18F-FDG activitiy PET/CT and CA-19.9 levels for the prediction of histopathological features and localization of peri- ampullary tumors.

BACKGROUND/AIMS: We sought to investigate the roles of maximum standardized uptake value (SUVmax) and serum carbohydrate antigen 19-9 (CA 19-9) in predicting the histopathological features of periampullary tumors. MATERIALS AND METHODS: Thirty-four patients with histologically confirmed periampullary tumors were classified into two groups, according to the localizations of their tumors (ampulla Vateri or pancreas). SUVmax was obtained from [(18)F]-fluorodeoxyglucose positron emission tomography computed tomography (18F-FDG PET/CT). SUVmax and CA 19-9 levels were measured and compared with histopathological features of the tumors. Logistic regression was used to assess the significance and independence of predictive factors. RESULTS: 18F-FDG PET/CT SUVmax (<2.5 vs. ≥2.5; p=0.031) and CA 19-9 level (normal vs. elevated; p=0.045) were significantly and independently predictive of the histopathological origin of the tumors (ampulla Vateri vs. pancreas). The ratio of CA 19-9 levels and SUVmax were found to be higher in cases of poorly differentiated tumors and tumors greater than 2 cm in diameter. CONCLUSION: A surgical approach to treatment may be considered for patients who have both i) an established or suspected diagnosis of periampullary tumors and ii) low SUVmax and CA 19-9 levels.

A case of uneventful ABO-incompatible liver transplantation from a deceased donor managed with routine immunosuppressive treatment.

ABO-incompatible liver transplantation (ILT) was formerly contraindicated because of the increased risk of antibody-mediated humoral graft rejection due to preformed anti-A/-B antibodies on recipient endothelial cells. A 2.5-year-old girl with end-stage liver disease underwent cadaveric donation ILT because of acute liver failure and esophageal variceal bleeding before transplantation. The patient's blood type was A Rh (-) and the donor's blood type B Rh (+). The operation and postoperative course were uneventful. The immunosuppression consisted of steroids, and tacrolimus was initiated on the day of the surgery. The patient's hemoglobin level did not change, and direct Coombs test performed daily was consistently negative. Anti-B titer was observed at a maximum of 1/8. The patient was followed up during the first year. This case of ILT from a cadaveric donor is significant because the 2.5-year-old recipient did not experience any complications after undergoing routine immunosuppressive treatment.

A rare cause of bile duct obstruction in adolescence: Neuroendocrine tumor.

Extrahepatic bile duct is one of the rare places for neuroendocrine tumors which comprise 0.2-2% off all neuroendocrine tumors of gastrointestinal tract. The aim of this paper is to report a case of a 16-year-old boy with a neuroendocrine tumor of extra hepatic bile duct. Laboratory and imaging findings is supported obstructive jaundice. After a pre-operative detailed evaluation, the common bile and common hepatic duct with gallbladder were resected and Roux-en-Y hepaticojejunostomy was performed. After a 36 months follow up, no locoregional recurrence or metastatic disease was observed. Pre-operative diagnosis of neuroendocrine tumors are extremely difficult and often confused with cholangiocarcinoma. Treatment modality of this rare entity depends on the location of the tumor and aggressive surgery is still the best chocie of treatment. It should be kept in mind that disease-free survival for patients with neuroendocrine tumor depends on success of surgery.

Neurofibromatosis type 1, gastrointestinal stromal tumor, leiomyosarcoma and osteosarcoma: four cases of rare tumors and a review of the literature.

BACKGROUND: Neurofibromatosis type 1 (NF1) is a genetic syndrome that predisposes patients to benign and malignant tumor development. Patients with NF1 develop multiple neurofibromas that can transform into aggressive sarcomas known as malignant peripheral nerve sheath tumors. In contrast, malignant tumors unrelated to the nervous system rarely coexist with neurofibromatosis. The aim of this article was to present four cases of adult NF1 patients with malignant tumors unrelated to the nervous system as well as a bibliographic search for papers describing these tumors in NF1, focusing on osteosarcomas, gastrointestinal stromal tumors (GISTs), leiomyosarcomas and somatostatinomas and their genetic alterations in NF1. METHODS: Search engines such as PubMed and MEDLINE were browsed for English-language articles since 1989 using a list of keywords, as well as references from review articles. Search terms were NF1, osteosarcoma, leiomyosarcoma, somatostatinoma and GIST. Data were summarized in a table at the end of the Results section. RESULTS: In our four NF1 cases, there were one osteosarcoma, one leiomyosarcoma, one somatostatinoma and GIST and one GIST. NF1 was diagnosed at an adult age when these patients were admitted to our oncology department. The results generated by the literature search yielded 75 articles about NF and GIST. We summarized the clinical characteristics of 43 patients with NF1 and somatostatinoma. Forty-five articles involving NF and osteosarcoma were found, and of these, 26 involved NF1; from these articles, we identified the clinical features of 8 patients. Twenty-five articles were found concerning NF1 and leiomyosarcoma, and of those, we summarized the clinical features of 15 patients. CONCLUSIONS: Here we reviewed somatostatinomas, GISTs, osteosarcomas and leiomyosarcomas occurring in NF1 patients. Patients with NF1 who present with gastrointestinal symptoms, should be carefully evaluated carefully with a high index of suspicion of potential GISTs, periampullary and duodenal tumors. Patients with pathological fractures or bone pain along with NF1 should be carefully screened for malignant bone tumors. Patients with NF1 can develop leiomyosarcoma less frequently than other malignancies, but the association of uterine leiomyoma and NF1 may not be fortuitous. Somatic mutations were defined for frequent tumors, including neurogenic tumors and GISTs but not for sarcomas due to the complexity of underlying mechanisms of the disease and tumorigenesis. Based on the findings; all NF patients can develop malignant tumors, including the less frequently observed ones. Therefore, we recommend that new genetic studies should be performed for rare malignancies in cases of NF1.

Primary hepatic neuroendocrine tumor: Five cases with different preoperative diagnoses.

Neuroendocrine tumors, also known as carcinoid tumors, behave like benign tumors; however, they show the characteristics of carcinoma. While more than 80% of the neuroendocrine tumors found in the liver are metastatic, primary hepatic neuroendocrine tumors are very rare. Five patients with hepatic mass who admitted to our clinic between August 2003 and July 2007 were treated surgically. Ultrasonography, computerized tomography and magnetic resonance imaging were performed in all patients. Endoscopy and colonoscopy were conducted to exclude malignancy of other sites. Hepatectomy was carried out in all patients. Diagnosis was confirmed with immunohistochemical examination. The five patients treated surgically were diagnosed as primary hepatic neuroendocrine tumor histopathologically. Abdominal pain was the most common complaint of all patients. Hepatectomy was conducted in all patients due to tumors originating from the liver lobes. Only one patient (Case 2) underwent transarterial chemoembolization before hepatectomy to reduce tumor bleeding. Owing to tumor recurrence on the left lobe of the liver in Case 2, transarterial chemoembolization was performed four years after hepatectomy. R0 resection was achieved in two patients (Cases 1 and 3). In conclusion, primary hepatic neuroendocrine tumors are very rare and asymptomatic tumors. Thus, high-sensitive laboratory and imaging examinations are required. At present, hepatectomy remains the main treatment for primary hepatic neuroendocrine tumor.

G-308A TNF-alpha polymorphism is associated with an increased risk of hepatocellular carcinoma in the Turkish population: case-control study.

BACKGROUND: Tumor necrosis factor-alpha (TNF-alpha) is a pro-inflammatory cytokine that may act as an endogenous tumor promoter. A genetic polymorphism of TNF-alpha gene at position -308 promoter region is involved in the regulation of expression level and has been found to be associated with susceptibility to various types of cancer. METHODS: To determine the association of the TNF-alpha gene G-308A polymorphism on the risk of hepatocellular carcinoma (HCC) in a Turkish population, a hospital-based case-control study was designed consisting of 110 diagnosis subjects with hepatocellular carcinoma and 110 cancer-free control subjects matched on age, gender, smoking and alcohol status. The genotype frequency of this polymorphism was determined by using a polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) assay. RESULTS: The distribution G-308A genotype was significantly associated with the risk of HCC (p<0.001, odds ratio [OR]=4.75, 95% confidence interval [CI]=2.25-9.82 for -308 AA/GA genotypes versus GG genotype). CONCLUSION: We suggested that the presence of the high producer allele -308A in the TNF-alpha gene appears to be associated with an increased risk for the development of HCC in Turkish population.

Renal function in patients with orthotopic liver transplantation.

Due to multiple reasons, acute renal failure (ARF) commonly develops in the early postoperative period of orthotopic liver transplantation (OLT) recipients. The records of OLT recipients between 1999 and 2004 were evaluated. Age, gender, primary disease, history of diabetes, immunosuppressive drugs, pre- and postoperative renal function tests, serum electrolytes, dialysis, liver functions tests, and renal function tests in follow-up period were noted. We followed 16 patients with OLT in our center. ARF developed in 8 patients. Dialysis was performed in only 2 patients, and other patients with ARF were managed with conservative measures. Hypertensive crisis and cerebrovascular stroke developed in 1 diabetic hypertensive patient.

Surgical approach to symptomatic giant cavernous hemangioma of the liver.

BACKGROUND/AIMS: Surgical treatment of giant cavernous hemangioma of the liver is still controversial. In this study, indications and results of surgical therapy were evaluated. METHODOLOGY: Fifteen patients with symptomatic giant cavernous hemangioma of the liver were treated by enucleation or liver resection. RESULTS: The surgical indications were abdominal pain in 11 patients, uncertain diagnosis in 3 patients and tumor enlargement in one patient. The median tumor size was 12.5cm (range, 6-30cm). Eleven patients underwent enucleation procedure while the other 4 patients underwent resection procedures. Complications occurred in 2 (13.4%) patients. The patient with the largest tumor underwent right extended lobectomy and died of bleeding and coagulopathy (6.7%). The postoperative hospital stay was 7 days (range, 4-16 days). Thirteen patients were followed-up for an average period of 32.8 months (range, 6-88 months). It was found that the symptoms for 12 of 13 patients disappeared. During the postoperative controls carried out by imaging procedures, no recurrences were observed. CONCLUSIONS: Abdominal pain, uncertain diagnosis and enlargement are major surgical indications of symptomatic giant cavernous hemangiomas. Most of the symptoms disappear after the surgical treatment. Enucleation can be successively performed with low morbidity rates in most of the patients and recurrences are rare. If the tumor location precludes safe enucleation, anatomic resections are preferred.

Helicobacter pylori in the etiology of cholesterol gallstones.

GOALS: We aimed to investigate the presence of bacterial structures in cholesterol gallstones and particularly presence of Helicobacter spp/H. pylori in gallstones by microbiologic cultivation, histopathologic staining, and polymerase chain reaction (PCR). BACKGROUND: Many studies suggest that different mechanisms are responsible for the formation of pigmented gallstones and cholesterol gallstones. Recently, studies showed that infection could have an important role in the formation of cholesterol gallbladder stones. STUDY: We examined 77 mixed cholesterol gallstones. After cholecystectomy, gallbladder cultures were done for H. pylori and other bacterium. Gallbladder has also been examined by three histopathologic staining methods (Warthin-Starry, hematoxylin eosin, and gram staining) for Helicobacter spp. In addition, 16S rRNA-PCR amplification was performed for Helicobacter spp in gallstones. Twenty postmortem gallbladders without gallstones were investigated by the same histopathologic and PCR methods for Helicobacter spp. as a control group. RESULTS: Different bacterium were isolated from 22 gallbladder samples (12 Escherichia coli, 8 Pseudomonas, and 2 clostridium) and H. pylori was isolated in 6 gallbladder samples. Helicobacter spp was found in 7 gallstones by PCR amplification. Helicobacter-like organisms were demonstrated in 18 samples by three different histopathologic methods. Helicobacter-like organisms were also found in five samples by the same histopathologic methods (Warthin-Starry, hematoxylin-eosin, and gram staining). Only four samples were found positive for Helicobacter spp/H. pylori by all methods. CONCLUSIONS: Bacterial population including H. pylori could have a possible role in the formation of cholesterol gallstones.