Diagnosis, morphopathological profile and treatment of mucinous cystadenoma of the pancreas - a single center experience.

AIM: Pancreatic mucinous cystadenoma (MCA) occurs almost exclusively in perimenopausal women and represents between 10% and 45% of cystic neoplasm of the pancreas, being considered a premalignant lesion. MATERIALS AND METHODS: From 1983 to 2017, 31 patients underwent surgery for MCA of the pancreas in our Center. The median age was 47 years (range 17-81 years). All data were obtained retrospectively. RESULTS: The female∕male gender ratio was 14.5∕1. Most of the patients (90.3%) were symptomatic. The most common clinical manifestation was non-specific abdominal pain (58.06%), followed by fatigue and vomiting. The median cyst size was 7 cm, with a range between 2 cm and 15 cm. There were 35 procedures in 31 patients (in four patients the resection was preceded by a drainage procedure). From the 28 resections, most of them (89.28%) were performed by an open approach; a minimal invasive approach was used in three patients (robotic - two; laparoscopic - one). Most of the resections (82.14%) were distal pancreatectomies. In all cases, the final diagnosis was based on histological examination that revealed columnar epithelium and ovarian-type stroma. Postoperative complications occurred in 10 (34.48%) patients. Postoperative mortality was 3.44% (one patient) by septic shock secondary to acute postoperative pancreatitis. CONCLUSIONS: MCAs represent a rare pancreatic pathology with challenging diagnostic and therapeutic implications. Multi-detector computed tomography (MDCT) scan, endoscopic ultrasound (EUS) and magnetic resonance imaging (MRI)∕magnetic resonance cholangiopancreatography (MRCP) are useful in the differential diagnosis with other pancreatic fluid collections and treatment. Oncological surgical resections are recommended. Histopathological examination establishes the final diagnosis. The most common postoperative complication is pancreatic fistula.

Resection of Concomitant Hepatic and Extrahepatic Metastases from Colorectal Cancer - A Worthwhile Operation?

Background: The benefit of hepatic resection in case of concomitant colorectal hepatic and extrahepatic metastases (CHEHMs) is still debatable. The purpose of this study is to assess the results of resection of hepatic and extrahepatic metastases in patients with CHEHMs in a high-volume center for both hepatobiliary and colorectal surgery and to identify prognostic factors that correlate with longer survival in these patients. METHOD: It was performed a retrospective analysis of 678 consecutive patients with liver resection for colorectal cancer metastases operated in a single Centre between April 1996 and March 2016. Among these, 73 patients presented CHEHMs. Univariate analysis was performed to identify the risk factors for overall survival (OS) in these patients. Results: There were 20 CHMs located at the lymphatic node level, 20 at the peritoneal level, 12 at the ovary and lung level, 12 presenting as local relapses and 9 other sites. 53 curative resections (R0) were performed. The difference in overall survival between the CHEHMs group and the CHMs group is statistically significant for the entire groups (p 0.0001), as well as in patients who underwent R0 resection (p 0.0001). In CHEHMs group, the OS was statistically significant higher in patients who underwent R0 resection vs. those with R1/R2 resection (p=0.004). Three variables were identified as prognostic factors for poor OS following univariate analysis: 4 or more hepatic metastases, major hepatectomy and the performance of operation during first period of the study (1996 - 2004). There was a tendency toward better OS in patients with ovarian or pulmonary location of extrahepatic disease, although the difference was not statistically significant. CONCLUSION: In patients with concomitant hepatic and extrahepatic metastases, complete resection of metastatic burden significantly prolong survival. The patients with up to 4 liver metastases, resectable by minor hepatectomy benefit the most from this aggressive onco-surgical management.

Curative Intent Treatment of Hepatocellular Carcinoma - 844 Cases Treated in a General Surgery and Liver Transplantation Center.

Background: The objective of this study is to assess the outcome of the patients treated for hepatocellular carcinoma (HCC) in a General Surgery and Liver Transplantation Center. Methods: This retrospective study includes 844 patients diagnosed with HCC and surgically treated with curative intent methods. Curative intent treatment is mainly based on surgery, consisting of liver resection (LR), liver transplantation (LT). Tumor ablation could become the choice of treatment in HCC cases not manageable for surgery (LT or LR). 518 patients underwent LR, 162 patients benefited from LT and in 164 patients radiofrequency ablation (RFA) was performed. 615 patients (73%) presented liver cirrhosis. Results: Mordidity rates of patient treated for HCC was 30% and mortality was 4,3% for the entire study population. Five year overall survival rate was 39 % with statistically significant differences between transplanted, resected, or ablated patients (p 0.05) with better results in case of LT followed by LR and RFA. Conclusions: In HCC patients without liver cirrhosis, liver resection is the treatment of choice. For early HCC occurred on cirrhosis, LT offers the best outcome in terms of overall and disease free survival. RFA colud be a curative method for HCC patients not amenable for LT of LR.

Fibrolamellar hepatocellular carcinoma with ovarian metastasis - an unusual presentation.

AIM: Fibrolamellar carcinoma (FLC) has been considered a distinct clinical entity vs. hepatocellular carcinoma, with respect to its epidemiology, etiology, and prognosis. CASE PRESENTATION: We describe the unusual case of a 23-year-old female patient with FLC and ovarian (Krukenberg) and peritoneal metastases, clinically mimicking an ovarian carcinoma. Multiple recurrences occurred despite initial R0 resection and chemotherapy, requiring surgical treatment. The patient survived five years and died from generalized disease. DISCUSSION: The particularities of our case are discussed by comparison with the other two similar cases and other date from the literature. CONCLUSIONS: To our knowledge, the ovarian involvement encountered in our case is the third case published in literature, being explained by the superficial location of the liver tumor.

Klatskin-mimicking lesions--a case series and literature review.

Obstruction of the hepatic hilum in patients without prior surgery is generally due to hilar adenocarcinoma (Klatskin tumor). However, not all the hilar strictures are malignant. Although uncommon, benign strictures of the proximal bile duct should be taken into consideration in differential diagnosis of Klatskin tumors, since the incidence could reach up to 25% of patients with presumed Klatskin tumor diagnosis. This group of benign proximal bile duct strictures (Klatskin-mimicking lesions) is usually represented by segmental fibrosis and non-specific chronic inflammation. The clinical and imaging features can not differentiate between benign and malignant strictures. Herein, we present a case series of three patients with benign proximal bile duct strictures (representing 4.1% of 73 patients resected with presumptive preoperative diagnosis of Klatskin tumor) and literature review. There are presented the clinical and biochemical features, imaging preoperative workup, surgical treatment and histological analysis of the specimen, along with postoperative outcome. For benign strictures of the hilum limited resections are curative. However, despite new diagnosis tools developed in the last years, patients with hilar obstructions still require unnecessary extensive resections due to impossibility of excluding the malignancy. In all cases of proximal bile duct obstruction presumed malignant, they should be managed accordingly, even with the risk of over-treatment for some benign lesions.

Isolated resection of the caudate lobe: indications, technique and results.

BACKGROUND/AIMS: This paper reports a series of 24 isolated caudate lobe resections (ICLR), performed for 13 benign tumors (10 hemangiomas, 2 focal nodular hyperplasias, 1 adenoma) and 11 malignant tumors (3 hepatocarcinomas, 1 peripheral cholangiocarcinoma and 7 metastatic - 5 colorectal carcinomas, 1 breast carcinoma, 1 adrenal carcinoma). Klatskin tumors were excluded. METHODOLOGY: There were 10 hemangioma enucleations, 7 Spiegel lobe resections and 7 high dorsal resections. Total vascular exclusion was performed in 7 cases. Vascular resection with reconstruction was necessary in 5 cases. RESULTS: Complications occurred in 7 cases (3 bile leaks, 3 abdominal fluid collections and one liver failure leading to death). From the 10 patients with malignant tumors who survived the operation, 7 developed recurrences: 2 intrahepatic, 1 retroperitoneal, 4 systemic. Five patients are alive (3 without recurrence). One patient died of multiple complications after a repeat hepatectomy and colectomy. Three patients died from generalized disease. Another patient, with generalized disease, was lost from follow-up. CONCLUSIONS: ICLR is a difficult operation, especially with malignant tumors. Total vascular exclusion of the liver is routinely recommended in high dorsal resection. Malignant tumors located in the caudate lobe have a poor prognosis; local and, especially, distant metastases are frequent. Aggressive chemotherapy and follow-up are recommended.

Adrenalectomy for metastases from hepatocellular carcinoma - a single center experience.

BACKGROUND: Adrenal metastases (AM) from hepatocellular carcinoma (HCC) are rarely seen in clinical practice. The treatment is not standardized, the indications and efficacy of different therapeutic approaches being still controversial. PATIENTS: Between January 1995 and December 2005, 174 patients underwent liver resection for HCC in our center. AM were detected in four patients (2.3%): three of them had HCC and synchronous AM, and the remaining one developed AM 10 months after liver resection. All the patients with AM were treated by adrenalectomy (simultaneously with liver resection in synchronous metastases), followed by systemic chemotherapy. Non-resectable multifocal liver recurrences occurred in two patients, one of them having also a contralateral adrenal metastasis; these two patients are presently alive 26 and 43 months after adrenalectomy, respectively. Another patient died by liver recurrence 27 months postoperatively. The fourth patient is disease-free at 17 months after the initial operation. CONCLUSIONS: Adrenalectomy for AM from HCC should be performed whenever the primary tumor is well therapeutically controlled and the patient has a good performance status. Adrenalectomy offers the chance of more than 2 years survival in many patients. However, once AM are detected, the prognosis remains poor.

Sequential therapy in a case of extrahepatic biliary atresia associated with ventricular septal defect: liver transplantation followed by surgical closure of the septal defect.

Patients with extrahepatic biliary atresia (EHBA), the commonest indication for liver transplantation in pediatric cases, may have associated congenital anomalies, e.g. interventricular septal defect (IVSD). Even if there is a well established therapeutical algorithm for a ventricular septal defect as an isolated disease, the surgical approach raises special problems in a liver transplanted patient. We present the case of a little girl with EHBA and IVSD. The patient underwent a living related liver transplantation when she was 1 year old. Persistence of the symptomatic IVSD required open heart surgery, performed 9 months posttransplantation. The decision to perform the closure of the IVSD was difficult, yet it proved to be beneficial; the child had an uneventful postoperative course, with remission of the symptoms and a good late outcome. To our knowledge, this is the first case of a living-related liver transplantation patient undergoing open heart surgery for IVSD closure.

Homozygous familial hypercholesterolemia: specific indication for domino liver transplantation.

BACKGROUND: Domino liver transplantation is one possibility to overcome the discrepancy between the small number of liver donors and the long waiting lists. Homozygous familial hypercholesterolemia (FHC) is a genetic disorder of lipoprotein metabolism defined by the absence or small number of functional low-density lipoprotein receptors (LDL-Rs) and the ensuing high levels of serum cholesterol. We report a case of a patient with FHC whose liver was used for domino transplantation in a patient with cirrhosis and hepatocellular carcinoma. METHODS: The patient diagnosed with FHC received the large part of a split liver. The liver of the patient with FHC was then transplanted into the patient with cirrhosis and hepatocellular carcinoma. Quantification of extrahepatic LDL-R was performed by flow cytometry on monocytes, and the gene expression of LDL-R was assayed by reverse transcriptase-polymerase chain reaction on monocyte-derived macrophages and cultured fibroblasts isolated from the patients. RESULTS: One year after surgery, the donor's serum cholesterol (without treatment) was normal, and the recipient's serum cholesterol (with simvastatin treatment) was slightly increased. Quantification of peripheral LDL-R on monocytes isolated from the patients revealed values of 6.7% in the patient with FHC and 71% in the patient with cirrhosis and hepatocellular carcinoma. The reverse transcriptase-polymerase chain reaction assay revealed the presence of gene expression for LDL-R. CONCLUSIONS: Domino transplantation can be efficiently used in a patient with marginal indications for transplantation using a liver from a patient with FHC. The slightly elevated serum cholesterol level in the recipient may be explained by the normal function of extrahepatic LDL-R.