Multidisciplinary Oncovascular Surgery is Safe and Effective in the Treatment of Intra-abdominal and Retroperitoneal Sarcomas: A Retrospective Single Centre Cohort Study and a Comprehensive Literature Review.

OBJECTIVE: Radical excision of retroperitoneal or intra-abdominal soft tissue sarcomas may necessitate vessel resection and reconstruction. The aim of this study was to assess surgical results of retroperitoneal or intra-abdominal sarcomas involving major blood vessels. METHODS: This was a retrospective single centre cohort study and a comprehensive review of literature. Patients with retroperitoneal or intra-abdominal sarcomas treated by the oncovascular team in Helsinki University Hospital from 2010 to 2018 were reviewed for vascular and oncological outcomes. A comprehensive literature review of vascular reconstructions in patients with retroperitoneal sarcoma was performed. RESULTS: Vascular reconstruction was performed in 17 patients, 11 of whom required arterial reconstructions. Sixteen of the operations were sarcoma resections; the post-operative diagnosis for one patient was thrombosis instead of the presumed recurrent leiomyosarcoma. Early graft thrombosis occurred in two venous and one arterial reconstruction. Late thrombosis was detected in three (18%). The median follow up was 27 (range 0-82) months. Of the patients with sarcoma resections 5 (31%) died of sarcoma and further 4 (25%) developed local recurrence or new distant metastases. The comprehensive review of literature identified 37 articles with 110 patients, 89 of whom had inferior vena cava reconstruction only. Eight arterial reconstructions were described. Late graft thrombosis occurred in 14%. The follow up was 0-181 months, during which 57% remained disease free and 7% died of sarcoma. CONCLUSION: Vascular reconstructions enable radical resection of retroperitoneal and intra-abdominal sarcomas in patients with advanced disease. The complex operations are associated with an acceptable rate of serious peri-operative complications and symptomatic thrombosis of the repaired vessel is rare. However, further studies are needed to assess the performance of the vascular reconstructions in the long term.

[Chronic liver disease and thrombosis risk]. / Krooninen maksasairaus lisää tromboosiriskiä.

The coagulopathy of chronic liver disease involves elevated risks for thrombosis in the portal vein and extra-splanchic sites. Hypercoagulability may moreover accelerate liver fibrosis progression. Cirrhosis-related portal vein thrombosis is associated with decompensation events and inferior prognosis; anticoagulation therapy achieves complete recanalization in -40% of recent thromboses and prevents thrombosis progression in chronic cases. Standard thrombosis prophylaxis seems appropriate for hospitalized cirrhotic patients. This review provides practical guidance to tailoring anticoagulation therapy in cirrhosis according to individual bleeding risk. We also propose an algorithm for using anticoagulation and transjugular intrahepatic portosystemic shunts in the treatment of cirrhotic portal vein thrombosis.

Biliary dysplasia in patients with primary sclerosing cholangitis: additional value of DNA ploidity.

BACKGROUND: Detection of biliary dysplasia in PSC is essential for proper timing of liver transplantation to prevent the development of cholangiocancer, which is considered a contraindication for liver transplantation in most centres. In patients with PSC, differential diagnosis of benign, premalignant and malignant biliary strictures is difficult. AIMS: This prospective study aimed to evaluate the role of DNA analysis in combination with brush cytology, scored ERCP findings, and tumour markers to detect hepatobiliary dysplasia and malignancy. MATERIAL AND METHODS: Brush samples for cytology and for evaluation of DNA content analysed with flow cytometry came from 102 consecutive PSC patients referred for ERCP. Symptoms, serum Ca19-9 and CEA were determined at the time of index biliary examination. ERCP findings were scored for intra- and extrahepatic changes. The end-points were liver transplantation or diagnosis of malignancy or dysplasia. RESULTS: Most of the patients were asymptomatic at the time of ERCP: 73% had no symptoms, and 12% had only mild symptoms. An aneuploid DNA content was evident in 20 (20%) patients, and cells suspected for malignancy in 22 (21%). Seven patients had both aneuploidity and cytology (7%) suspicious for malignancy. An end-point, diagnosis of malignancy or liver transplantation was achieved in 42 patients. Combining DNA ploidity and cytology in patients at the end-point, sensitivity was 72%, specificity 82%, positive predictive value 86% and negative predictive value 67%. DISCUSSION AND CONCLUSION: In this mostly asymptomatic PSC-patient population, 33% demonstrated abnormal brush cytology or aneuploidity. Determining DNA ploidy and brush cytology during ERCP offers a useful tool for identifying those PSC patients who are at high risk of developing cholangiocancer.

Radiation exposure during nasojejunal intubation for MRI enteroclysis.

BACKGROUND: Patients with Crohn's disease are often investigated using MRI enteroclysis which may provide better visual quality than MRI enterography, but exposes patients to radiation. Only few data exist of the radiation dose used in fluoroscopy prior to MRI enteroclysis. SUBJECTS AND METHODS: During the 12-month study period, all 95 patients (40 men) undergoing MRI enteroclysis with nasojejunal intubation using fluoroscopy for suspicion or evaluation of Crohn's disease were included. Average age at the time of MRI was 40.1 years (range 17-79). Conversion factors from dose-area product to effective dose were determined with a Monte Carlo-based software PCXMC. The conversion factors were determined for a standard-sized adult phantom for posterior-anterior and right-posterior-oblique projections. RESULTS: The average total time of fluoroscopy was 3 min 17 s (range 0 min 7 s to 31 min). The average effective dose of ionizing radiation was 0.21 mSv (range 0.01-2.67). The average dose is equivalent to 10 PA chest x-rays. Standard deviation was 0.41 mSv. The highest effective dose of a single patient was 2.67 mSv. In comparison, a standard abdominal CT scan causes an effective dose of 12 mSv. CONCLUSIONS: The effective dose of ionizing radiation with nasojejunal intubation is relatively small in the majority of patients. When repeated imaging is necessary, it seems advisable to consider imaging techniques, which do not subject patients to ionizing radiation. Also if a previous nasojejunal intubation has been difficult, a different imaging technique is recommended.

[Liver disease and hemostasis--evaluation of bleeding risk]. / Maksan vajaatoiminta ja hemostaasi--verenvuotovaaran arviointi.

In severe liver disease, simultaneous abnormalities in procoagulant and anticoagulant pathways seem to maintain the hemostatic balance, provided that the platelet level is sufficient. Common coagulation screening tests such as INR fail to measure the concomitant anticoagulant deficiencies and fibrinolytic abnormalities, and do not predict bleeding in patients with compensated liver disease undergoing invasive procedures. Thus, specific INR cut-off levels and prophylactic use of fresh-frozen plasma are discouraged. Volume expansion, hemodynamic disruption, endothelial dysfunction, and infections increase the bleeding risk. Individualized bleeding risk assessment mandates evaluation of the patient's clinical condition and a comprehensive assessment of the hemostatic system.

Humanin vivoliver and tumor bioimpedance measured with biopsy needle.

Objective.Liver biopsy is an essential procedure in cancer diagnostics but targeting the biopsy to the actual tumor tissue is challenging. Aim of this study was to evaluate the clinical feasibility of a novel bioimpedance biopsy needle system in liver biopsy and simultaneously to gatherin vivobioimpedance data from human liver and tumor tissues.Approach.We measured human liver and tumor impedance datain vivofrom 26 patients who underwent diagnostic ultrasound-guided liver biopsy. Our novel 18 G core biopsy needle tip forms a bipolar electrode that was used to measure bioimpedance during the biopsy in real-time with frequencies from 1 kHz to 349 kHz. The needle tip location was determined by ultrasound. Also, the sampled tissue type was determined histologically.Main results.The bioimpedance values showed substantial variation between individual cases, and liver and tumor data overlapped each other. However, Mann-Whitney U test showed that the median bioimpedance values of liver and tumor tissue are significantly (p < 0.05) different concerning the impedance magnitude at frequencies below 25 kHz and the phase angle at frequencies below 3 kHz and above 30 kHz.Significance.This study uniquely employed a real-time bioimpedance biopsy needle in clinical liver biopsies and reported the measured humanin vivoliver and tumor impedance data. Impedance is always device-dependent and therefore not directly comparable to measurements with other devices. Although the variation in tumor types prevented coherent tumor identification, our study provides preliminary evidence that tumor tissue differs from liver tissuein vivo,and this association is frequency-dependent.

[Prolonged abdominal discomfort, ascites and pleural fluid]. / Pulmallinen vatsavaiva, askites ja pleuraneste.

A 67-year-old man had been examined for 1.5 years for hypersedimentation, abdominal discomfort and pleural effusion, but the cause had remained unclear. With progressive weight loss the patient was admitted to the ward for examinations, whereupon the diagnosis of a rare disease began to unravel.

[Interventional radiology of the liver and bile ducts]. / Maksan ja sappiteiden toimenpideradiologia.

Procedures of the liver and bile ducts performed under imaging guidance are directed to hepatic blood vessels, bile ducts or alterations of the liver parenchyma. Most of them are associated with the treatment of malignant tumors or complications caused by them. Embolization can be applied to treat tumors and hemorrhages caused by traumas or tumors. Vascular procedures are also applied to the treatment of portal hypertension as well as occlusions of the hepatic and portal veins. Tissue specimens of the liver and its focal changes, effusions associated with bile ducts, and intrahepatic abscesses are most commonly also channeled under imaging guidance.

The natural history of Shwachman-Diamond syndrome-associated liver disease from childhood to adulthood.

OBJECTIVES: In order to characterize the natural course of Shwachman-Diamond syndrome (SDS)-associated hepatopathy we evaluated liver biochemistry and imaging findings, and their evolution with age, in patients with SDS and verified SBDS mutations. STUDY DESIGN: Retrospective and cross-sectional liver imaging, biochemical and histologic data of 12 patients (age range 2.1 to 37 years) with SBDS mutations were analyzed. Hepatic volume and parenchymal structure were determined from magnetic resonance imaging data. RESULTS: Hepatomegaly and aminotransaminase elevation was observed in most of the patients with SDS at an early age; values normalized by age 5 years and remained normal over extended follow-up. Mild to moderate serum bile acid elevation was noted in 7 patients (58%). On magnetic resonance imaging, no patients (n = 11) had evidence of hepatic steatosis, cirrhosis, or fibrosis. Three middle-aged patients had hepatic microcysts. CONCLUSIONS: SDS-associated hepatopathy has overall good prognosis. No major hepatic abnormalities developed during extended follow-up to adulthood. Mild cholestasis in follow-up even after normalization of transaminase levels may reflect primary alterations in liver metabolism in SDS.

Preoperative hepatic 3D models: virtual liver resection using three-dimensional imaging technique.

Emerging new techniques for liver resections set new requirements for the preoperative imaging and planning. Open surgery is a three-dimensional procedure and planning of the resection line may be difficult when basing on conventional two-dimensional CTs or MRIs, although all the information is there. With multidetector-row CT (MDCT), thin slices can be obtained with excellent temporal resolution, and precise three-dimensional (3D) models can be created. We regard 3D imaging technique useful in most liver resections. It improves the surgeon's knowledge of liver anatomy and makes even more complicated liver resections safe. Better knowledge of three-dimensional appearances of liver structures may further improve the results of curative liver surgery. However, before becoming a routine clinical procedure, research and development are still needed. Also, careful testing and evaluation of the methods have to be performed. In the future, 3D models will probably play an important role in the preoperative planning of liver resections.

Spontaneous rupture of a hepatic cystadenoma and cystadenocarcinoma: report of two cases.

Hepatobiliary cystadenomas and cystadenocarcinomas are rare tumors. Differentiating between these tumors and benign hepatic cysts may be difficult. Because of their rarity, diagnosis is often delayed and may result in inaccurate treatment, resulting in unnecessary morbidity and mortality. The purpose of this report is to draw attention to these entities and their complications. We report on two cases with spontaneous rupture of hepatobiliary cystadenoma and cystadenocarcinoma cysts, initially treated as simple hepatic cysts by aspiration, or by aspiration combined with sclerotherapy. The spontaneous rupture of the cysts appeared years after the initial treatment of the cysts, leading in one case to a prolonged stay in an intensive care unit. In both cases, a formal liver resection was carried out and microscopic investigations revealed a mucinous cystadenocarcinoma and cystadenoma. In conclusion, although hepatobiliary cystadenomas and cystadenocarcinomas are rare findings, they should not be forgotten in the diagnostic workshop when examining patients with hepatic cysts. If hepatobiliary cystadenomas and cystadenocarcinomas cannot be excluded following radiological imaging, surgery is recommended.

Non-invasive diagnosis of liver cirrhosis: magnetic resonance imaging presents special features.

OBJECTIVE: Liver cirrhosis and its complications constitute a daily clinical challenge. The diagnosis of cirrhosis is traditionally established with the invasive method of liver biopsy. We undertook the present study in order to investigate the sensitivity and specificity of magnetic resonance imaging (MRI) in diagnosing cirrhosis. MATERIAL AND METHODS: A total of 56 patients were included in our retrospective study. The liver cirrhosis group comprised 30 patients and the control group 26 patients. All cases were histologically verified. A 1.5T MRI unit was used. Twelve radiological features of cirrhosis (enlargement of segment one, narrowing of hepatic veins, enlargement of spleen, fibrosis, nodular liver surface, ascites, regenerative nodules, enlargement of hilar periportal space, atrophy of right lobe, portosystemic collaterals, expanded gallbladder fossa, iron depositions) and tumour-load were evaluated by consensus reading. The ratio between segment one and right lobe was measured and the livers were classified as cirrhotic or non-cirrhotic. The chi2-test and t-test were used to indicate statistical difference. RESULTS: The sensitivity of MRI in diagnosing liver cirrhosis was 87% and the specificity 92%. The most characteristic MRI features were enlargement of segment one (83%), narrowing of hepatic veins (83%), signs of portal hypertension (77%), fibrosis (77%), and nodular liver margin (67%). There were statistical differences in all MRI signs between the cirrhosis and control groups. The ratio between segment one and right lobe also showed statistical significance between the two groups. All occult hepatocellular carcinomas were detected in the cirrhosis group. CONCLUSION: MRI has high sensitivity and specificity in the diagnosis of liver cirrhosis.

Magnetic resonance imaging of the liver: true fast imaging with steady state free precession sequence facilitates rapid and reliable distinction between hepatic hemangiomas and liver malignancies.

OBJECTIVE: To assess the capability of the true fast imaging with steady state free precession (true FISP) sequence in the distinction between hemangiomas and malignant liver lesions. METHODS: Sixty-eight patients with 45 hemangiomas and 51 liver malignancies were included in this study. A 1.5-T magnetic resonance system and a phased-array body coil were used. In addition to true FISP, breath-hold and fat-suppressed, T2-weighted, half-Fourier single-shot turbo spin echo (HASTE) and both unenhanced and gadolinium (Gd)-enhanced T1-weighted sequences were acquired. Two radiologists evaluated the magnetic resonance images independently in a blinded fashion. Interobserver variations with true FISP and HASTE were determined. Lesion contrast-to-noise ratios were calculated from true FISP images. RESULTS: With true FISP, readers 1 and 2 made a correct distinction between hemangiomas and liver malignancies in 43 of 45 (96%) cases and 40 of 45 (89%) cases, respectively. The kappa value was 0.65. With HASTE, the success rates were 40 of 45 cases (89%) and 36 of 45 cases (80%), respectively, and the kappa value was 0.33. With a Gd-enhanced T1-weighted sequence, the correct classifications were 35 of 45 cases (78%) and 37 of 45 cases (82%), respectively. All hemangiomas appeared as bright and well-demarcated lesions on true FISP images. Malignant liver foci were heterogeneous with unsharp margins and nearly isointense relative to liver. The specificities of true FISP in lesion differentiation were 100% and 98% for readers 1 and 2, respectively. The mean contrast to noise ratio value of hemangiomas was 21.2 (standard deviation [SD] = 9.2), and that of malignant lesions was 4.9 (SD = 3.9). This difference was highly significant (P < 0.0001). CONCLUSION: Noninvasive, rapid, and reliable differentiation between hemangiomas and malignant liver lesions is possible by using the true FISP sequence.