ライフサイエンスコーパス: angiomyolipoma

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 transitional cell carcinoma, and one was an angiomyolipoma.

7 tor, similar to lymphangioleiomyomatosis and angiomyolipoma.

8 ignal transduction pathways underlying renal angiomyolipomas.

9 TSC2 LOH was detected in seven (54%) of the angiomyolipomas.

10 he TSC2 gene occurs in 60% of TSC-associated angiomyolipomas.

11 ssor model for the pathogenesis of SEGAs and angiomyolipomas.

12 smooth muscle, fat, and vessels) of six TSC2 angiomyolipomas.

13 th muscle and fat but not the vessels of two angiomyolipomas.

14 Women with LAM can also develop renal angiomyolipomas.

15 chylous effusions, lymphangioleiomyomas, and angiomyolipomas.

16 n benign tumors, neurological disorders, and angiomyolipomas.

17 d p70 S6 kinase and p56 were present only in angiomyolipomas.

18 hose with cysts and 9.2 years for those with angiomyolipomas.

19 TSC2 mutations cause LAM in patients without angiomyolipomas.

20 were hemorrhagic cysts (5%), and one was an angiomyolipoma (2%).

21 nts aged 18 years or older with at least one angiomyolipoma 3 cm or larger in its longest diameter (d

22 rocytoma (37.1% vs. 14.6%; P = 0.018), renal angiomyolipoma (60.0% vs. 27.1%; P = 0.003), cognitive i

23 Angiomyolipoma, although rare, should be added to the di

24 ost common abdominal findings included renal angiomyolipoma (AML) in 43 patients (54%), enlarged abdo

25 Angiomyolipoma (AML) is a tumor closely related to lymph

26 mechanisms may also operate in the cells of angiomyolipoma (AML), which develops as a result of muta

27 patients with oncocytoma and 5 patients with angiomyolipoma (AML).

28 ells in the lungs, lymphatics, and the tumor angiomyolipoma (AML).

29 aturia with underlying giant bilateral renal angiomyolipomas (AML) with estimated total tumor burden

30 d by cystic degeneration of the lungs, renal angiomyolipomas (AML), and lymphatic abnormalities.

31 All nine cyst-positive patients had angiomyolipomas (AML), which were larger (p < 0.05) and

32 LOH has been documented in renal angiomyolipomas (AMLs), but loss of the wild-type allele

33 thoracic duct dilatation, hepatic and renal angiomyolipomas (AMLs), lymphangioleiomyoma (LALM), asci

34 In this case, one would predict that the angiomyolipoma and LAM cells would have identical LOH pa

35 marker, the results were concordant between angiomyolipoma and LAM.

36 ts of tamoxifen therapy for the treatment of angiomyolipoma and lymphangioleiomyomatosis.

37 ompared the chromosome 16 LOH region between angiomyolipoma and pulmonary LAM from two patients with

38 ns, size and/or number increased in 32 (40%) angiomyolipomas and 21 (26%) cysts.

39 Serial magnetic resonance imaging of angiomyolipomas and brain lesions, computed tomography o

40 Lastly, LOH was found in 56% of renal angiomyolipomas and cardiac rhabdomyormas but in only 4%

41 Both angiomyolipomas and cysts occur commonly in pediatric pa

42 Angiomyolipomas and cysts were identified and characteri

43 Human renal angiomyolipomas and lymphangioleiomyomatosis also expres

44 onnexin 43, were up-regulated in TSC-related angiomyolipomas and lymphangioleiomyomatosis.

45 nts had TSC2 mutations and TSC2 LOH in their angiomyolipomas and pulmonary LAM cells but not in norma

46 atic mutations in the TSC2 gene occur in the angiomyolipomas and pulmonary LAM cells of women with sp

47 childhood, but some findings, notably renal angiomyolipomas and pulmonary lymphangioleiomyomatosis (

48 as been seen in two other TSC lesions: renal angiomyolipomas and pulmonary lymphangiomyomatosis.

49 sclerosis complex and to reduce the size of angiomyolipomas and stabilize lung function in humans.

50 of typically vascularised tumours including angiomyolipomas and subependymal giant cell astrocytomas

51 most frequent tumors in TSC patients, renal angiomyolipomas and subependymal giant cell astrocytomas

52 The vascular elements of angiomyolipomas and the giant cells of SEGAs may be reac

53 nted with symptoms due to LAM, 19 with renal angiomyolipomas, and 10 with seizures.

54 e, 34% (72-48 units) for predominately fatty angiomyolipomas, and 39% (85-52 units) for vertebral hem

55 -3 was increased in TSC-related skin tumors, angiomyolipomas, and lymphangioleiomyomatosis with serum

56 (including 18 oncocytomas, seven lipid-poor angiomyolipomas, and one hemangioblastoma) and 68 malign

57 ; 14 (7%), oncocytomas; six (3%), lipid-poor angiomyolipomas; and 16 (8%), other or unclassified rena

58 Angiomyolipomas appear to reflect novel vascular mechani

59 rin heterozygous mice and from a human renal angiomyolipoma are highly sensitive to PDGFR antagonists

60 onary lymphangioleiomyomatosis and abdominal angiomyolipoma are related lesions for which there is no

61 Angiomyolipomas are benign tumors of the kidney derived

62 Renal angiomyolipomas are highly vascular tumors that occur sp

63 Angiomyolipomas are more common than cysts and tend to b

64 determine whether the distinctive vessels of angiomyolipomas are neoplastic or reactive.

65 Angiomyolipomas are slow-growing tumours associated with

66 To our knowledge, angiomyolipomas are the first benign vascular tumor in w

67 uating sirolimus for the treatment of kidney angiomyolipomas associated with TSC or TSC/LAM.

68 esting it could be a potential treatment for angiomyolipomas associated with tuberous sclerosis.

69 tion, the patient had no evidence of a renal angiomyolipoma at autopsy and therefore demonstrated for

70 In human angiomyolipomas, benign renal neoplasms often found in t

71 ion in LAM and decreases the volume of renal angiomyolipomas, but lung function declines and angiomyo

72 Tuberin (TSC2) was weak or absent in angiomyolipomas, but present in healthy kidney, whereas,

73 yolipomas, we have generated the first human angiomyolipoma cell line by sequential introduction of S

74 rowth factor (PDGF) receptor signaling in an angiomyolipoma cell model.

75 bited by STI571, we found that SV7tert human angiomyolipoma cells are sensitive to STI571.

76 rmal pulmonary smooth muscle cells and renal angiomyolipoma cells from patients with sporadic pulmona

77 ith a model in which pulmonary LAM cells and angiomyolipoma cells have a common genetic origin.

78 Angiomyolipoma cells without LOH, including the endothel

79 -null tumor cells and immortalized TSC2-null angiomyolipoma cells, but not in cells with intact TSC.

80 ge T antigen and human telomerase into human angiomyolipoma cells.

81 istics of lymphangioleiomyomatosis (LAM) and angiomyolipoma cells.

82 subependymal giant cell astrocytomas, renal angiomyolipomas, cognitive impairment, and epilepsy.

83 We found that angiomyolipomas contain five morphologically distinct ve

84 Further, an angiomyolipoma-derived cell line carrying biallelic TSC2

85 ied Rapamycin-dependent miRNA in LAM patient angiomyolipoma-derived cells using two separate screens.

86 Cysts and angiomyolipomas did not occur in significantly different

87 ns, some features (grade 2-4 kidney cysts or angiomyolipomas, forehead plaques, retinal hamartomas, a

88 2 loss of heterozygosity in 7 of 13 (54%) of angiomyolipomas from sporadic LAM patients, suggesting t

89 n of somatic TSC2 mutations in five of seven angiomyolipomas from sporadic LAM patients.

90 en expression of HIF1alpha and PTEN in renal angiomyolipomas from TSC patients.

91 We analyzed renal angiomyolipomas, from 13 women with sporadic LAM, for LO

92 ore subependymal giant cell astrocytomas and angiomyolipomas, higher incidence of pharmacoresistant e

93 Excluding lipid-rich angiomyolipoma, hypovascularity-which has high interobse

94 Angiomyolipomas in patients with the tuberous sclerosis

95 uggested to contribute lower risk for kidney angiomyolipomas in patients with TSC2 gene mutations.

96 We tested this hypothesis in eight renal angiomyolipomas in which the loss of tuberous sclerosis

97 Angiomyolipoma is a benign mesenchymal tumor composed of

98 gamma allele 2 and the development of kidney angiomyolipomas (KAMLs) in this TSC2 cohort.

99 clinical decisions related to the fact that angiomyolipomas larger than 4 cm in diameter are more ap

100 n = 66) and hyperattenuating (n = 28) cysts, angiomyolipomas (n = 18), and solid enhancing lesions (n

101 us ascites (n = 3), complications from renal angiomyolipomas (n = 4), and recurrent LAM (n = 1).

102 s measured included adipose tissue (n = 10), angiomyolipomas (n = 8), and vertebral hemangiomas (n =

103 ese cells show phenotypic characteristics of angiomyolipomas, namely differentiation markers of smoot

104 Renal angiomyolipomas occur in approximately 50% of sporadic L

105 test was used to determine whether cysts and angiomyolipomas occurred in different subpopulations.

106 0 S6 kinase, and the ribosomal S6 protein in angiomyolipomas occurring in tuberous scierosis.

107 Of the 28 patients with angiomyolipomas or rhabdomyomas, 16p13 LOH was detected

108 We report here that LAM lesions and angiomyolipomas overexpress urokinase-type plasminogen a

109 correlating with impaired lung function and angiomyolipoma presence.

110 Angiomyolipomas regressed somewhat during sirolimus ther

111 iomyolipomas, but lung function declines and angiomyolipomas regrow when treatment is discontinued, s

112 st a 50% reduction in total volume of target angiomyolipomas relative to baseline.

113 2(-/-) ASM cells, derived from a human renal angiomyolipoma, require epidermal growth factor (EGF) fo

114 as the proportion of patients with confirmed angiomyolipoma response of at least a 50% reduction in t

115 We compared the angiomyolipoma response rate on everolimus with placebo

116 The angiomyolipoma response rate was 42% (33 of 79 [95% CI 3

117 nohistochemical stains of both LAM and renal angiomyolipoma showed positive immunoreactivity for hama

118 elevated at baseline, correlates with kidney angiomyolipoma size at baseline and 12 months, and decre

119 A similar trend was observed in kidney angiomyolipoma size but not in pulmonary function tests.

120 ponse to treatment with sirolimus and kidney angiomyolipoma size in patients with TSC, but confirmati

121 to be hypervascular, chromophobe lesions and angiomyolipomas tended to enhance moderately, and papill

122 tion, %SI change was significantly higher in angiomyolipomas than in clear cell carcinomas, but only

123 t and benign renal lesions: in patients with angiomyolipoma the ADC value was 2.36+/-0.32x10(-3) mm(2

124 migration of smooth muscle cells from renal angiomyolipomas to the lung.

125 lid small renal masses, excluding lipid-rich angiomyolipomas, underwent qualitative contrast-enhanced

126 We found that angiomyolipoma vessel types in which LOH were detected w

127 ntiate into the separate components of renal angiomyolipomas (vessels, smooth muscle and fat).

128 The mean (+/-SD) angiomyolipoma volume at 12 months was 53.2+/-26.6% of t

129 l to determine whether sirolimus reduces the angiomyolipoma volume in patients with the tuberous scle

130 e patients had a persistent reduction in the angiomyolipoma volume of 30% or more.

131 Everolimus reduced angiomyolipoma volume with an acceptable safety profile,

132 The diagnosis of angiomyolipoma was made.

133 as available, the same mutation found in the angiomyolipoma was present in the abnormal pulmonary smo

134 To gain further insight into angiomyolipomas, we have generated the first human angio

135 ophobe carcinomas, four oncocytomas, and one angiomyolipoma were analyzed.

136 Angiomyolipomas were identified in 47 (80%) patients and

137 imaging for the diagnosis of RCCs (excluding angiomyolipomas) were 0.856, 86%, and 80%, respectively.

138 ehead plaques, retinal hamartomas, and liver angiomyolipomas) were very rare or not seen at all in TS

139 Renal angiomyolipomas, which are found in most tuberous sclero

140 We report the first case of primary orbital angiomyolipoma with negative melanocytic marker.

141 We studied angiomyolipomas with loss of heterozygosity (LOH) in the

142 imaging, can be confidently diagnosed as an angiomyolipoma without further diagnostic intervention.