Improved detection of hepatic metastases of adrenocortical cancer by contrast-enhanced ultrasound.

Adrenocortical carcinoma (ACC) is a rare and heterogeneous malignancy whose pathogenesis and poor prognosis is poorly understood. Computerized tomography (CT) and magnetic resonance imaging (MRI) are routinely performed for the imaging of the adrenal mass and for standard staging of the chest and abdomen as the lung and liver are the primary organs for metastasis in ACC. Contrast-enhanced ultrasound has been shown to have a high sensitivity and specifity for the differentiation of hepatic and neuroendocrine tumors. Twelve patients (7 women and 5 men; aged 24-74 years) with ACC were treated in our centre from 2004 to 2006. The patients received staging with a contrast-enhanced multislice spiral computed tomography (MSCT) as well as with a conventional and an echo-enhanced ultrasound of the liver. Contrast-enhanced ultrasound demonstrated liver metastases in 8 out of 12 patients (67%) and MSCT in 6 out of 12 patients (50%). In 2 out of 8 patients (25%) MSCT did not detect the liver metastases. Even in retrospective analysis with knowledge of the ultrasound results, the hepatic lesions were not recognized by the MSCT, but became detectable by MSCT at a later time point. All hepatic lesions diagnosed by MSCT were also seen by ultrasound. The detection of liver metastases by ultra-sound resulted in a change of therapy in two patients. The contrast-enhanced ultrasound has a high sensitivity in detecting the highly-vascularized liver metastases of ACC and should be included in the staging algorithm of ACC.

Osteoclast-like giant cell tumors of the pancreas and liver.

Osteoclast-like giant cell tumors (OGCT) are rare abdominal tumors, which mainly occur in the pancreas. The neoplasms are composed of two distinct cell populations and frequently show an inhomogenous appearance with cystic structures. However, due to the rarity of these tumors, only very limited clinical data are available. Imaging features and sonographic appearance have hardly been characterized. Here we report on two cases of osteoclast-like giant cell tumors, one located within the pancreas, the other within the liver, in which OGCTs are extremely rare. Both patients were investigated by contrast sonography, which demonstrated a complex, partly cystic and strongly vascularized tumor within the head of the pancreas in the first patient and a large, hypervascularized neoplasm with calcifications within the liver in the second patient. The liver OGCT responded well to a combination of carboplatin, etoposide and paclitaxel. With a combination of surgical resection, radiofrequency ablation and chemotherapy, the patient's survival is currently more than 15 mo, making him the longest survivor with an OGCT of the liver to date.

Hepatic steatosis in Dunnigan-type familial partial lipodystrophy.

OBJECTIVES: Characterization of familial clusters of subjects with metabolic derangements predisposing to hepatic steatosis and nonalcoholic steatohepatitis could facilitate genomic studies to identify risk factors for their development. Dunnigan-type familial partial lipodystrophy (FPLD) is an autosomal dominantly inherited disorder caused by mutations in the LMNA gene. Affected subjects have loss of subcutaneous fat from the extremities and symptoms similar to those characterizing the metabolic syndrome, including insulin resistance and dyslipidemia. The goal of this study was to determine the prevalence of steatosis in subjects with FPLD. METHODS: We examined 18 subjects from six families with FPLD for mutations in LMNA and analyzed plasma lipid and serum glucose concentrations. Liver ultrasound and serum aminotransferase activities were used as indicators of steatosis or steatohepatitis. In two subjects, histological examination of hepatic tissue was performed. RESULTS: All subjects had FPLD-causing mutations in LMNA. Plasma lipids were measured in 17 subjects, 16 of whom had hyperlipidemia and 14 presented with either documented insulin resistance or diabetes mellitus. Hepatic steatosis was present in 15 subjects who had ultrasound examinations and 9 of these had elevated serum aminotransferase activities. Liver biopsy confirmed steatosis in 2 subjects. CONCLUSIONS: Hepatic steatosis is part of the clinical phenotype of FPLD. This familial disorder may provide a human metabolic model system to facilitate genomic and environmental studies to determine risk factors for hepatic steatosis and nonalcoholic steatohepatitis.

Evaluation of criteria for the activity of Crohn's disease by power Doppler sonography.

BACKGROUND/AIM: In recent years, power Doppler sonography has been proposed as a method to assess disease activity in patients with Crohn's disease. The aim of this prospective study was to evaluate diagnostic criteria for power Doppler sonography by blinded comparison with ileocolonoscopy. METHODS: Twenty-two patients with confirmed Crohn's disease were prospectively investigated with B-mode and power Doppler sonography (HDI 5000, Philips Ultrasound) as well as ileocolonoscopy. Sonography was performed within 3 days before endoscopy. All procedures were performed by experienced examiners who were blinded to the clinical data and other results. Defined ultrasound parameters (bowel wall thickness, vascularization pattern) were used to determine a sonographic score of the activity. The degree of activity was scored from 1 (none) to 4 (high) by both ultrasound and ileocolonoscopy (pattern, extent of typical lesions). For each patient all segments of the colon and the terminal ileum were evaluated by both ultrasound and endoscopy. The weighted kappa test was used (StatXact software) for statistical analysis. RESULTS: In total, 126 bowel segments were evaluated by both ultrasound and endoscopy. The study showed a high concordance of power Doppler sonography and ileocolonoscopy (weighted kappa by region: sigmoid colon: 0.81; transverse colon: 0.78; ascending colon: 0.75; cecum: 0.84; terminal ileum: 0.82). Highest concordance was found in the descending colon (weighted kappa: 0.91; 95% CI: 0.83-0.98). CONCLUSIONS: Combination of B-mode and power Doppler sonography has a high accuracy in the determination of disease activity in Crohn's disease when compared to ileocolonoscopy. The diagnostic criteria established in this study can be useful for the evaluation of inflammatory bowel diseases by ultrasound.

Evaluation of diagnostic criteria for liver metastases of adenocarcinomas and neuroendocrine tumours at conventional ultrasound, unenhanced power Doppler sonography and echo-enhanced ultrasound.

PURPOSE: In order to improve the differential diagnosis between liver metastases of neuroendocrine tumours and adenocarcinomas, criteria for the masses at conventional ultrasound, unenhanced power Doppler sonography and echo-enhanced ultrasound were evaluated. METHODS: Seventy-three patients with histologically proven liver metastases of a neuroendocrine tumour (n = 26) or an adenocarcinoma (n = 47) were investigated by conventional ultrasound as well as unenhanced power Doppler sonography and echo-enhanced ultrasound focusing on specific properties of the lesions. RESULTS: Liver metastases of neuroendocrine tumours and adenocarcinomas showed a different contrast behaviour with echo-enhanced sonography. A hypervascularisation at the arterial and capillary phase were found in 85% of the neuroendocrine metastases, and in 17% of the masses of adenocarcinomas, respectively (p < 0.05). CONCLUSIONS: The successful treatment of liver metastases requires a highly sensitive and specific diagnostic procedure for their differentiation. A hypervascularisation of the lesions during the arterial and capillary phase at echo-enhanced ultrasound may point to a neuroendocrine primary tumour. However, histology is the only standard of reference for the differentiation of liver metastases, and is necessary for optimal therapy.

Differentiation of cystic pancreatic neoplasms and pseudocysts by conventional and echo-enhanced ultrasound.

BACKGROUND AND AIMS: Echo-enhanced sonography is a non-invasive and increasingly used procedure for the differentiation of pancreatic tumors. However, the diagnostic accuracy of this procedure compared to conventional ultrasound for the differential diagnosis of cystic pancreatic neoplasms from pseudocysts has never been investigated in a prospective study. METHODS: Thirty-one patients with a cystic pancreatic lesion at the conventional ultrasound (mean age 57 years, range 36-82 years) were included in the study. Sonography was performed by an experienced examiner who was unaware of the patients' clinical diagnosis. The exact diagnosis was based on histological evidence from biopsy examination (surgical or transabdominal fine needle biopsy for all cystic neoplasms and five pseudocysts), or a follow-up of at least 18 months (four pseudocysts). RESULTS: Of the 31 patients, 19 had cystadenomas, three had cystadenocarcinomas, and nine had pseudocysts. Only 27% of the cystadenomas and 67% of the pseudocysts could be correctly classified by conventional ultrasound. Conversely, 95% of the cystadenomas (P = 0.0001) and all pseudocysts were diagnosed correctly by echo-enhanced sonography. The sensitivity of echo-enhanced sonography with respect to diagnosing cystadenoma was 95% and its specificity was 92%. The corresponding values for pseudocysts were both 100%. CONCLUSION: Echo-enhanced sonography has a high sensitivity and specificity in the differential diagnosis of cystic pancreatic tumors. With this procedure the differentiation of cystadenomas and pseudocysts can be improved. However, histology is the standard of reference.

[Aneurysm of the superior mesenteric artery--be careful with the diagnosis of a pancreatic tumor]. / Aneurysma der oberen Mesenterialarterie--Vorsicht mit der Diagnose Pankreastumor.

HISTORY AND CLINICAL FINDINGS: A 42-year-old woman was admitted with blood pressure crisis. She complained of frontal headache. On clinical examination, a stenotic noise was found localized to the epigastrium. Some days earlier, a pancreatic "tumor" had been diagnosed sonographically by her general practitioner. INVESTIGATIONS: At B-mode sonography, a sharply delineated lesion at the pancreatic tail was found. With color Doppler sonography, blood flow could be detected within the "mass", which was interpreted as an aneurysm of the superior mesenteric artery. Furthermore, an aneurysmatic gastroduodenal artery was diagnosed. The sonographic findings were confirmed by CT scan, which additionally revealed an aneurysm of a segmental renal artery. COURSE: Given the danger of spontaneous rupture, the aneurysm of the superior mesenteric artery was resected. Under antihypertensive therapy, normal blood pressure values could be achieved, and the patient is symptom-free. CONCLUSION: Color and power Doppler sonography are useful in the differential diagnosis of pancreatic tumors. With these methods, erroneously suspected "tumors", e.g., an aneurysm of a mesenteric artery, can be differentiated from true lesions. Thus, complications of the aneurysm such as spontaneously rupture or intervention-associated bleeding can be prevented.

Improved diagnosing of small hepatocellular carcinomas by echo-enhanced power Doppler sonography in patients with cirrhosis.

BACKGROUND AND AIMS: Echo-enhanced power Doppler sonography is an increasingly used procedure for the differentiation of liver tumours. The aim of this prospective study was to investigate the accuracy of echo-enhanced power Doppler sonography in comparison with conventional ultrasound and fundamental power Doppler sonography in diagnosing hepatocellular carcinomas and regenerative nodules in patients with cirrhosis. SUBJECTS AND METHODS: Eighty-seven patients with cirrhosis and 90 liver tumours at conventional ultrasound were included in the study, selected from 103 consecutive patients with a mean age of 60 years (range 23-87 years) who presented to our department from January 1998 through January 2002. Sonography was performed by an experienced examiner, who was unaware of the clinical diagnosis. The exact diagnosis was based upon histological evidence from biopsy examination, laboratory results, and/or a follow-up of at least 18 months. RESULTS: There were 65 hepatocellular carcinomas, 21 regenerative nodules, and four metastases in the study group. Only 43% of the carcinomas (mainly nodules > 3 cm in diameter) could be classified correctly by conventional ultrasound or fundamental power Doppler sonography. However, 46% of the malign lesions (mainly nodules < 3 cm in diameter) were not differentiable. All non-differentiable tumours were classified correctly by echo-enhanced power Doppler sonography. The overall sensitivity of echo-enhanced power Doppler sonography with respect to diagnosing hepatocellular carcinoma was 89%; its specificity was 60%. The corresponding values for regenerative nodules were 71% and 100%, respectively. CONCLUSIONS: Only hepatocellular carcinomas greater than 3 cm in size can be diagnosed with a high accuracy by conventional ultrasound or fundamental power Doppler sonography. However, small malignant lesions in cirrhotic livers are often not differentiable with these techniques. With echo-enhanced power Doppler sonography, the differentiation of small hepatocellular carcinomas can be improved. However, histology is the standard of reference.