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Long-term follow-up study of the malignant transformation potential of the simple renal cysts.

ABSTRACT: Background:We conducted a multi-center study to investigate the prevalence, the malignant transformation potential of the simple renal cysts and the factors that might predict malignancy. Methods:We defined the simple renal cysts as Bosniak class I & II (including IIF) lesions. In the prevalence study, data from 115,132 ultrasonographies was collected from individuals who participated in a multiphasic health wellness screen. In the natural history and progression study, we retrospectively reviewed 333 participants with simple renal cysts and were followed for at the least 3 years with a mean of 6.3±2.9 years (ranged from 3 to 13 years). Results:About 7.2% (8,303) of the individuals who participated in the study were found to have at the least one simple renal cyst. The incidence increased with age from 0.6% in the first decade to 28.0% in the eighth or later decade of life. The Bosniak class I lesion accounted for 7,559 or 91.0% of the cysts whereas 744 or 9.0% were class II. A slower growth rate was observed in the older age group. Twenty-four patients (7.2%) had their renal cysts upgraded according to the Bosniak classification. Gender, age, initial cyst size, number of cysts and bi-laterality failed to predict the malignancy tendency. Conclusions:Most of the cysts discovered were the Bosniak class I and II lesions and they rarely progress further. Treatment for the asymptomatic simple renal cyst is not warranted. Treatment decision-making based on older age or larger initial cyst size should not be recommended.

SUBMITTER: Yang B

PROVIDER: S-EPMC7215042 | biostudies-literature | 2020 Apr

REPOSITORIES: biostudies-literature

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Long-term follow-up study of the malignant transformation potential of the simple renal cysts.

Yang Bicheng B Qiu Chunhua C Wan Shawpong S Liu Jianhua J Li Qingxiu Q Mai Zanlin Z Zeng Tao T Liu Yang Y He Weizhen W Zeng Guohua G

Translational andrology and urology 20200401 2

<h4>Background</h4>We conducted a multi-center study to investigate the prevalence, the malignant transformation potential of the simple renal cysts and the factors that might predict malignancy.<h4>Methods</h4>We defined the simple renal cysts as Bosniak class I & II (including IIF) lesions. In the prevalence study, data from 115,132 ultrasonographies was collected from individuals who participated in a multiphasic health wellness screen. In the natural history and progression study, we retrosp ...[more]

PMID: 32420175

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Project description:ObjectiveMegalencephalic leukoencephalopathy with subcortical cysts (MLC, OMIM 604004) is a rare neurological deterioration disease. We aimed to clarify clinical and genetic features of Chinese MLC patients.MethodsClinical information and peripheral venous blood of 20 patients and their families were collected, Sanger-sequencing and Multiple Ligation-dependent Probe Amplification were performed to make genetic analysis. Splicing-site mutation was confirmed with RT-PCR. UPD was detected by haplotype analysis. Follow-up study was performed through telephone for 27 patients.ResultsOut of 20 patients, macrocephaly, classic MRI features, motor development delay and cognitive impairment were detected in 20(100%), 20(100%), 17(85%) and 4(20%) patients, respectively. 20(100%) were clinically diagnosed with MLC. 19(95%) were genetically diagnosed with 10 novel mutations in MLC1, MLC1 and GlialCAM mutations were identified in 15 and 4 patients, respectively. Deletion mutation from exon4 to exon9 and a homozygous point mutation due to maternal UPD of chromosome22 in MLC1 were found firstly. c.598-2A>C in MLC1 leads to the skip of exon8. c.772-1G>C in MLC1 accounting for 15.5%(9/58) alleles in Chinese patients might be a founder or a hot-spot mutation. Out of 27 patients in the follow-up study, head circumference was ranged from 56cm to 61cm in patients older than 5yeas old, with a median of 57cm. Motor development delay and cognitive impairment were detected in 22(81.5%) and 5(18.5%) patients, respectively. Motor and cognitive deterioration was found in 5 (18.5%) and 2 patients (7.4%), respectively. Improvements and MRI recovery were first found in Chinese patients. Rate of seizures (45.5%), transient motor retrogress (45.5%) and unconsciousness (13.6%) after head trauma was much higher than that after fever (18.2%, 9.1%, 0%, respectively).SignificanceIt's a clinical and genetic analysis and a follow-up study for largest sample of Chinese MLC patients, identifying 10 novel mutations, expanding mutation spectrums and discovering clinical features of Chinese MLC patients.

Project description:The effect of migalastat on long-term renal outcomes in enzyme replacement therapy (ERT)-naive and ERT-experienced patients with Fabry disease is not well defined. An integrated posthoc analysis of the phase 3 clinical trials and open-label extension studies was conducted to evaluate long-term changes in renal function in patients with Fabry disease and amenable GLA variants who were treated with migalastat for ≥2 years during these studies. The analysis included ERT-naive (n = 36 [23 females]; mean age 45 years; mean baseline estimated glomerular filtration rate (eGFR), 91.4 mL/min/mL/1.73 m2) and ERT-experienced (n = 42 [24 females]; mean age, 50 years; mean baseline eGFR, 89.2 mL/min/1.73m2) patients with amenable variants who received migalastat 123 mg every other day for ≥2 years. The annualized rate of change from baseline to last observation in estimated glomerular filtration rate using the Chronic Kidney Disease Epidemiology Collaboration equation (eGFRCKD-EPI) was calculated by both simple linear regression and a random coefficient model. In ERT-naive patients, mean annualized rates of change from baseline in eGFRCKD-EPI were - 1.6 mL/min/1.73 m2 overall and - 1.8 mL/min/1.73 m2 and - 1.4 mL/min/1.73 m2 in male and female patients, respectively, as estimated by simple linear regression. In ERT-experienced patients, mean annualized rates of change from baseline in eGFRCKD-EPI were - 1.6 mL/min/1.73 m2 overall and - 2.6 mL/min/1.73 m2 and - 0.8 mL/min/1.73 m2 in male and female patients, respectively. Mean annualized rate of change in eGFRCKD-EPI in ERT-naive patients with the classic phenotype (defined by white blood cell alpha galactosidase A [α-Gal A] activity of <3% of normal and multiorgan system involvement) was -1.7 mL/min/1.73 m2. When calculated using the random coefficient model, which adjusted for sex, age, and baseline renal function, the annualized eGFRCKD-EPI change was minimal (mean: -0.1 and 0.1 mL/min/1.73 m2 in ERT-naive and ERT-experienced patients, respectively). In conclusion, patients with Fabry disease and amenable GLA variants receiving long-term migalastat treatment (≤8.6 years) maintained renal function irrespective of treatment status, sex, or phenotype.

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Long-term follow-up study of the malignant transformation potential of the simple renal cysts.

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Long-term follow-up study of the malignant transformation potential of the simple renal cysts.

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