Inter- and Intraobserver Agreement in Measuring Urolithiasis Density on Nonenhanced Computed Tomography.

Introduction: Treatment choice for urolithiasis is partially based on measuring stone density in HU on nonenhanced computed tomography (NECT). Interobserver variability in these measurements could have treatment consequences. This study aims to assess the observer agreement of measuring HU and whether the use of a protocol leads to a better agreement. Materials and Methods: We retrospectively included 155 consecutive NECTs of patients with stones ≥4 mm. Five observers (two radiologists, one urologist, one urology resident, and one radiology resident) assessed all anonymized NECTs four times in randomized order. HU was measured without instruction (rounds 1 and 2) and subsequently using two protocols (A and B, rounds 3 and 4). Protocols comprised using bone setting, zoom, and measuring HU without the penumbra, in either three (A) or one (B) axial plane. The inter- and intraobserver agreement was evaluated using the intraclass correlation coefficient (ICC). Results: Interobserver agreement on HU measurement without protocol was as follows: ICC = 0.84 (confidence interval [CI]: 0.79-0.87). Agreement diminished with protocol A, ICC = 0.62 (CI: 0.37-0.76), and improved with protocol B, ICC = 0.90 (CI: 0.86-0.92). Intraobserver agreement without protocol was ICC = 0.87, with protocol A, ICC = 0.87, and with protocol B, ICC = 0.93. The biggest improvement was seen for urologists' agreement from no protocol to protocol B, where ICC improved from 0.81 (CI: 0.70-0.87) to 0.91 (CI: 0.84-0.94). Conclusions: Observer agreement of HU measurement of urolithiasis without protocol is already good but using zoom, bone setting, and measuring in a representative plane is recommended. This protocol results in higher agreement, especially among urologists. Measuring in three axial planes does not increase agreement.

Renal imaging in 199 Dutch patients with Birt-Hogg-Dubé syndrome: Screening compliance and outcome.

Birt-Hogg-Dubé syndrome is associated with an increased risk for renal cell carcinoma. Surveillance is recommended, but the optimal imaging method and screening interval remain to be defined. The main aim of our study was to evaluate the outcomes of RCC surveillance to get insight in the safety of annual US in these patients. Surveillance data and medical records of 199 patients with Birt-Hogg-Dubé syndrome were collected retrospectively using medical files and a questionnaire. These patients were diagnosed in two Dutch hospitals and data were collected until June 2014. A first screening for renal cell carcinoma was performed in 172/199 patients (86%). Follow-up data were available from 121 patients. The mean follow-up period per patient was 4.2 years. Of the patients known to be under surveillance, 83% was screened at least annually and 94% at least every two years. Thirty-eight renal cell carcinomas had occurred in 23 patients. The mean age at diagnosis of the first tumour was 51. Eighteen tumours were visualized by ultrasound. Nine small tumours (7-27 mm) were visible on MRI or CT and not detected using ultrasound. Our data indicate that compliance to renal screening is relatively high. Furthermore, ultrasound might be a sensitive, cheap and widely available alternative for MRI or part of the MRIs for detecting clinically relevant renal tumours in BHD patients,but the limitations should be considered carefully. Data from larger cohorts are necessary to confirm these observations.

Differences in patterns of survival in metastatic adenoid cystic carcinoma of the head and neck.

BACKGROUND: We examined the assumption in conventional teaching about metastatic adenoid cystic carcinoma (ACC) being an indolent type of disease. METHODS: A single center analysis of 105 cases of ACC was performed. Radiographs were reviewed and tumor response to chemotherapy was measured. Distant disease-free survival (DDFS) and time to death since distant metastases diagnosis were analyzed. RESULTS: Forty-two percent of the patients were diagnosed with distant metastases. DDFS showed significant negative associations with advanced T classification, N+ classification, solid type tumor, and positive surgical margins. Distant metastases (91%) developed in the first 5 years after presentation. Median distant metastatic survival was 13.8 months. The most frequent organ sited was the lung. Solid type ACC showed a preponderance for multiorgan metastases (17/28; 61%). Distant metastases seemed not to occur in case of clear surgical margins. Solid type ACC had a significant poorer survival after development of distant metastases. CONCLUSION: Metastatic ACC is not always an indolent disease. © 2016 Wiley Periodicals, Inc. Head Neck 39: 456-463, 2017.

Are lung cysts in renal cell cancer (RCC) patients an indication for FLCN mutation analysis?

Renal cell cancer (RCC) represents 2-3% of all cancers and is the most lethal of the urologic malignancies, in a minority of cases caused by a genetic predisposition. Birt-Hogg-Dubé syndrome (BHD) is one of the hereditary renal cancer syndromes. As the histological subtype and clinical presentation in BHD are highly variable, this syndrome is easily missed. Lung cysts--mainly under the main carina--are reported to be present in over 90% of all BHD patients and might be an important clue in differentiating between sporadic RCC and BHD associated RCC. We conducted a retrospective study among patients diagnosed with sporadic RCC, wherein we retrospectively scored for the presence of lung cysts on thoracic CT. We performed FLCN mutation analysis in 8 RCC patients with at least one lung cysts under the carina. No mutations were identified. We compared the radiological findings in the FLCN negative patients to those in 4 known BHD patients and found multiple basal lung cysts were present significantly more frequent in FLCN mutation carriers and may be an indication for BHD syndrome in apparent sporadic RCC patients.

A Clinical and Experimental Comparison of Time of Flight PET/MRI and PET/CT Systems.

PURPOSE: The purpose of the study was to compare image quality and quantitative accuracy of positron emission tomography/magnetic resonance imaging (PET/MRI) and PET/computed tomography (PET/CT) systems with time of flight PET gantries, using phantom and clinical studies. PROCEDURES: Identical phantom experiments were performed on both systems. Calibration, uniformity, and standardized uptake value (SUV) recovery were measured. A clinical PET/CT versus PET/MRI comparison was performed using [(18)F]fluoromethylcholine ([(18)F]FCH). RESULTS: Calibration accuracy and image uniformity were comparable between systems. SUV recovery met EANM/EARL requirements on both scanners. Thirty-four lesions with comparable PET image quality were identified. Lesional SUVmax differences of 4 ± 26% between PET/MRI and PET/CT data were observed (R (2) = 0.79, slope = 1.02). In healthy tissues, PET/MRI-derived SUVs were 16 ± 11% lower than on PET/CT (R (2) = 0.98, slope = 0.86). CONCLUSION: PET/MRI and PET/CT showed comparable performance with respect to calibration accuracy, image uniformity, and SUV recovery. [(18)F]FCH uptake values for both healthy tissues and lesions corresponded reasonably well between MR- and CT-based systems, but only in regions free of MR-based attenuation artifacts.

[A boy with abdominal pain, fever and liver abcesses]. / Een jongen met buikpijn, koorts en leverabcessen.

An 18-year-old male with right lower abdominal pain and elevated infection parameters is diagnosed with an abdominal abscess, multiple liver abscesses and a thrombus in his portal vein. This combination of findings leads to the diagnosis 'foie appendiculaire', a complicated form of appendicitis.

Whole-body-MR imaging including DWIBS in the work-up of patients with head and neck squamous cell carcinoma: a feasibility study.

OBJECTIVES: To assess the feasibility of whole-body magnetic resonance imaging (WB-MRI) including diffusion-weighted whole-body imaging with background-body-signal-suppression (DWIBS) for the evaluation of distant malignancies in head and neck squamous cell carcinoma (HNSCC); and to compare WB-MRI findings with (18)F-fluorodeoxyglucose positron emission tomography/computed tomography ((18)F-FDG-PET/CT) and chest-CT. METHODS: Thirty-three patients with high risk for metastatic spread (26 males; range 48-79 years, mean age 63 ± 7.9 years (mean ± standard deviation) years) were prospectively included with a follow-up of six months. WB-MRI protocol included short-TI inversion recovery and T1-weighted sequences in the coronal plane and half-fourier acquisition single-shot turbo spin-echo T2 and contrast-enhanced-T1-weighted sequences in the axial plane. Axial DWIBS was reformatted in the coronal plane. Interobserver variability was assessed using weighted kappa and the proportion specific agreement (PA). RESULTS: Two second primary tumors and one metastasis were detected on WB-MRI. WB-MRI yielded seven clinically indeterminate lesions which did not progress at follow-up. The metastasis and one second primary tumor were found when combining (18)F-FDG-PET/CT and chest-CT findings. Interobserver variability for WB-MRI was κ=0.91 with PA ranging from 0.82 to 1.00. For (18)F-FDG-PET/CT κ could not be calculated due to a constant variable in the table and PA ranged from 0.40 to 0.99. CONCLUSIONS: Our WB-MRI protocol with DWIBS is feasible in the work-up of HNSCC patients for detection and characterization of distant pathology. WB-MRI can be complementary to (18)F-FDG-PET/CT, especially in the detection of non (18)F-FDG avid second primary tumors.

A de novo FLCN mutation in a patient with spontaneous pneumothorax and renal cancer; a clinical and molecular evaluation.

Birt-Hogg-Dubé syndrome (BHD) is an autosomal dominant condition due to germline FLCN (folliculin) mutations, characterized by skin fibrofolliculomas, lung cysts, pneumothorax and renal cancer. We identified a de novo FLCN mutation, c.499C>T (p.Gln167X), in a patient who presented with spontaneous pneumothorax. Subsequently, typical skin features and asymptomatic renal cancer were diagnosed. Probably, de novo FLCN mutations are rare. However, they may be under-diagnosed if BHD is not considered in sporadic patients who present with one or more of the syndromic features. Genetic and immunohistochemical analysis of the renal tumour indicated features compatible with a tumour suppressor role of FLCN. The finding that mutant FLCN was expressed in the tumour might indicate residual functionality of mutant FLCN, a notion which will be explored in future studies.