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1 h Tsc2(+/-) mice (adenoma) and TSC patients (angiomyolipoma).
2 nclassified carcinomas; 16 oncocytomas; nine angiomyolipomas).
3 C), including facial angiofibromas and renal angiomyolipoma.
4 r TSC2 was further demonstrated in the renal angiomyolipoma.
5 ent with lipid-does not necessarily indicate angiomyolipoma.
6 elopment in multiple organs, including renal angiomyolipoma.
7 transitional cell carcinoma, and one was an angiomyolipoma.
8 tor, similar to lymphangioleiomyomatosis and angiomyolipoma.
9 TSC2 mutations cause LAM in patients without angiomyolipomas.
10 is complex to evaluate both kidney cysts and angiomyolipomas.
11 ignal transduction pathways underlying renal angiomyolipomas.
12 TSC2 LOH was detected in seven (54%) of the angiomyolipomas.
13 he TSC2 gene occurs in 60% of TSC-associated angiomyolipomas.
14 ssor model for the pathogenesis of SEGAs and angiomyolipomas.
15 smooth muscle, fat, and vessels) of six TSC2 angiomyolipomas.
16 th muscle and fat but not the vessels of two angiomyolipomas.
17 Women with LAM can also develop renal angiomyolipomas.
18 chylous effusions, lymphangioleiomyomas, and angiomyolipomas.
19 n benign tumors, neurological disorders, and angiomyolipomas.
20 d p70 S6 kinase and p56 were present only in angiomyolipomas.
21 hose with cysts and 9.2 years for those with angiomyolipomas.
23 nts aged 18 years or older with at least one angiomyolipoma 3 cm or larger in its longest diameter (d
24 rocytoma (37.1% vs. 14.6%; P = 0.018), renal angiomyolipoma (60.0% vs. 27.1%; P = 0.003), cognitive i
25 nign renal neoplasia (such as oncocytoma and angiomyolipoma), alterations of BAP1, FLCN, and MITF wer
27 ost common abdominal findings included renal angiomyolipoma (AML) in 43 patients (54%), enlarged abdo
29 mechanisms may also operate in the cells of angiomyolipoma (AML), which develops as a result of muta
33 aturia with underlying giant bilateral renal angiomyolipomas (AML) with estimated total tumor burden
38 thoracic duct dilatation, hepatic and renal angiomyolipomas (AMLs), lymphangioleiomyoma (LALM), asci
39 In this case, one would predict that the angiomyolipoma and LAM cells would have identical LOH pa
42 ompared the chromosome 16 LOH region between angiomyolipoma and pulmonary LAM from two patients with
51 nts had TSC2 mutations and TSC2 LOH in their angiomyolipomas and pulmonary LAM cells but not in norma
52 atic mutations in the TSC2 gene occur in the angiomyolipomas and pulmonary LAM cells of women with sp
53 ed by multiorgan hamartomas, including renal angiomyolipomas and pulmonary lymphangioleiomyomatosis (
54 childhood, but some findings, notably renal angiomyolipomas and pulmonary lymphangioleiomyomatosis (
56 sclerosis complex and to reduce the size of angiomyolipomas and stabilize lung function in humans.
57 most frequent tumors in TSC patients, renal angiomyolipomas and subependymal giant cell astrocytomas
58 of typically vascularised tumours including angiomyolipomas and subependymal giant cell astrocytomas
61 e, 34% (72-48 units) for predominately fatty angiomyolipomas, and 39% (85-52 units) for vertebral hem
62 -3 was increased in TSC-related skin tumors, angiomyolipomas, and lymphangioleiomyomatosis with serum
63 (including 18 oncocytomas, seven lipid-poor angiomyolipomas, and one hemangioblastoma) and 68 malign
64 ; 14 (7%), oncocytomas; six (3%), lipid-poor angiomyolipomas; and 16 (8%), other or unclassified rena
66 rin heterozygous mice and from a human renal angiomyolipoma are highly sensitive to PDGFR antagonists
67 onary lymphangioleiomyomatosis and abdominal angiomyolipoma are related lesions for which there is no
74 promising accuracy for detecting lipid-poor angiomyolipomas (area under the receiver operating chara
77 tion, the patient had no evidence of a renal angiomyolipoma at autopsy and therefore demonstrated for
79 ion in LAM and decreases the volume of renal angiomyolipomas, but lung function declines and angiomyo
82 yolipomas, we have generated the first human angiomyolipoma cell line by sequential introduction of S
85 rmal pulmonary smooth muscle cells and renal angiomyolipoma cells from patients with sporadic pulmona
88 -null tumor cells and immortalized TSC2-null angiomyolipoma cells, but not in cells with intact TSC.
96 ied Rapamycin-dependent miRNA in LAM patient angiomyolipoma-derived cells using two separate screens.
97 ATX expression is upregulated in human renal angiomyolipoma-derived TSC2-deficient cells compared wit
98 with cluster 1, with increased likelihood of angiomyolipomas, dermatological findings and subependyma
99 of TSC1 or TSC2 is a known genetic driver of angiomyolipoma development, however, whether an altered
101 ns, some features (grade 2-4 kidney cysts or angiomyolipomas, forehead plaques, retinal hamartomas, a
102 2 loss of heterozygosity in 7 of 13 (54%) of angiomyolipomas from sporadic LAM patients, suggesting t
107 ore subependymal giant cell astrocytomas and angiomyolipomas, higher incidence of pharmacoresistant e
109 MITF-A) was consistently highly expressed in angiomyolipoma, immunohistochemistry showed microphthalm
111 uggested to contribute lower risk for kidney angiomyolipomas in patients with TSC2 gene mutations.
112 We tested this hypothesis in eight renal angiomyolipomas in which the loss of tuberous sclerosis
115 clinical decisions related to the fact that angiomyolipomas larger than 4 cm in diameter are more ap
116 n = 66) and hyperattenuating (n = 28) cysts, angiomyolipomas (n = 18), and solid enhancing lesions (n
117 us ascites (n = 3), complications from renal angiomyolipomas (n = 4), and recurrent LAM (n = 1).
118 s measured included adipose tissue (n = 10), angiomyolipomas (n = 8), and vertebral hemangiomas (n =
119 ese cells show phenotypic characteristics of angiomyolipomas, namely differentiation markers of smoot
121 test was used to determine whether cysts and angiomyolipomas occurred in different subpopulations.
125 distinct disease subgroups were identified: angiomyolipoma-predominant TSC (cluster 1), TSC with inf
128 iomyolipomas, but lung function declines and angiomyolipomas regrow when treatment is discontinued, s
130 2(-/-) ASM cells, derived from a human renal angiomyolipoma, require epidermal growth factor (EGF) fo
131 as the proportion of patients with confirmed angiomyolipoma response of at least a 50% reduction in t
134 nohistochemical stains of both LAM and renal angiomyolipoma showed positive immunoreactivity for hama
135 elevated at baseline, correlates with kidney angiomyolipoma size at baseline and 12 months, and decre
137 ponse to treatment with sirolimus and kidney angiomyolipoma size in patients with TSC, but confirmati
138 to be hypervascular, chromophobe lesions and angiomyolipomas tended to enhance moderately, and papill
139 tion, %SI change was significantly higher in angiomyolipomas than in clear cell carcinomas, but only
140 t and benign renal lesions: in patients with angiomyolipoma the ADC value was 2.36+/-0.32x10(-3) mm(2
142 F-A is a transcriptional oncogenic driver of angiomyolipoma tumor development, acting through regulat
143 lid small renal masses, excluding lipid-rich angiomyolipomas, underwent qualitative contrast-enhanced
147 l to determine whether sirolimus reduces the angiomyolipoma volume in patients with the tuberous scle
151 as available, the same mutation found in the angiomyolipoma was present in the abnormal pulmonary smo
155 imaging for the diagnosis of RCCs (excluding angiomyolipomas) were 0.856, 86%, and 80%, respectively.
156 ehead plaques, retinal hamartomas, and liver angiomyolipomas) were very rare or not seen at all in TS
160 imaging, can be confidently diagnosed as an angiomyolipoma without further diagnostic intervention.