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Objective To evaluate the therapeutic effect of everolimus on tuberous sclerosis related renal angiomyolipoma (TSC-RAML) based on tumor components, and identify the types of main components in reducing RAML by everolimus. Methods To retrospectiely analyze the clinical data of 47 patients with TSC-RAML who were treated in the Urology Department of the First Affiliated Hospital of the Air Force Medical University and met the diagnostic criteria of ITSCCC from September 2017 to September 2022. According to the attenuation range of CT tissue specific threshold, patients were divided into fat rich group (HU ≤-10, n=26) and fat deficient group (HU ≥30, n=21). Collect patients' baseline CT data and 6 months after treatment, record the average CT value of RAML before and after treatment. Three-dimensional reconstruction of RAML was performed using Mimics software, and record the volume of RAML before and after treatment. The volume of RAML and mean CT value were compared between the two groups before and after treatment. Results No statistical difference existed in baseline characteristics between the two groups (P>0.05). The median reduction of tumor volume in fat rich group and fat deficient group of patients were 4.94 (3.12, 27.23) cm3 and 27.31 (10.83, 40.38) cm3, respectively, and the volume response rates were 11.52%±0.96% and 62.09%±12.60% respectively, the differences were statistically significant (P<0.05). The differences of average CT values between the fat rich group and fat deficient group were (4.23±3.01) HU and (14.52±3.61) HU respectively, and the reduction rates of CT values were 14.25%±11.94% and 29.23%±0.53% respectively, all were statistically significant (P<0.05). After treatment, the average CT value of tumors in fat deficient group decreased significantly compared to that in fat rich group with statistically significant difference (P<0.05). Six months after treatment with everolimus, the composition of high-density cord like vascular tissue in RAML tumors reduced significantly and fat conversion occurred. Conclusions The effect of everolimus on reducing tumor volume and average CT value of tumor in fat deficient RAML is better and significant, which confirmed that the reduction is the high-density component of TSC-RAML mainly composed of vascular components.
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| 科 Family | 属数 Number of genus | 种数 Number of species | 占总种数比例 Percentage of total species (%) | 属 Genus | 种数 Number of species | 占总种数比例 Percentage of total species (%) |
|---|---|---|---|---|---|---|
| 鹅膏菌科Amanitaceae | 2 | 11 | 5.26 | 鹅膏菌属 Amanita | 10 | 4.78 |
| 小菇科 Mycenaceae | 2 | 12 | 5.74 | 丝盖伞属 Inocybe | 5 | 2.39 |
| 多孔菌科 Polyporaceae | 8 | 14 | 6.70 | 蜡蘑属 Laccaria | 5 | 2.39 |
| 红菇科 Russulaceae | 3 | 23 | 11.00 | 小皮伞属 Marasmius | 6 | 2.87 |
| 小菇属 Mycena | 11 | 5.26 | ||||
| 光柄菇属 Pluteus | 5 | 2.39 | ||||
| 红菇属 Russula | 17 | 8.13 | ||||
| 栓菌属 Trametes | 5 | 2.39 |
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