The effect of sirolimus on angiomyolipoma is determined by decrease of fat-poor compartments and includes striking reduction of vascular structures
Abstract Renal angiomyolipomas hemorrhage is associated with their size and vascular constitution. The effects of sirolimus on different components of angiomyolipomas was analyzed in patients with tuberous sclerosis complex, sporadic lymphangioleiomyomatosis and multiple sporadic angiomyolipomas. Th...
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2021
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oai:doaj.org-article:97404f7e1c2e4f0797e6d60ba625d2c82021-12-02T13:39:23ZThe effect of sirolimus on angiomyolipoma is determined by decrease of fat-poor compartments and includes striking reduction of vascular structures10.1038/s41598-021-87930-42045-2322https://doaj.org/article/97404f7e1c2e4f0797e6d60ba625d2c82021-04-01T00:00:00Zhttps://doi.org/10.1038/s41598-021-87930-4https://doaj.org/toc/2045-2322Abstract Renal angiomyolipomas hemorrhage is associated with their size and vascular constitution. The effects of sirolimus on different components of angiomyolipomas was analyzed in patients with tuberous sclerosis complex, sporadic lymphangioleiomyomatosis and multiple sporadic angiomyolipomas. Thirty angiomyolipomas from 14 patients treated with sirolimus were retrospectively evaluated. A Hounsfield-unit threshold was used to classify angiomyolipomas in fat-rich, fat-poor and intermediate-fat tumors, and to categorize tumor compartments in fat rich, fat poor, intermediate fat and highly vascularized. Diameter variations were measured to assess the effects on aneurysmatic/ectatic vascular formations. Volume reduction following treatment with sirolimus was higher in fat-poor than fat-rich angiomyolipomas. Tumor reduction was mainly determined by decrease of the fat-poor and highly-vascularized compartments while the volume of the fat-rich compartment increased. Broad liposubstitution was observed in some tumors. A median reduction of 100% (75 to 100) in the diameter of aneurysmatic/ectatic vascular structures was observed. Our study showed that sirolimus reduces the size of angiomyolipomas by decreasing primarily their highly-vascularized and fat-poor compartments. This effect is associated with a remarkable reduction of tumoral aneurysms/ectatic vessels, revealing the likely mechanism responsible for the risk-decreasing effect of mTOR inhibitors on angiomyolipoma bleeding. These findings support the role of mTOR in the development of angiomyolipoma blood vessels.Elieser Hitoshi WatanabeFernando Morbeck Almeida CoelhoHilton Leão FilhoBruno Eduardo Pedroso BalboPrecil Diego Miranda de Menezes NevesFernanda Maria FranzinFernando Ide YamauchiLuiz Fernando OnuchicNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 11, Iss 1, Pp 1-12 (2021) |
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Medicine R Science Q Elieser Hitoshi Watanabe Fernando Morbeck Almeida Coelho Hilton Leão Filho Bruno Eduardo Pedroso Balbo Precil Diego Miranda de Menezes Neves Fernanda Maria Franzin Fernando Ide Yamauchi Luiz Fernando Onuchic The effect of sirolimus on angiomyolipoma is determined by decrease of fat-poor compartments and includes striking reduction of vascular structures |
| description |
Abstract Renal angiomyolipomas hemorrhage is associated with their size and vascular constitution. The effects of sirolimus on different components of angiomyolipomas was analyzed in patients with tuberous sclerosis complex, sporadic lymphangioleiomyomatosis and multiple sporadic angiomyolipomas. Thirty angiomyolipomas from 14 patients treated with sirolimus were retrospectively evaluated. A Hounsfield-unit threshold was used to classify angiomyolipomas in fat-rich, fat-poor and intermediate-fat tumors, and to categorize tumor compartments in fat rich, fat poor, intermediate fat and highly vascularized. Diameter variations were measured to assess the effects on aneurysmatic/ectatic vascular formations. Volume reduction following treatment with sirolimus was higher in fat-poor than fat-rich angiomyolipomas. Tumor reduction was mainly determined by decrease of the fat-poor and highly-vascularized compartments while the volume of the fat-rich compartment increased. Broad liposubstitution was observed in some tumors. A median reduction of 100% (75 to 100) in the diameter of aneurysmatic/ectatic vascular structures was observed. Our study showed that sirolimus reduces the size of angiomyolipomas by decreasing primarily their highly-vascularized and fat-poor compartments. This effect is associated with a remarkable reduction of tumoral aneurysms/ectatic vessels, revealing the likely mechanism responsible for the risk-decreasing effect of mTOR inhibitors on angiomyolipoma bleeding. These findings support the role of mTOR in the development of angiomyolipoma blood vessels. |
| format |
article |
| author |
Elieser Hitoshi Watanabe Fernando Morbeck Almeida Coelho Hilton Leão Filho Bruno Eduardo Pedroso Balbo Precil Diego Miranda de Menezes Neves Fernanda Maria Franzin Fernando Ide Yamauchi Luiz Fernando Onuchic |
| author_facet |
Elieser Hitoshi Watanabe Fernando Morbeck Almeida Coelho Hilton Leão Filho Bruno Eduardo Pedroso Balbo Precil Diego Miranda de Menezes Neves Fernanda Maria Franzin Fernando Ide Yamauchi Luiz Fernando Onuchic |
| author_sort |
Elieser Hitoshi Watanabe |
| title |
The effect of sirolimus on angiomyolipoma is determined by decrease of fat-poor compartments and includes striking reduction of vascular structures |
| title_short |
The effect of sirolimus on angiomyolipoma is determined by decrease of fat-poor compartments and includes striking reduction of vascular structures |
| title_full |
The effect of sirolimus on angiomyolipoma is determined by decrease of fat-poor compartments and includes striking reduction of vascular structures |
| title_fullStr |
The effect of sirolimus on angiomyolipoma is determined by decrease of fat-poor compartments and includes striking reduction of vascular structures |
| title_full_unstemmed |
The effect of sirolimus on angiomyolipoma is determined by decrease of fat-poor compartments and includes striking reduction of vascular structures |
| title_sort |
effect of sirolimus on angiomyolipoma is determined by decrease of fat-poor compartments and includes striking reduction of vascular structures |
| publisher |
Nature Portfolio |
| publishDate |
2021 |
| url |
https://doaj.org/article/97404f7e1c2e4f0797e6d60ba625d2c8 |
| work_keys_str_mv |
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