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• W2998098194 abstract "<b><i>Background:</i></b> Mammalian target of rapamycin (mTOR) inhibitors have been shown to have excellent effects in the management of kaposiform hemangioendothelioma (KHE); however, the mechanism of action is unclear. This study identified the expressions of mTOR pathway-related proteins in different vascular tumors to provide insight into the pathogenesis of KHE. <b><i>Methods:</i></b> We retrospectively reviewed the pathologic specimens of 30 patients (KHE, 15; tufted angioma [TA], 5; infantile hemangioma [IH], 5; and lymphatic malformation [LM], 5). The immunohistochemical expression of mTOR-related proteins tuberous sclerosis complex 2 (TSC2), phosphatase and tensin homologue (PTEN), phosphorylated eukaryotic translation initiation factor 4E binding protein 1 (p-4EBP1), phosphorylated mTOR (p-mTOR), and phosphorylated ribosomal protein S6 kinase B1 (p-P70S6K) were analyzed using Image-Pro Plus software. KHE had the following pattern of expression in the spindle vascular endothelial cells: TSC2 (–); PTEN (–); p-4EBP1 (+); p-mTOR (+); and p-P70S6K (+). <b><i>Results:</i></b> All 3 patients treated with sirolimus had good responses. The TA results were similar to KHE with no significant differences (p-4EBP1: <i>p</i> = 0.0687; p-mTOR: <i>p</i> = 0.0832). The expressions of TSC2, PTEN, p-4EBP1, p-mTOR, and p-P70S6K were negative or weakly positive in IH with a statistically significant difference compared to KHE (p-4EBP1: <i>p</i> &#x3c; 0.001; p-mTOR: <i>p</i> &#x3c; 0.001; p-P70S6K: <i>p</i> &#x3c; 0.001). LM had no significant differences when compared to KHE. <b><i>Conclusions:</i></b> The absence of TSC2 and PTEN caused abnormal activation of the mTOR signaling pathway and may be involved in the pathogenesis of KHE. The expression of mTOR-related proteins in TA and LM was similar to KHE, unlike IH. The KHE pattern of expression [PTEN (–), TSC2 (–), p-mTOR (+), p-P70S6K (+), and p-4EBP1 (+)] suggested that sirolimus may be a good therapeutic choice." @default.
• W2998098194 created "2020-01-10" @default.
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• W2998098194 date "2020-01-01" @default.
• W2998098194 modified "2023-10-14" @default.
• W2998098194 title "Immunohistochemical Analysis of mTOR Pathway-Related Proteins in Kaposiform Hemangioendothelioma" @default.
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• W2998098194 doi "https://doi.org/10.1159/000503604" @default.
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