Focal nodular hyperplasia (FNH) is the second most common type of benign liver tumour, and it is more frequently encountered in young women and the right hemi-liver. Most patients are asymptomatic, and there is no malignant potential. Thus, the current guidelines do not recommend any treatment for uncomplicated FNH, and surgery is indicated only for some atypical patients (with complications or uncertain diagnosis). Pedunculated forms are atypical, rare features of FNH and appear to be more frequently associated with complications. We hereby report the case of a 40-year-old woman diagnosed at computed tomography with a lobulated subhepatic FNH with a long pedicle originating from segment III of the liver, which was successfully resected. Pedunculated FNH is associated with a challenging diagnosis and may require resection in order to avert potential complications such as compression of surrounding organs or torsion of the pedicle.

Invasion of portal vein (PV)/ superior mesenteric vein (SMV) in pancreatic ductal adenocarcinoma (PDAC) is no longer a contraindication for resection when reconstruction is technically feasible. However, the literature data reached conflicting conclusions regarding the early and long-term outcomes of patients with venous resection and pancreatectomies for PDAC. The study aims to present the outcomes in a large series of patients with pancreatectomies and associated PV/ SMV resection for PDAC, in a single center experience. The data of 100 patients with pancreatectomies and PV and/ or SMV resection performed between 2002 and 2016 (February, 1st) were retrospectively analyzed from a prospectively maintained electronic database, which included 474 pancreatectomies for PDAC. Only patients with a final pathological diagnosis of PDAC were included in the present study. Overall, 21.1% of patients with pancreatectomies for PDAC required a venous resection (100 patients out of 474 patients). Segmental resection was performed in 77 patients (out of 100 patients with pancreatectomies and venous resection - 77%), while 23 patients (23%) have had tangential venous resection. In the group of patients with segmental venous resection, reconstruction was made by end-to-end anastomosis in 53 patients (out of 77 patients - 68.8%), while in 24 patients (out of 77 patients - 31.2%) a graft interposition was necessary. Negative resections margins were obtained in 63 patients (63%). Histological tumor invasion of the resected vein was confirmed in 64 patients (64%). Postoperative complications occurred in 47 patients (47%), with severe complications (i.e., Dindo-Clavien grade III-V) in 19 patients (19%). Postoperative pancreatic fistulae, delayed gastric emptying and post-pancreatectomy hemorrhage rates were 9%, 20% and 15%, respectively. PV/ SMV thrombosis occurred in 5 patients (5%). The 90-day mortality rate in the group of patients with venous only resection, without any associated procedures, was 8%. Adjuvant treatment was performed in 63 patients (63%), while only 2 patients (2%) underwent neoadjuvant chemotherapy. Median follow-up time was 105 months (range, 3 - 186 months), with a median overall survival time of 13 months (range, 3 - 186 months). In the group of patients with negative resection margins, the median overall survival time was 16 months (range, 3 - 186 months). PV/ SMV resection during pancreatectomies for PDAC is technically feasible, and grafts are rarely required for venous reconstruction. However, venous resection is associated with high postoperative complications rates, and the mortality rate is not neglectable. The main goal of such complex procedure is to obtain negative resection margins, a situation associated with encouraging survival rates.

Introducere: Cancerul gastric este rareori cauza unei hemoragii digestive superioare acute. Comorbidităţile pacientului pot avea un efect negativ atât asupra rezultatelor imediate cât şi asupra celor tardive, după rezecţia chirurgicală a unui cancer gastric. Asocierea cancerului gastric cu hemofilia A şi angiodisplazie nu a mai fost raportată până în prezent în literatura de specialitate, iar impactul acestei asocieri asupra rezultatelor postoperatorii este necunoscut. Prezentarea cazului: Un bărbat de 49 de ani, cunoscut cu hemofilie A, se prezintă cu hemoragie digestivă superioară şi anemie secundară severă, fiind diagnosticat cu adenocarcinom gastric. Se practică gastrectomie totală cu splenectomie şi limfodisecţie D2. Evoluţia postoperatorie a fost complicată de apariţia unui nou episod de hemoragie digestivă datorat prezenţei leziunior angiodisplazice la nivelul cecului şi jejunului; episodul de hemoragie digestivă a fost tratat fde această dată, cu success, prin embolizare radiologică. În perioada pre şi postoperatorie pacientul a primit factor VIII, dar a dezvoltat auto-anticorpi împotriva factorului VIII. Astfel, administrarea de factor VIII a fost întreruptă şi înlocuită cu FEIBA ("factor

A 48-year-old patient was diagnosed with an invasive ductal carcinoma with the immunohistochemistry positive for estrogen and progesterone receptors, positive Her2 and three liver metastases. After nine cycles of chemotherapy, a favorable tumor response was identified at the level of the primary tumor as well as for the liver lesions: two of the metastases have disappeared, and the third one decreased in dimensions. The patient was operated in our unit, a lumpectomy together with a level II axillary lymph nodes dissection and a non-anatomic resection of the segment V of the liver was performed. A subgroup of patients with stage IV breast cancer with limited liver metastases and no extrahepatic disease might benefit from an aggressive combined cytotoxic and surgical strategy regarding disease control and overall survival.

Purpose Incidence, risk factors, and clinical consequences of pancreatic fistula (POPF) after D1+/D2 radical gastrectomy have not been well investigated in Western patients, particularly those from Eastern Europe. Materials and Methods A total of 358 D1+/D2 radical gastrectomies were performed by surgeons with high caseloads in a single surgical center from 2002 to 2017. A retrospective analysis of data that were prospectively gathered in an electronic database was performed. POPF was defined and graded according to the International Study Group for Pancreatic Surgery (ISGPS) criteria. Uni- and multivariate analyses were performed to identify potential predictors of POPF. Additionally, the impact of POPF on early complications and long-term outcomes were investigated. Results POPF was observed in 20 patients (5.6%), according to the updated ISGPS grading system. Cardiovascular comorbidities emerged as the single independent predictor of POPF formation (risk ratio, 3.051; 95% confidence interval, 1.161–8.019; P=0.024). POPF occurrence was associated with statistically significant increased rates of postoperative hemorrhage requiring re-laparotomy (P=0.029), anastomotic leak (P=0.002), 90-day mortality (P=0.036), and prolonged hospital stay (P<0.001). The long-term survival of patients with gastric adenocarcinoma was not affected by POPF (P=0.661). Conclusions In this large series of Eastern European patients, the clinically relevant rate of POPF after D1+/D2 radical gastrectomy was low. The presence of co-existing cardiovascular disease favored the occurrence of POPF and was associated with an increased risk of postoperative bleeding, anastomotic leak, 90-day mortality, and prolonged hospital stay. POPF was not found to affect the long-term survival of patients with gastric adenocarcinoma.

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Background: The deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessively inherited disease resulting from loss-of-function mutations in ADA2, formerly named CECR1 (cat eye syndrome chromosome region, candidate 1) gene. Disease manifestations could be separated into three major phenotypes: inflammatory/vascular, immune dysregulatory and hematologic, however, most patients presented with significant overlap between these three phenotype groups. Due to its large heterogeneity, DADA2 is often mistaken with other connective tissue diseases. Over 60 DADA2-associated mutations have been identified so far, still we describe a new mutation with possible relation to severe hematological involvement Case presentation: We present a case of DADA2 deficiency with disease onset at 3 years old with fever, oligoarthritis, mouth sores and recurrent episodes of neutropenia misdiagnosed as Behcet disease, then systemic lupus erythematosus. At the age of 18 she developed gastrointestinal vasculitis complicated with multiple bowel resections, enterocutaneous fistulas and lower limb ischemia. Genetic testing reveals heterozygous mutations at positions 139 (exon 2) and 661_664 (exon 4) of the coding sequence of ADA2 (c.139G>A, c.661_664del), resulting respectively in amino acid substitution p.Gly47Arg and premature translational stop signal p.Ala221Glnfs*45 in ADA2. Thus, the patient was diagnosed with ADA2 deficiency. TNF blockers were started but the patient died due to septic complications related to severe, nonresponsive pancytopenia. Conclusions: Our case highlights the valuable diagnostic benefit of early specific genetic testing for patients with complex unclear diseases in which vasculopathy/vasculitis, dysregulated immune function and/or hematologic abnormalities may predominate.

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