Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) syndrome: Spectrum of imaging findings.

PURPOSE: To retrospectively investigate the radiological presentations of HLRCC-associated renal tumors to facilitate accurate lesion characterization and compare these presentations with simple cysts and characteristics of other subtypes of renal cell carcinoma (RCC) as reported in the literature. METHODS: The MRI and CT imaging characteristics of 39 pathologically confirmed lesions from 30 patients (20 male, 10 female) with HLRCC syndrome were evaluated by two radiologists. Patients had an average age at diagnosis of 43.8 ± 13.1 years. Lesion characteristics including laterality, homogeneity, diameter (cm), nodularity, septations, T1 and T2 signal intensity, enhancement, and restricted diffusion were recorded. Imaging characteristics of the lesions were further compared to characteristics of benign simple cysts surgically removed at the same time point. RESULTS: The examined lesions had a mean diameter of 5.06 ± 3.80 cm, an average growth rate of 2.91 × 10-3 cm/day and an estimated annual growth rate of 1.06 cm/year. 50% of lesions demonstrated nodularity, 65% were mostly T2-hyperintense, 83% demonstrated restricted diffusion in solid portions of the lesions, and 65% had well-defined margins. 76% of patients demonstrated extra-renal manifestations, 53% lymphadenopathy, and 43% distant metastasis. CONCLUSIONS: Our analysis confirmed that while HLRCC-associated renal lesions demonstrate diversity in imaging presentations, the majority are unilateral and solitary, T2-hyperintense, heterogeneous with well-defined margins, and frequently demonstrate restricted diffusion and nodularity.

Differentiating papillary type I RCC from clear cell RCC and oncocytoma: application of whole-lesion volumetric ADC measurement.

PURPOSE: To determine whether objective volumetric whole-lesion apparent diffusion coefficient (ADC) distribution analysis improves upon the capabilities of conventional subjective small region-of-interest (ROI) ADC measurements for prediction of renal cell carcinoma (RCC) subtype. METHODS: This IRB-approved study retrospectively enrolled 55 patients (152 tumors). Diffusion-weighted imaging DWI was acquired at b values of 0, 250, and 800 s/mm2 on a 1.5T system (Aera, Siemens Healthcare). Whole-lesion measurements were performed by a research fellow and reviewed by a fellowship-trained radiologist. Mean, median, skewness, kurtosis, and every 5th percentile ADCs were determined from the whole-lesion histogram. Linear mixed models that accounted for within-subject correlation of lesions were used to compare ADCs among RCC subtypes. Receiver-operating characteristic (ROC) curve analysis with optimal cutoff points from the Youden index was used to test the ability of ADCs to differentiate clear cell RCC (ccRCC), papillary RCC (pRCC), and oncocytoma subtypes. RESULTS: Whole-lesion ADC values were significantly different between pRCC and ccRCC, and between pRCC and oncocytoma, demonstrating strong ability to differentiate subtypes across the quantiles (both P < 0.001). Best percentile ROC analysis demonstrated AUC values of 95.2 for ccRCC vs. pRCC; 67.6 for oncocytoma vs. ccRCC; and 95.8 for oncocytoma vs. pRCC. Best percentile ROC analysis further indicated model sensitivities/specificities of 84.5%/93.1% for ccRCC vs. pRCC; 100.0%/10.3% for oncocytoma vs. ccRCC; and 88.5%/93.1% for oncocytoma vs. pRCC. CONCLUSION: The objective methodology of whole-lesion volumetric ADC measurements maintains the sensitivity/specificity of conventional expert-based ROI analysis, provides information on lesion heterogeneity, and reduces observer bias.

Multimedia-enhanced Radiology Reports: Concept, Components, and Challenges.

Multimedia-enhanced radiology report (MERR) development is defined and described from an informatics perspective, in which the MERR is seen as a superior information-communicating entity. Recent technical advances, such as the hyperlinking of report text directly to annotated images, improve MERR information content and accessibility compared with text-only reports. The MERR is analyzed by its components, which include hypertext, tables, graphs, embedded images, and their interconnections. The authors highlight the advantages of each component for improving the radiologist's communication of report content information and the user's ability to extract information. Requirements for MERR implementation (eg, integration of picture archiving and communication systems, radiology information systems, and electronic medical record systems) and the authors' initial experiences and challenges in MERR implementation at the National Institutes of Health are reviewed. The transition to MERRs has provided advantages over use of traditional text-only radiology reports because of the capacity to include hyperlinked report text that directs clinicians to image annotations, images, tables, and graphs. A framework is provided for thinking about the MERR from the user's perspective. Additional applications of emerging technologies (eg, artificial intelligence and machine learning) are described in the crafting of what the authors believe is the radiology report of the future. ©RSNA, 2018.

Active Surveillance of Nonfatty Renal Masses in Patients With Lymphangioleiomyomatosis: Use of CT Features and Patterns of Growth to Differentiate Angiomyolipoma From Renal Cancer.

OBJECTIVE: The objective of this study was to report our experience with active surveillance of nonfatty renal masses in a large cohort of patients with lymphangioleiomyomatosis (LAM), correlate their CT features and patterns of growth with histopathology results, and provide guidelines for management. SUBJECTS AND METHODS: Yearly CT examinations were performed of 367 women (age range, 21-75 years; mean age, 47 years). For the 31 patients with 37 nonfatty renal masses that were biopsied, excised, or followed for ≥ 5 years, CT enhancement characteristics and patterns of growth were compared with the histopathology results. RESULTS: Four of 37 nonfatty renal masses were biopsied without follow-up CT examinations: Two were heterogeneous renal cell carcinomas (RCCs), one was a heterogeneous nonfatty angiomyolipoma (AML), and one was homogeneous nonfatty AML. In the remaining 33 nonfatty renal masses with multiple follow-up CT examinations, two growth patterns were identified. Four showed a continuous increase in size of > 0.5 cm/y in some years, and all four in this first group were heterogeneous and were biopsy-proven RCC. The second group was composed of the remaining 29 masses. These 29 masses showed yearly no change, increase, or decrease in diameter. Eight were heterogeneous, and 21 were homogeneous. Of the masses showing a yearly increase, the increase was < 0.5 cm/y in all except one. In the one exception, the increase followed a decrease. Nine of the 29 masses were biopsied, and all nine were nonfatty renal masses (five homogeneous, four heterogeneous). CONCLUSION: Our data provide further evidence in a large prospective study with longterm follow-up that active surveillance is an appropriate strategy in the management of nonfatty renal masses in patients with LAM. Our analysis of the growth patterns reveals duration of growth in addition to growth rate as criteria for biopsy or excision. Biopsy should be reserved for nonfatty renal masses that show sustained growth or growth > 0.5 cm/y during follow-up.

Associations among pericolonic fat, visceral fat, and colorectal polyps on CT colonography.

OBJECTIVE: To determine the association between pericolonic fat and colorectal polyps using CT colonography (CTC). METHODS: A total of 1169 patients who underwent CTC and optical colonoscopy on the same day were assessed. Pericolonic fat was measured on CTC in a band surrounding the colon. Visceral adipose tissue volume was measured at the L2-L3 levels. Student's t-tests, odds ratio, logistic regression, binomial statistics, and weighted kappa were performed to ascertain associations with the incidence of colorectal polyps. RESULTS: Pericolonic fat volume fractions (PFVF) were 61.5 ± 11.0% versus 58.1 ± 11.5%, 61.6 ± 11.1% versus 58.7 ± 11.5%, and 62.4 ± 10.6% versus 58.8 ± 11.5% for patients with and without any polyps, adenomatous polyps, and hyperplastic polyps, respectively (P<0.0001). Similar trends were observed when examining visceral fat volume fractions (VFVF). When patients were ordered by quintiles of PFVF or VFVF, there were 2.49-, 2.19-, and 2.39-fold increases in odds ratio for the presence of any polyp, adenomatous polyps, or hyperplastic polyps from the first to the fifth quintile for PFVF and 1.92-, 2.00-, and 1.71-fold increases in odds ratio for VFVF. Polyps tended to occur more commonly in parts of the colon that had more PFVF than the spatially adjusted average for patients in the highest quintile of VFVF. CONCLUSIONS: Pericolonic fat accumulations, like visceral fat, are correlated with an increased risk of adenomatous and hyperplastic polyps.

Feasibility of using the marginal blood vessels as reference landmarks for CT colonography.

OBJECTIVE: The purpose of this study was to show the spatial relationship of the colonic marginal blood vessels and the teniae coli on CT colonography (CTC) and the use of the marginal blood vessels for supine-prone registration of polyps and for determination of proper connectivity of collapsed colonic segments. MATERIALS AND METHODS: We manually labeled the marginal blood vessels on 15 CTC examinations. Colon segmentation, centerline extraction, teniae detection, and teniae identification were automatically performed. For assessment of their spatial relationships, the distances from the marginal blood vessels to the three teniae coli and to the colon were measured. Student t tests (paired, two-tailed) were performed to evaluate the differences among these distances. To evaluate the reliability of the marginal vessels as reference points for polyp correlation, we analyzed 20 polyps from 20 additional patients who underwent supine and prone CTC. The average difference of the circumferential polyp position on the supine and prone scans was computed. Student t tests (paired, two-tailed) were performed to evaluate the supine-prone differences of the distance. We performed a study on 10 CTC studies from 10 patients with collapsed colonic segments by manually tracing the marginal blood vessels near the collapsed regions to resolve the ambiguity of the colon path. RESULTS: The average distances (± SD) from the marginal blood vessels to the tenia mesocolica, tenia omentalis, and tenia libera were 20.1 ± 3.1 mm (95% CI, 18.5-21.6 mm), 39.5 ± 4.8 mm (37.1-42.0 mm), and 36.9 ± 4.2 mm (34.8-39.1 mm), respectively. Pairwise comparison showed that these distances to the tenia libera and tenia omentalis were significantly different from the distance to the tenia mesocolica (p < 0.001). The average distance from the marginal blood vessels to the colon wall was 15.3 ± 2.0 mm (14.2-16.3 mm). For polyp localization, the average difference of the circumferential polyp position on the supine and prone scans was 9.6 ± 9.4 mm (5.5-13.7 mm) (p = 0.15) and expressed as a percentage of the colon circumference was 3.1% ± 2.0% (2.3-4.0%) (p = 0.83). We were able to trace the marginal blood vessels for 10 collapsed colonic segments and determine the paths of the colon in these regions. CONCLUSION: The marginal blood vessels run parallel to the colon in proximity to the tenia mesocolica and enable accurate supine-prone registration of polyps and localization of the colon path in areas of collapse. Thus, the marginal blood vessels may be used as reference landmarks complementary to the colon centerline and teniae coli.

Cervical spine anomalies in Menkes disease: a radiologic finding potentially confused with child abuse.

BACKGROUND: Menkes disease is an X-linked recessive disorder of copper transport caused by mutations in ATP7A, a copper-transporting ATPase. Certain radiologic findings reported in this condition overlap with those caused by child abuse. However, cervical spine defects simulating cervical spine fracture, a known result of nonaccidental pediatric trauma, have not been reported previously in this illness. OBJECTIVE: To assess the frequency of cervical spine anomalies in Menkes disease after discovery of an apparent C2 posterior arch defect in a child participating in a clinical trial. MATERIALS AND METHODS: We examined cervical spine radiographs obtained in 35 children with Menkes disease enrolled in a clinical trial at the National Institutes of Health Clinical Center. RESULTS: Four of the 35 children with Menkes disease had apparent C2 posterior arch defects consistent with spondylolysis or incomplete/delayed ossification. CONCLUSION: Defects in C2 were found in 11% of infants and young children with Menkes disease. Discovery of cervical spine defects expands the spectrum of radiologic findings associated with this condition. As with other skeletal abnormalities, this feature simulates nonaccidental trauma. In the context of Menkes disease, suspicions of child abuse should be considered cautiously and tempered by these findings to avoid unwarranted accusations.

Association between visceral adiposity and colorectal polyps on CT colonography.

OBJECTIVE: The purpose of this article is to determine whether there is an association between visceral adiposity measured on CT colonography (CTC) and colorectal polyps. MATERIALS AND METHODS: Patients who underwent CTC and same-day optical colonoscopy (n = 1186) were analyzed. Visceral adipose tissue volumes and volume percentages relative to total internal body volume were measured on slices in the L2-L3 regions on supine CTC scans with validated fully automated software. Student t test, odds ratio, logistic regression, and receiver operating characteristic analyses were performed. RESULTS: For subjects with (n = 345) and without (n = 841) adenomatous polyps, the mean (± SD) volume percentages were 31.2% ± 10.8% and 28.2% ± 11.3%, respectively (p < 0.0001). For subjects with (n = 244) and without (n = 942) hyperplastic polyps, the volume percentages were 31.8% ± 10.7% and 28.3% ± 11.2%, respectively (p < 0.0001). Comparing the lowest and highest quintiles of volume percentage, the odds ratios for having at least one adenomatous polyp or hyperplastic polyp versus no polyp were 2.06 (95% CI, 1.36-3.13) and 1.71 (95% CI, 1.08-2.71), and the prevalence of having adenomatous polyps or hyperplastic polyps increased by 14% and 8%, respectively. CONCLUSION: Subjects with higher visceral adiposity measurements on CTC have a greater risk for the presence of colonic polyps.

Costimulated tumor-infiltrating lymphocytes are a feasible and safe alternative donor cell therapy for relapse after allogeneic stem cell transplantation.

Donor lymphocyte infusion (DLI), a standard relapse treatment after allogeneic stem cell transplantation (AlloSCT), has limited efficacy and often triggers GVHD. We hypothesized that after AlloSCT tumor-infiltrating donor lymphocytes could be costimulated ex vivo to preferentially activate/expand antitumor effectors. We tested the feasibility and safety of costimulated, tumor-derived donor lymphocyte (TDL) infusion in a phase 1 trial. Tumor was resected from 8 patients with B-cell malignancy progression post-AlloSCT; tumor cell suspensions were costimulated with anti-CD3/anti-CD28 Ab-coated magnetic beads and cultured to generate TDL products for each patient. Costimulation yielded increased proportions of T-bet(+)FoxP3(-) type 1 effector donor T cells. A median of 2.04 × 10(7) TDL/kg was infused; TDLs were well tolerated, notably without GVHD. Two transient positron emission tomography (PET) responses and 2 mixed responses were observed in these refractory tumors. TDL are a feasible, tolerable, and novel donor cell therapy alternative for relapse after AlloSCT.

Phase I trial of adoptive cell transfer with mixed-profile type-I/type-II allogeneic T cells for metastatic breast cancer.

PURPOSE: Metastatic breast cancer (MBC) response to allogeneic lymphocytes requires donor T-cell engraftment and is limited by graft-versus-host disease (GVHD). In mice, type-II-polarized T cells promote engraftment and modulate GVHD, whereas type-I-polarized T cells mediate more potent graft-versus-tumor (GVT) effects. This phase I translational study evaluated adoptive transfer of ex vivo costimulated type-I/type-II (T1/T2) donor T cells with T-cell-depleted (TCD) allogeneic stem cell transplantation (AlloSCT) for MBC. EXPERIMENTAL DESIGN: Patients had received anthracycline, taxane, and antibody therapies, and been treated for metastatic disease and a human leukocyte antigen (HLA)-identical-sibling donor. Donor lymphocytes were costimulated ex vivo with anti-CD3/anti-CD28 antibody-coated magnetic beads in interleukin (IL)-2/IL-4-supplemented media. Patients received reduced intensity conditioning, donor stem cells and T1/T2 cells, and monitoring for toxicity, engraftment, GVHD, and tumor response; results were compared with historical controls, identically treated except for T1/T2 product infusions. RESULTS: Mixed type-I/type-II CD4(+) T cells predominated in T1/T2 products. Nine patients received T1/T2 cells at dose level 1 (5 × 10(6) cells/kg). T-cell donor chimerism reached 100% by a median of 28 days. Seven (78%) developed acute GVHD. At day +28, five patients had partial responses (56%) and none had MBC progression; thereafter, two patients had continued responses. Donor T-cell engraftment and tumor responses appeared faster than in historical controls, but GVHD rates were similar and responders progressed early, often following treatment of acute GVHD. CONCLUSION: Allogeneic T1/T2 cells were safely infused with TCD-AlloSCT, appeared to promote donor engraftment, and may have contributed to transient early tumor responses.

Imaging features of lymphangioleiomyomatosis: diagnostic pitfalls.

OBJECTIVE: The objective of this article is to illustrate CT findings that may be misinterpreted and lead to unnecessary biopsy or surgical procedures in patients with lymphangioleiomyomatosis. CONCLUSION: Sequelae of pleurodesis, acutely hemorrhagic renal angiomyolipomas, and lymphatic involvement with lymphangioleiomyomatosis including enlarged lymph nodes and lymphangioleiomyomas are common benign conditions seen in patients with lymphangioleiomyomatosis that may be misdiagnosed on CT for malignancy and may prompt unnecessary biopsy and surgery. Ruptured abdominal pelvic lymphangioleiomyomas may be mistaken for appendicitis and other acute abdominal pelvic events.

CT of sclerotic bone lesions: imaging features differentiating tuberous sclerosis complex with lymphangioleiomyomatosis from sporadic lymphangioleiomymatosis.

PURPOSE: To determine if sclerotic bone lesions evident at body computed tomography (CT) are of value as a diagnostic criterion of tuberous sclerosis complex (TSC) and in the differentiation of TSC with lymphangioleiomyomatosis (LAM) from sporadic LAM. MATERIALS AND METHODS: Informed consent was signed by all patients in this HIPAA-compliant study approved by the institutional review board. Retrospective analysis was performed of the body CT studies of 472 patients: 365 with sporadic LAM, 82 with TSC/LAM, and 25 with TSC. The images were reviewed by using a picture archiving and communication system workstation with bone settings (window width, 1500 HU; window level, 300 HU) and fit-to-screen option. CT image characteristics assessed included shape, size, and distribution of sclerotic bone lesions with subsequent calculation of differences in the frequency of these lesions. RESULTS: Most commonly the sclerotic bone lesions were round, measured 0.3 cm (range, 0.2-3.2), and were distributed throughout the spine. The frequencies differed among the three patient groups Four or more sclerotic bone lesions were detected in all 25 (100%) of those with TSC, with a sensitivity of .89 (72 of 82) and specificity of .97 (355 of 367) in the differentiation of sporadic LAM from TSC/LAM (P < .01). CONCLUSION: The number of sclerotic bone lesions at body CT is of potential value in the diagnosis of TSC and in the differentiation of patients with sporadic LAM from those with TSC/LAM. (c) RSNA, 2010.

Normalized distance along the colon centerline: a method for correlating polyp location on CT colonography and optical colonoscopy.

OBJECTIVE: The ability to accurately locate a polyp found on CT colonography (CTC) at subsequent optical colonoscopy (OC) is an important part of the successful implementation of CTC for colorectal cancer screening. The purpose of this study was to determine whether a polyp's normalized distance along the colon centerline derived from CTC data can accurately predict its location on OC. MATERIALS AND METHODS: The polyp population consisted of 152 polyps in 121 patients. CTC polyp findings were verified by same-day segmentally-unblinded OC. Each polyp's normalized distance along the colon centerline was computed by dividing its distance from the anorectal junction measured along the colon centerline by the length of the colon at CTC. The predicted polyp location at OC was computed by multiplying the normalized distance along the colon centerline by the colon length at OC (i.e., the distance to the cecum as determined at full colonoscope insertion). The differences between the true and predicted polyp locations at OC were compared using paired Student's t tests, linear regression, prediction interval assessment, and Bland-Altman analyses. RESULTS: The differences between the true and predicted polyp locations at OC using the supine and prone CTC-normalized distances along the colon centerline were 2.2 +/- 10.5 cm (mean +/- SD; n = 136) and 1.5 +/- 10.5 cm (n = 135), respectively. The predicted location was within 10 cm of its true location for 71.3% (97/136) to 74.8% (101/135) of polyps and within 20 cm of its true location for 93.3% (126/135) to 93.4% (127/136) of polyps. CONCLUSION: By computing the normalized distance along the colon centerline of a polyp found at CTC, the location of a polyp at OC can be predicted to within 10 cm (i.e., 1 colonoscope mark) for the majority of polyps.

Conspicuity of colorectal polyps at CT colonography: visual assessment, CAD performance, and the important role of polyp height.

RATIONALE AND OBJECTIVES: The factors that influence the conspicuity of polyps on computed tomographic (CT) colonography (CTC) are poorly understood. The aim of this study is to compare radiologists' visual assessment of polyp conspicuity to quantitative image features and show the relationship between visual conspicuity and the detection of colonic polyps by computer-aided detection (CAD) on CTC. METHODS: One polyp (size range 6-10 mm) was selected from the CTC examination of each of 29 patients from a larger cohort. All patients underwent oral contrast-enhanced CTC with same-day optical colonoscopy with segmental unblinding. The polyps were analyzed by a previously validated CAD system and placed into one of two groups (detected [n = 12] or not detected [n = 17] by CAD). The study population was intentionally enriched with polyps that were not detected by the CAD system. Four board-certified radiologists, blinded to the CAD results, reviewed two- and three-dimensional CTC images of the polyps and scored the conspicuity of the polyps using a 4-point scale (0 = least conspicuous, 3 = most conspicuous). Polyp height and width were measured by a trained observer. A t-test (two-tailed, unpaired equal variance) was done to determine statistical significance. Intra- and interobserver variabilities of the conspicuity scores were assessed using the weighted kappa test. Regression analysis was used to investigate the relationship of conspicuity to polyp height and width. RESULTS: A statistically significant difference was found between the average conspicuity scores for polyps that were detected by CAD compared to those that were not (2.3 +/- 0.6 vs. 1.4 +/- 0.8) (P = .004). There was moderate intraobserver agreement of the conspicuity scores (weighted kappa 0.57 +/- 0.09). Interobserver agreement was fair (average weighted kappa for six pair-wise comparisons, 0.38 +/- 0.15). Conspicuity was correlated with manual measurement of polyp height (r(2) = 0.38-0.56, P < .001). CONCLUSIONS: This CAD system tends to detect 6-10 mm polyps that are more visually conspicuous. Polyp height is a major determinant of visual conspicuity. The generalizability of these findings to other CAD systems is currently unknown. Nevertheless, CAD developers may need to specifically target flatter and less conspicuous polyps for CAD to better assist the radiologist to find polyps in this clinically important size category.

Quality assessment for CT colonography: validation of automated measurement of colonic distention and residual fluid.

OBJECTIVE: The purpose of this study was to validate automated quality assessment (QA) software for CT colonography (CTC) by comparing results obtained with the software with results of interpretation by radiologists in the assessment of colonic distention and surface area obscured by residual fluid. MATERIALS AND METHODS: CTC scans of 30 patients were selected retrospectively to span ranges of luminal distention (well distended to poorly distended) and surface area covered by residual fluid (high amount of coverage to low amount of coverage). We used QA software developed in our laboratory to automatically measure the mean distention of each of five colonic segments (ascending, transverse, descending, sigmoid, and rectum). Three experienced radiologists visually graded each scan for distention and fluid coverage. Distention and fluid scores for specific segments were assessed with Bland-Altman analysis (mean difference with 95% limits of agreement) and the weighted kappa test. Interobserver and intraobserver variability was determined with the weighted kappa test. RESULTS: For distention scoring, the mean difference between radiologists and the QA software was 0.1% (95% limits of agreement, -25.6% and 25.9%). For fluid scoring, the mean difference was -0.6% (95% limits of agreement, -8.2% and 7.1%). There was moderate to good agreement (weighted kappa value, 0.50-0.78) between the radiologists' mean scores and the scores obtained with the QA software and for interreader and intrareader assessments of distention and fluid coverage. CONCLUSION: Results with the QA software agreed with radiologists' assessment of colonic distention and residual fluid coverage but were a more objective assessment. Use of this QA software can help standardize two important factors, distention and residual fluid coverage, that affect the quality of CTC, reducing two known causes of poor CTC performance.

Sporadic lymphangioleiomyomatosis and tuberous sclerosis complex with lymphangioleiomyomatosis: comparison of CT features.

PURPOSE: To retrospectively compare the frequencies of computed tomographic (CT) findings in patients with lymphangioleiomyomatosis (LAM) and patients with tuberous sclerosis complex (TSC) and LAM. MATERIALS AND METHODS: Institutional review board approval and informed consent were obtained for the HIPAA-compliant study. In 256 patients with LAM (mean age, 44 years) and 67 patients with TSC/LAM (mean age, 40 years), CT scans of the chest, abdomen, and pelvis were reviewed by a single radiologist. The fraction of lung involvement with cysts was estimated from high-spatial-resolution CT scans. Other findings assessed included noncalcified pulmonary nodules, pleural effusion, thoracic duct dilatation, hepatic and renal angiomyolipomas (AMLs), lymphangioleiomyoma (LALM), ascites, nephrectomy, and renal embolization. Confidence intervals and hypothesis tests of differences in frequencies, comparison of age quartiles, RIDIT analysis, analysis of variance, and correlation coefficients were used in the statistical analysis. RESULTS: Patients with LAM had more extensive lung involvement (RIDIT score, 0.36) and higher frequency of LALM (29% vs 9%, P<.001), thoracic duct dilatation (4% vs 0, P=.3), pleural effusion (12% vs 6%, P=.2), or ascites (10% vs 6%, P=.3). Patients with TSC/LAM had higher frequency of noncalcified pulmonary nodules (12% vs 1%, P<.01), hepatic (33% vs 2%, P<.001) and renal (93% vs 32%, P<.001) AMLs, nephrectomy (25% vs 7%, P<.001), or renal artery embolization (9% vs 2%, P<.05). CONCLUSION: The extent of lung disease is greater in LAM than TSC/LAM. Hepatic and renal AMLs and noncalcified lung nodules are more common in TSC/LAM, while lymphatic involvement-thoracic duct dilatation, chylous pleural effusion, ascites, and LALM-is more common in LAM.

Assessment of polyp and mass histopathology by intravenous contrast-enhanced CT colonography.

RATIONALE AND OBJECTIVES: We sought to demonstrate that intravenous contrast-enhanced CT colonography (CTC) can distinguish colonic adenomas from carcinomas. METHODS: Supine intravenous contrast-enhanced CTC with colonoscopic and/or surgical correlation was performed on 25 patients with colonic adenomas or carcinomas. Standard deviation of mean polyp CT attenuation was computed and assessed using ANOVA and receiver-operating characteristic analyses. RESULTS: Colonoscopy confirmed 32 polyps or masses 1 to 8 cm in size. The standard deviations of CT attenuation were carcinomas (n = 13; 36 +/- 6 HU; range 28-48 HU) and adenomas (n = 19; 49 +/- 14 HU; range 31-100 HU) (P = 0.005). At a standard deviation threshold of 42 HU, the sensitivity and specificity for classifying a polyp or mass as a carcinoma were 92% and 79%, respectively. The area under the receiver-operating characteristic curve was 0.89 +/- 0.06 (95% confidence interval 0.73-0.96). CONCLUSIONS: Measurement of the standard deviation of CT attenuation on intravenous contrast-enhanced CTC permits histopathologic classification of polyps 1 cm or larger as carcinomas versus adenomas. The presence of ulceration or absence of muscular invasion in carcinomas creates overlap with adenomas, reducing the specificity of carcinoma classification.

CT of pleural abnormalities in lymphangioleiomyomatosis and comparison of pleural findings after different types of pleurodesis.

OBJECTIVE: The objective of our article was to describe the spectrum and frequency of pleural abnormalities on CT in patients with lymphangioleiomyomatosis (LAM) and the pleural findings associated with different types of pleurodesis (talc, mechanical, and chemical) performed to treat the complications of pleural disease in these patients. MATERIALS AND METHODS: Two hundred fifty-eight patients with LAM underwent CT of the chest. Pleural abnormalities assessed included pleural thickening, presence of a pleural mass, areas of high attenuation, effusion, and pneumothorax. In patients who had had pleurodesis, the CT findings were correlated with the type of procedure performed. RESULTS: One hundred thirty-three (52%) of 258 patients had pleurodesis (unilateral, 68/133; bilateral, 65/133). Pleural abnormalities were more common in patients who had pleurodesis (101/133, 76%) than in those who had not (47/125, 38%) and were more prevalent on the operated side than on the unoperated side of those 68 patients who had unilateral pleurodesis. The frequencies of findings for the group without pleurodesis versus the group with pleurodesis were pleural thickening (26% vs 65%), effusion (10% vs 13%), loculated effusion (2.4% vs 11%), pneumothorax (1.6% vs 10%), areas of high attenuation (1.6% vs 23%), and mass (0.8% vs 14%), respectively. Areas of high attenuation in the pleura were present in all types of pleurodesis (mechanical, 8%; chemical, 13%; talc, 40%) and in two patients who had had repeated thoracentesis or pleurectomy. Pleural masses were present in patients who had had all types of pleurodesis (mechanical, 10%; chemical, 9%; talc, 24%) and in one patient who had had thoracentesis and thoracostomy; the masses commonly enhanced and did not change in size over time. CONCLUSION: Pleural abnormalities are common in patients with LAM as complications of the disease itself and as sequelae of pleurodesis and other pleura manipulations. Pneumothorax and pleural effusion result from the underlying pathophysiology of LAM, whereas areas of high attenuation and masses develop after all types of pleurodesis and other manipulations of the pleura (i.e., thoracentesis, thoracostomy).

Sonography of lymphangioleiomyoma in lymphangioleiomyomatosis: demonstration of diurnal variation in lesion size.

OBJECTIVE: Our aims were to define the sonographic features of abdominal and pelvic lymphangioleiomyomas in lymphangioleiomyomatosis (LAM) and to evaluate the utility of sonography in visualizing diurnal change in the size of the masses. MATERIALS AND METHODS: Forty-four patients with LAM and abdominal and pelvic lymphangioleiomyomas found on screening CT underwent sonography. Twenty-two patients had two studies on the same day, one in the morning and the other late in the afternoon. RESULTS: Forty-nine masses were scanned in the 44 patients. The anatomic distribution of the masses was the following: retroperitoneal (29/44 patients, 66%), pelvic (10/44, 23%), and both retroperitoneal and pelvic (5/44, 11%). Of the 49 masses, 12 (24%) were cystic, 16 (33%) were solid, and 21 (43%) were complex. Twenty-two patients underwent sonography in the morning and afternoon. The masses increased in size between the two studies in all 21 patients in whom the masses were visualized in both studies. In three of 21 patients, the echotexture of the masses changed between the morning and afternoon studies: In two the echotexture changed from solid to complex, and in the other, it changed from hyperechoic to isoechoic relative to the liver. CONCLUSION: The sonographic characteristics of lymphangioleiomyomas are similar to some neoplasms such as lymphoma and ovarian cancer (a similarity that sometimes prompts biopsy). After a mass is shown in a patient with LAM, repeat sonography in the morning and afternoon is useful to depict diurnal variation in size and echotexture and to confirm the diagnosis of lymphangioleiomyoma and avoid biopsy.

Functional tumor imaging with dynamic contrast-enhanced magnetic resonance imaging.

Dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) is the acquisition of serial MRI images before, during, and after the administration of an MR contrast agent. Unlike conventional enhanced MRI, which simply provides a snapshot of enhancement at one point in time, DCE-MRI permits a fuller depiction of the wash-in and wash-out contrast kinetics within tumors, and thus provides insight into the nature of the bulk tissue properties. Such data is readily amenable to two-compartment pharmacokinetic modeling from which parameters based on the rates of exchange between the compartments can be generated. These parameters can be used to generate color-encoded images that aid in the visual assessment of tumors. DCE-MRI is used currently to characterize masses, stage tumors, and noninvasively monitor therapy. While DCE-MRI is in clinical use, there are also a number of limitations, including overlap between malignant and benign inflammatory tissue, failure to resolve microscopic disease, and the inconsistent predictive value of enhancement pattern with regard to clinical outcome. Current research focuses on improving understanding of the meaning of DCE-MRI at a molecular level, evaluating macromolecular and targeted contrast agents, and combining DCE-MRI with other physiologic imaging techniques such as positron emission tomography. Efforts to standardize DCE-MRI acquisition, analysis, and reporting methods will allow wider dissemination of this useful functional imaging technique.