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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.
22  were hemorrhagic cysts (5%), and one was an angiomyolipoma (2%).
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
26                                              Angiomyolipoma, although rare, should be added to the di
27 ost common abdominal findings included renal angiomyolipoma (AML) in 43 patients (54%), enlarged abdo
28                                              Angiomyolipoma (AML) is a tumor closely related to lymph
29  mechanisms may also operate in the cells of angiomyolipoma (AML), which develops as a result of muta
30 s to recapitulate a rare kidney tumor called angiomyolipoma (AML).
31 patients with oncocytoma and 5 patients with angiomyolipoma (AML).
32 ells in the lungs, lymphatics, and the tumor angiomyolipoma (AML).
33 aturia with underlying giant bilateral renal angiomyolipomas (AML) with estimated total tumor burden
34 d by cystic degeneration of the lungs, renal angiomyolipomas (AML), and lymphatic abnormalities.
35          All nine cyst-positive patients had angiomyolipomas (AML), which were larger (p < 0.05) and
36                        Background Lipid-poor angiomyolipomas (AMLs) are challenging to differentiate
37             LOH has been documented in renal angiomyolipomas (AMLs), but loss of the wild-type allele
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
40  marker, the results were concordant between angiomyolipoma and LAM.
41 ts of tamoxifen therapy for the treatment of angiomyolipoma and lymphangioleiomyomatosis.
42 ompared the chromosome 16 LOH region between angiomyolipoma and pulmonary LAM from two patients with
43  lung (lymphangioleiomyomatosis) and kidney (angiomyolipoma and renal cell carcinoma).
44 ns, size and/or number increased in 32 (40%) angiomyolipomas and 21 (26%) cysts.
45         Serial magnetic resonance imaging of angiomyolipomas and brain lesions, computed tomography o
46        Lastly, LOH was found in 56% of renal angiomyolipomas and cardiac rhabdomyormas but in only 4%
47                                         Both angiomyolipomas and cysts occur commonly in pediatric pa
48                                              Angiomyolipomas and cysts were identified and characteri
49                                  Human renal angiomyolipomas and lymphangioleiomyomatosis also expres
50 onnexin 43, were up-regulated in TSC-related angiomyolipomas and lymphangioleiomyomatosis.
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 (
55 as been seen in two other TSC lesions: renal angiomyolipomas and pulmonary lymphangiomyomatosis.
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
59                     The vascular elements of angiomyolipomas and the giant cells of SEGAs may be reac
60 nted with symptoms due to LAM, 19 with renal angiomyolipomas, and 10 with seizures.
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
65                                              Angiomyolipomas appear to reflect novel vascular mechani
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
68                                              Angiomyolipomas are benign tumors of the kidney derived
69                                        Renal angiomyolipomas are highly vascular tumors that occur sp
70                                              Angiomyolipomas are more common than cysts and tend to b
71 determine whether the distinctive vessels of angiomyolipomas are neoplastic or reactive.
72                                              Angiomyolipomas are slow-growing tumours associated with
73                            To our knowledge, angiomyolipomas are the first benign vascular tumor in w
74  promising accuracy for detecting lipid-poor angiomyolipomas (area under the receiver operating chara
75 uating sirolimus for the treatment of kidney angiomyolipomas associated with TSC or TSC/LAM.
76 esting it could be a potential treatment for angiomyolipomas associated with tuberous sclerosis.
77 tion, the patient had no evidence of a renal angiomyolipoma at autopsy and therefore demonstrated for
78                                     In human angiomyolipomas, benign renal neoplasms often found in t
79 ion in LAM and decreases the volume of renal angiomyolipomas, but lung function declines and angiomyo
80         Tuberin (TSC2) was weak or absent in angiomyolipomas, but present in healthy kidney, whereas,
81                                    Using the angiomyolipoma cell line 621-101, MITF knockout (MITF.KO
82 yolipomas, we have generated the first human angiomyolipoma cell line by sequential introduction of S
83 rowth factor (PDGF) receptor signaling in an angiomyolipoma cell model.
84 bited by STI571, we found that SV7tert human angiomyolipoma cells are sensitive to STI571.
85 rmal pulmonary smooth muscle cells and renal angiomyolipoma cells from patients with sporadic pulmona
86 ith a model in which pulmonary LAM cells and angiomyolipoma cells have a common genetic origin.
87                                              Angiomyolipoma cells without LOH, including the endothel
88 -null tumor cells and immortalized TSC2-null angiomyolipoma cells, but not in cells with intact TSC.
89 ge T antigen and human telomerase into human angiomyolipoma cells.
90 istics of lymphangioleiomyomatosis (LAM) and angiomyolipoma cells.
91  subependymal giant cell astrocytomas, renal angiomyolipomas, cognitive impairment, and epilepsy.
92                                We found that angiomyolipomas contain five morphologically distinct ve
93 al abnormalities associated with TSC include angiomyolipoma, cysts, and renal cell carcinoma.
94                                  Further, an angiomyolipoma-derived cell line carrying biallelic TSC2
95                       Using a TSC2-deficient angiomyolipoma-derived cell line indicated that TSC2(-/-
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
100                                    Cysts and angiomyolipomas did not occur in significantly different
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
103 n of somatic TSC2 mutations in five of seven angiomyolipomas from sporadic LAM patients.
104 en expression of HIF1alpha and PTEN in renal angiomyolipomas from TSC patients.
105                            We analyzed renal angiomyolipomas, from 13 women with sporadic LAM, for LO
106            Importantly, TSC-associated renal angiomyolipomas have higher expression of LPA receptor 1
107 ore subependymal giant cell astrocytomas and angiomyolipomas, higher incidence of pharmacoresistant e
108                         Excluding lipid-rich angiomyolipoma, hypovascularity-which has high interobse
109 MITF-A) was consistently highly expressed in angiomyolipoma, immunohistochemistry showed microphthalm
110                                              Angiomyolipomas in patients with the tuberous sclerosis
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
113                                              Angiomyolipoma is a benign mesenchymal tumor composed of
114 gamma allele 2 and the development of kidney angiomyolipomas (KAMLs) in this TSC2 cohort.
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
120                                        Renal angiomyolipomas occur in approximately 50% of sporadic L
121 test was used to determine whether cysts and angiomyolipomas occurred in different subpopulations.
122 0 S6 kinase, and the ribosomal S6 protein in angiomyolipomas occurring in tuberous scierosis.
123                      Of the 28 patients with angiomyolipomas or rhabdomyomas, 16p13 LOH was detected
124          We report here that LAM lesions and angiomyolipomas overexpress urokinase-type plasminogen a
125  distinct disease subgroups were identified: angiomyolipoma-predominant TSC (cluster 1), TSC with inf
126  correlating with impaired lung function and angiomyolipoma presence.
127                                              Angiomyolipomas regressed somewhat during sirolimus ther
128 iomyolipomas, but lung function declines and angiomyolipomas regrow when treatment is discontinued, s
129 st a 50% reduction in total volume of target angiomyolipomas relative to baseline.
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
132                              We compared the angiomyolipoma response rate on everolimus with placebo
133                                          The angiomyolipoma response rate was 42% (33 of 79 [95% CI 3
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
136       A similar trend was observed in kidney angiomyolipoma size but not in pulmonary function tests.
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
141  migration of smooth muscle cells from renal angiomyolipomas to the lung.
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
144                                We found that angiomyolipoma vessel types in which LOH were detected w
145 ntiate into the separate components of renal angiomyolipomas (vessels, smooth muscle and fat).
146                             The mean (+/-SD) angiomyolipoma volume at 12 months was 53.2+/-26.6% of t
147 l to determine whether sirolimus reduces the angiomyolipoma volume in patients with the tuberous scle
148 e patients had a persistent reduction in the angiomyolipoma volume of 30% or more.
149                           Everolimus reduced angiomyolipoma volume with an acceptable safety profile,
150                             The diagnosis of angiomyolipoma was made.
151 as available, the same mutation found in the angiomyolipoma was present in the abnormal pulmonary smo
152                 To gain further insight into angiomyolipomas, we have generated the first human angio
153 ophobe carcinomas, four oncocytomas, and one angiomyolipoma were analyzed.
154                                              Angiomyolipomas were identified in 47 (80%) patients and
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
157                                        Renal angiomyolipomas, which are found in most tuberous sclero
158  We report the first case of primary orbital angiomyolipoma with negative melanocytic marker.
159                                   We studied angiomyolipomas with loss of heterozygosity (LOH) in the
160  imaging, can be confidently diagnosed as an angiomyolipoma without further diagnostic intervention.

 
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