Diagnostic performance of the "drooping" sign in CT diagnosis of exophytic renal angiomyolipoma.

OBJECTIVE: To evaluate the prevalence of angular interface and the "drooping" sign in exophytic renal angiomyolipomas (AMLs) and the diagnostic performance in differentiating exophytic lipid-poor AMLs from other solid renal masses. METHODS: This IRB-approved, two-center study included 185 patients with 188 exophytic solid renal masses < 4 cm with histopathology and pre-operative CT within 30 days of surgical resection or biopsy. Images were reviewed for the presence of angular interface and the "drooping" sign qualitatively by three readers blinded to the final diagnosis, with majority rules applied. Both features were assessed quantitatively by cohort creators (who are not readers) independently. Free-marginal kappa was used to assess inter-reader agreement and agreement between two methods assessing each feature. Fisher's exact test, Mann-Whitney test, and multivariable logistic regression with two-tailed p < 0.05 were used to determine statistical significance. Diagnostic performance was assessed. RESULTS: Ninety-four patients had 96 AMLs, and 91 patients had 92 non-AMLs. Seventy-four (77%) of AMLs were lipid-poor based on quantitative assessment on CT. The presence of angular interface and the "drooping" sign by both qualitative and quantitative assessment were statistically significantly associated with AMLs (39% (qualitative) and 45% (quantitative) vs 15% (qualitative) and 13% (quantitative), and 48% (qualitative) and 43% (quantitative) vs 4% (qualitative) and 1% (quantitative), respectively, all p < 0.001) in univariable analysis. In multivariable analysis, only the "drooping" sign in either qualitative or quantitative assessment was a statistically significant predictor of AMLs (both p < 0.001). Inter-reader agreement for the "drooping" sign was moderate (k = 0.55) and for angular interface was fair (k = 0.33). Agreement between the two methods of assessing the "drooping" sign was substantial (k = 0.84) and of assessing the angular interface was moderate (k = 0.59). The "drooping" sign both qualitatively and quantitatively, alone or in combination of angular interface, had very high specificity (96-100%) and positive predictive value (PPV) (89-100%), moderate negative predictive value (62-68%), but limited sensitivity (23-49%) for lipid-poor AMLs. CONCLUSION: The "drooping" sign by both qualitative and quantitative assessment is highly specific for lipid-rich and lipid-poor AMLs. This feature alone or in combination with angular interface can aid in CT diagnosis of lipid-poor AMLs with very high specificity and PPV.

Volumetric analysis of IDH-mutant lower-grade glioma: a natural history study of tumor growth rates before and after treatment.

BACKGROUND: Lower-grade gliomas (LGGs) with isocitrate dehydrogenase 1 and/or 2 (IDH1/2) mutations have long survival times, making evaluation of treatment efficacy difficult. We investigated the volumetric growth rate of IDH mutant gliomas before and after treatment with established glioma therapies to determine whether a significant change in growth rate could be documented and perhaps be used in the future to evaluate treatment response to investigational agents in LGG trials. METHODS: In this multicenter retrospective study, 230 adult patients with IDH1/2 mutated LGGs (World Health Organization grade II or III) undergoing surgery, radiation, or chemotherapy for progressive non-enhancing tumor were identified. Subjects were required to have 3 MRI scans containing T2/fluid attenuated inversion recovery imaging spanning a minimum of 6 months prior to treatment. A mixed-effect model was used to estimate tumor growth prior to treatment. A subset of 95 patients who received chemotherapy, radiotherapy, or chemoradiotherapy and had 2 posttreatment imaging time points available were evaluated for change in pre- and posttreatment volumetric growth rates using a piecewise mixed model. RESULTS: The pretreatment volumetric growth rate across all 230 patients was 27.37%/180 days (95% CI: [23.36%, 31.51%]). In the 95 patients with both pre- and posttreatment scans available, there was a significant difference in volumetric growth rates before (26.63%/180 days, 95% CI: [19.31%, 34.40%]) and after treatment (-15.24% /180 days, 95% CI: [-21.37%, -8.62%]) (P < 0.0001). The growth rates for patient subgroup with 1p/19q codeletion (N = 118) was significantly slower than the rate of the 1p/19q non-codeleted group (N = 68) (22.84% vs 35.49%, P = 0.0108). CONCLUSION: In this study, we evaluated the growth rates of IDH mutant gliomas before and after standard therapy. Further study is needed to establish whether a change in growth rate is associated with patient survival and its use as a surrogate endpoint in clinical trials for IDH mutant LGGs.