Extrahepatic metastases from hepatocellular carcinoma: multimodality image evaluation.

The most prevalent primary malignancy of the liver is hepatocellular carcinoma (HCC); its poor prognosis is mainly related to intrahepatic recurrence and extrahepatic metastases. However, survival from HCC has improved due to better control of the primary tumor, the development of newer treatment modalities, including liver transplant, together with advances in imaging techniques. Therefore, the significance of patient management as corresponds with distant metastases has increased; since the proper evaluation and detection of extrahepatic metastases is crucial to optimize potential therapy for patients. Conventional imaging like CT, MRI play crucial rule in patient's diagnosis and qualifying for a certain type of therapy. More recently, a molecular imaging tool with radiolabeled deoxyglucose and fluorocholine has proved its promising value as a complementary tool to conventional studies. In this review, the frequent sites of metastases and HCC spread are discussed as well as the imaging findings as seen by both conventional imaging techniques and by molecular imaging tools, namely 18F-Choline PET/CT, and FDG PET. The implications of guiding treatment planning have also been discussed.

Gastric wall metastases from hepatocellular carcinoma: case report and review of the literature.

A 69-year-old male patient who had a history of well-differentiated hepatocellular carcinoma (HCC) post right hepatectomy presented a year later with iron-deficiency anemia. His anemia work-up included upper endoscopy that revealed multiple gastric polyp a biopsy from the largest demonstrated metastatic hepatocellular carcinoma. His magnetic resonance imaging (MRI) showed a gastric "polyp" without evidence of local HCC recurrence within the liver. His subsequent dual imaging with Choline/fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) confirmed the gastric metastases and in addition revealed other sites of unexpected metastatic disease in the right adrenal and the bone that was asymptomatic. Patient was started on sorafenib and currently he is alive one-and-half-year postdetection of his metastatic disease under palliative care. This case showed that the possibility of gastric metastases should be kept in mind when confronted with anemia in HCC patient and also highlight the complementary role of molecular imaging modality along with MRI in the metastatic work-up for hepatocellular carcinoma postcurative resection.

High prevalence of coronary artery calcification in Saudi patients with normal myocardial perfusion.

BACKGROUND: Normal single-photon emission computed tomography (SPECT) myocardial perfusion imaging (MPI) has a high negative predictive value for ischemic heart disease. Thus, the presence of subclinical coronary atherosclerosis detected by coronary artery calcification (CAC) score in patients who have under-gone SPECT MPI is unknown. OBJECTIVES: Determine the prevalence of coronary artery calcification (CAC) in patients with normal SPECT MPI and examine the association of CAC with conventional coronary artery disease (CAD) risk factors. DESIGN: Cross-sectional analytical study using medical records from February 2010 to April 2016. SETTINGS: Single tertiary-care center. PATIENTS AND METHODS: We studied patients referred from the outpatient clinical services for clinically indicated noninvasive CAD diagnosis with MPI SPECT. CAC scoring was subsequently performed within 3 months after a normal MPI. We excluded patients with chest pain or decompensated heart failure or patients with a history of CAD. The study population was divided into three groups: patients with a CAC score of 0, a CAC score from 1 to 300, and a CAC score more than 300. The groups were analyzed by age and other demographic and clinical characteristics. MAIN OUTCOME MEASURE(S): Prevalence of CAC in patients with normal MPI. RESULTS: The prevalence of CAC was 55% (n=114) in 207 patients with a mean (SD) age of 57.1 (10.4) years. Twelve percent had severe coronary atherosclerosis (CAC score > 300). All patients had a normal MPI SPECT. CAC scores were 0 for 93 patients (45%), 1 to 300 for 89 (43%), and more than 300 for 24 (12%). There was a strong association between CAC score and age (P < .0001), male sex (P < .0001), and diabetes mellitus (P=.042), but no association between CAC score and hypertension (P=.153), family history of CAD (P=.23), obesity (P=.31), hypercholesterolemia (P=.071), or smoking (P=.308). CONCLUSIONS: The prevalence of CAC is high in this study population of patients with normal SPECT MPI. Age, male sex and diabetes were risk factors associated with CAC. LIMITATIONS: Single center and small study population.

Clinical and biochemical features associated with BCS1L mutation.

Our study describes a novel phenotype in a series of nine Saudi patients with lactic acidosis, from four consanguineous families three of which are related. Detailed genetic studies including linkage, homozygosity mapping and targeted sequencing identified a causative mutation in the BCS1L gene. All affected members of the families have an identical mutation in this gene, mutations of which are recognized causes of Björnstad syndrome, GRACILE syndrome and a syndrome of neonatal tubulopathy, encephalopathy, and liver failure (MIM 606104) leading to isolated mitochondrial respiratory chain complex III deficiency. Here we report the appearance of a novel behavioral (five patients) and psychiatric (two patients) phenotype associated with a p.Gly129Arg BCS1L mutation, differing from the phenotype in a previously reported singleton patient with this mutation. The psychiatric symptoms emanated after childhood, initially as hypomania later evolving into intermittent psychosis. Neuroradiological findings included subtle white matter abnormalities, whilst muscle histopathology and respiratory chain studies confirmed respiratory chain dysfunction. The variable neuro-psychiatric manifestations and cortical visual dysfunction are most unusual and not reported associated with other BCS1L mutations. This report emphasizes the clinical heterogeneity associated with the mutation in BCS1L gene, even within the same family and we recommend that defects in this gene should be considered in the differential diagnosis of lactic acidosis with variable involvement of different organs.

Importance of adjusting dual-energy X-ray output for body size: an example from survivors of childhood acute lymphoblastic leukemia.

We compared DXA whole body and lumbar spine bone mineral density (BMD) using manufacturers software with a body size correction which derived bone mineral content (BMC) for bone area in survivors of acute lymphoblastic leukemia in Saudi Arabia (n = 51, mean age 13.5 y). With no corrections, 29 patients (57%) had lumbar spine BMD Z score < -1.0 and 21 (41%) had whole body BMD Z score < -2. After correction, only 6 (12%) had lumbar spine BMC Z score < -1.0 and 4 (8%) had whole body BMC Z score < -2. Agreement between the methods was "poor" by weighted κ analysis.

Diagnostic value of recombinant human thyrotropin-stimulated ¹²³I whole-body scintigraphy in the follow-up of patients with differentiated thyroid cancer.

PURPOSE: Published data on recombinant human thyrotropin- (rhTSH-) stimulated iodine-123 (¹²³I) diagnostic whole-body scintigraphy (DxWBS) in differentiated thyroid cancer (DTC) surveillance after initial treatment are limited. We sought to evaluate this modality's diagnostic value in this setting. MATERIALS AND METHODS: We retrospectively compared rhTSH-stimulated ¹²³I DxWBS results with DTC status concurrently determined by stimulated serum thyroglobulin (Tg) measurement, neck ultrasonography, and other imaging studies. Disease was considered present based on stimulated Tg level ≥1 µg/L without interfering Tg autoantibodies with or without positive imaging or biopsy-proven DTC. We also compared scan positivity and disease detection rates of rhTSH-stimulated DxWBS scans obtained with ¹²³I with those acquired with iodine-131 (¹³¹I) during the same period. The sample comprised 105 consecutive totally thyroidectomized patients undergoing rhTSH-aided DxWBS with I-123 (n = 67) or with ¹³¹I (n = 38) for diagnostic follow-up. rhTSH, 0.9 mg/d, was injected intramuscularly on 2 consecutive days. Oral diagnostic activities of 5 to 10 mCi (185-370 MBq) ¹²³I or 3 mCi (111 MBq) ¹³¹I were given on the third day. DxWBS was performed 24 hours (¹²³I) or 48 to 72 hours (¹³¹I) later. RESULTS: rhTSH-aided ¹²³I DxWBS scans showed 35.3% sensitivity, 98.0% specificity, 85.7% positive predictive value, and 81.6% negative predictive value. rhTSH-stimulated ¹²³I and ¹³¹I DxWBS did not differ in scan positivity (10.4% vs. 13.2%, P = 0.75) or disease detection rates (35.3% vs. 27.8%, P = 1.00). CONCLUSIONS: In DTC, rhTSH-aided ¹²³I DxWBS achieves comparable results in diagnostic follow-up with those of rhTSH-aided ¹³¹I DxWBS. Future studies should address the preablation setting and scan activity and timing.

Obesity and metabolic syndrome in adolescent survivors of standard risk childhood acute lymphoblastic leukemia in Saudi Arabia.

BACKGROUND: This study estimated prevalence of unhealthy weight status and metabolic syndrome (MS) amongst Saudi survivors of standard risk ALL. PROCEDURE: We recruited 56 survivors, mean age 13.4 years (SD 4.1), a mean of 9.1 years (SD 4.1) post-diagnosis. The BMI for age was used to define weight status relative to national (Saudi) and international (Cole et al., Cole-IOTF, WHO, and CDC) reference data. We measured body composition by dual-energy X-ray absorptiometry (DXA), waist circumference, blood pressure, lipid profile (HDL-C, Triglycerides), fasting glucose and insulin. RESULTS: According to international definitions based on BMI for age, around half of the sample had unhealthy weight status. All of the approaches based on BMI for age underestimated overfatness, present in 27/51 (53%) of the sample according to DXA. Prevalence of MS was 7.1% (3/42 of those over 9-years old) and 5.4% (3/56) by applying the International Diabetes Federation (IDF) definition and National Cholesterol Education Program Third Adult Treatment panel Guidelines (NCEP III), respectively. However, MS by the NCEP III definition was present in 19% of the overweight and obese survivors and 7.1% of the sample had at least two of the components of MS. CONCLUSION: Unhealthy body weight and overfatness may be common amongst adolescent Saudi survivors of standard risk ALL, though overweight and obesity may be no more common than in the general Saudi adolescent population. Defining weight status using BMI underestimates overfatness. Ideally, body composition and cardiometabolic risk factors should be monitored at late effects clinics.

Phenotypical spectrum of cerebellar ataxia associated with a novel mutation in the CA8 gene, encoding carbonic anhydrase (CA) VIII.

We define the neurological characteristics of familial cases from multiple branches of a large consanguineous family with cerebellar ataxia, mental retardation (MR), and dysequilibrium syndrome type 3 caused by a mutation in the recently cloned CA8 gene. The linkage analysis revealed a high logarithm of the odds (LOD) score region on 8q that harbors the CA8 in which a novel homozygous c.484G>A (p.G162R) mutation was identified in all seven affected members. The patients had variable cerebellar ataxia and mild cognitive impairment without quadrupedal gait. The brain MRI showed variable cerebellar volume loss and ill-defined peritrigonal white matter abnormalities. The Fluorodeoxyglucose Positron Emission Tomography (FDG PET) revealed hypometabolic cerebellar hemispheres, temporal lobes, and mesial cortex. This report expands the neurological and radiological phenotype associated with CA8 mutations. CA8 involvement should be considered in the differential diagnosis of other genetically unresolved autosomal recessive cerebellar ataxias.

The role of F-18-fluorodeoxyglucose positron emission tomography in the postoperative evaluation of differentiated thyroid cancer.

OBJECTIVES: The objective of the study was to compare F-18-fluorodeoxyglucose position emission tomography (FDG-PET) with diagnostic whole body scanning (DxWBS) and post-ablation radioiodine whole body scanning (TxWBS) and to assess its prognostic value in newly diagnosed differentiated thyroid cancer (DTC) patients, hypothesizing that FDG-PET is more likely to disclose locoregional and distant metastases. PATIENTS AND METHODS: DxWBS and FDG-PET scanning were performed in 26 newly diagnosed DTC patients who underwent thyroidectomy and TxWBS in 24 cases who had radioactive iodine ablation. The results of the FDG-PET scans were correlated with the stage of the disease and the long-term outcome of DTC. RESULTS: Overall, 18 FDG-PET scans (69.2%) were positive showing a total of 40 foci while 8 scans (30.8%) were negative. The corresponding 26 DxWBS were all positive and showed a total of 47 foci. DxWBS and TxWBS showed similar foci in the 24 patients who had ablation therapy. In contrast to the FDG-PET scans that showed uptake of 26 foci (65%) outside the thyroid bed, 45 foci (95.7%) on DxWBS were in the thyroid bed while 2 foci (4.3%) were in cervical lymph nodes and no focus was seen outside the neck area (P=0.000). There was a clear correlation between the FDG-PET results, the stage of the disease and long-term outcome; seven of the eight negative FDG-PET scans were in stage 1, while all patients with disease higher than stage 1 (six patients) had positive scans. Over a median of 30 months (10-48), seven out of eight patients (87.5%) with negative FDG-PET scans were in remission compared with only eight patients (44.4%) with positive FDG-PET (P=0.04). CONCLUSIONS: In the postoperative evaluation of DTC, compared with DxWBS and TxWBS, FDG-PET scans are more likely to reveal uptake outside the thyroid bed and to correlate with the stage of the disease and long-term outcome.

Role of [18F]fluorodeoxyglucose positron emission tomography in follow-up of differentiated thyroid cancer.

OBJECTIVE: To assess the diagnostic utility of [(18)F]flu-orodeoxyglucose positron emission tomography (FDG PET) in the follow-up of patients with differentiated thyroid cancer (DTC). METHODS: In this study, we used strict definitions of presence and absence of the disease and performed all FDG PET scans while the patients were not taking levothyroxine (LT4). We report the results of conventional FDG PET scans obtained during the follow-up of 50 nonselected patients with DTC (34 female and 16 male patients; median age, 40.5 years; range, 18 to 68). All FDG PET scans and measurement of thyroglobulin (Tg) levels were performed while the patients were not taking LT4 (thyrotropin>or=25 microIU/mL). Tg antibodies were negative in all patients. The initial surgical procedure was total thyroidectomy in all cases, and 26 patients underwent additional operations (2 to 4 procedures). Radioactive iodine (131I) therapy was given to 48 patients (median dose, 5,550 MBq). In 42 patients, FDG PET was used for evaluation of Tg-positive (Tg>2 ng/mL in the absence of LT4 therapy), scan-negative disease. In 8 patients, Tg was 2 ng/mL without LT4 therapy) for >1 year. Disease was considered absent if Tg was

Overview of exercise stress testing.

Exercise stress testing is a non-invasive, safe and affordable screening test for coronary artery disease (CAD), provided there is careful patient selection for better predictive value. Patients at moderate risk for CAD are best served with this kind of screening, with the exception of females during their reproductive period, when a high incidence of false positive results has been reported. Patients with a high pretest probability for CAD should undergo stress testing combined with cardiac imaging or cardiac catheterization directly. Data from the test, other than ECG changes, should be taken into consideration when interpreting the exercise stress test since it has a strong prognostic value, i.e. workload, heart rate rise and recovery and blood pressure changes. Only a low-level exercise stress test can be performed early post myocardial infarction (first week), and a full exercise test should be delayed 4 to 6 weeks post uncomplicated myocardial infarction. The ECG interpretation with myocardial perfusion imaging follows the same criteria, but the sensitivity is much lower and the specificity is high enough to overrule the imaging part.