blood flow.

Pancreatico-duodenectomy and postoperative pancreatic fistula: risk factors and technical considerations in a specialized HPB center.

Postoperative pancreatic fistula (POPF) is a common complication of pancreatic resection. Aim of this study is to identify variables related to the development of POPF, analyze their clinical significance and discuss our current approach to the pancreatico-jejunal anastomosis. A series of 129 patients undergoing pancreatico-duodenectomy (PD) have been analyzed. Patients were divided in two groups: group F, 26 patients who have developed POPF; group NF, 103 patients who have not developed POPF. Demographic, clinical and intraoperative data were compared. Seventy-six patients had an end-to-side (ES) pancreatico-jejuno anastomosis, 53 an end-to-end (EE) anastomosis. Fifteen patients developed grade A fistulas, seven grade B, and four grade C; two patients with grade C fistula died from septic shock. Preoperative bile duct lithiasis, diameter of the pancreatic duct and consistency of the pancreatic stump were significantly different between F and NF groups at multivariate analysis. POPF has been related to clinical and biological parameters: preoperative bile duct lithiasis and challenging pancreatico-jejunal anastomosis (with small pancreatic duct and friable pancreatic stump) are the most prominent according to our experience. As the incidence of POPF seems to be related to technically demanding surgery, we presently reserve the EE anastomosis to the cases in which a friable gland or a very small duct will make a direct anastomosis on the pancreatic duct unreliable. In case of grade C fistulas a total spleen-preserving pancreatectomy should be considered an adequate treatment to prevent the onset of a multi-organ failure or a septic shock if no other treatment seems suitable.

Pancreatoduodenectomy: Risk Factors of Postoperative Pancreatic Fistula.

BACKGROUND/AIMS: The aim of the present study is the analysis of risk factors of postoperative pancreatic fistula (POPF) and of clinical outcome after pancreatoduodenectomy (PD) in a retrospective multicentric review of the patient cohort. METHODOLOGY: From January 2003 to July 2013 143 patients underwent PD: 138 cases were included and 3 groups were identified according to the different types of anastomosis: Group 1 invaginating end-to-end pancreatojejunostomy, Group 2 end-to-side pancreatojejunostomy with duct-to-mucosa anastomosis, Group 3 end-to-side pancreatogastrostomy. RESULTS: Twenty-one % of patients developed POPF, 16% in Group 1, 27% in Group 2, 12% in Group 3. Forty % grade A, 13% grade B and 47% grade C total POPF. It results that POPF occurred in 16% of hard and in 40% of soft pancreatic texture; in 11.4% of dilated Wirsung versus 30.8% of non dilated (p = 0.007). Overall actuarial 1 and 3 year survival after PD is 69% and 48% respectively. Perioperative mortality is 5.8% overall, 17.85% for grade C. CONCLUSIONS: No differences have been found among surgical anastomosis techniques. Soft tissues seem to increase, while dilated Wirsung seems to decrease POPF rate. The development of POPF increase morbidity but it doesn't affect overall survival, more strictly related to tumour histopathology.