ライフサイエンスコーパス: desmoid

1 matic APC mutation association exists in FAP desmoids.

2 tations were observed in 117 of 138 (85%) of desmoids.

3 als to UTMDACC, especially primary untreated desmoids.

4 ival was significantly poorer in 45F-mutated desmoids (23%, P < 0.0001) versus either 41A (57%) or no

5 Among 160 patients with desmoids, 72 were managed with observation, and 37 of th

6 ion has been commonly identified in sporadic desmoids although the incidence of CTNNB1 (the gene enco

7 NNB1 mutations in a large cohort of sporadic desmoids and examined whether mutation type was relevant

8 f molecular alterations in HG-FS, LG-FS, and desmoids appear to be related to biological aggressivene

9 Desmoids are a life threatening extra-colonic manifestat

10 ional study of the molecular determinates of desmoid behavior is needed to guide therapeutic selectio

11 ate for young patients with large, extremity desmoids, but surgery alone is curative for most abdomin

12 Untreated desmoids can grow, remain stable, or regress, but reliab

13 common region encompassing 48 % of published desmoid cases and 40 % of the reference population.

14 In addition, the occurrence of desmoid fibromas was strongly enhanced by p53 deficiency

15 ic polyps and extracolonic lesions including desmoid fibromas, osteomas, epidermoid cysts, and congen

16 m1 showed no influence on the development of desmoid fibromas, whereas the combination of piroxicam a

17 he differential diagnosis of GIST, including desmoid fibromatosis (0 of 17) and Schwannoma (0 of 3),

18 s with treatment-induced biologic changes in desmoid fibromatosis (DF) earlier than conventional resp

19 Desmoid fibromatosis is a rare, nonmetastatic neoplasm m

20 ed study included 17 single-lesion extremity desmoid fibromatosis patients who underwent standard-of-

21 Sarcoma Patient Advocacy Global Network, the Desmoid Foundation Italy, and the Desmoid Tumor Research

22 to colonic polyps, loss of heterozygosity in desmoids involved deletion rather than mitotic recombina

23 amined whether mutation type was relevant to desmoid outcome.

24 t treatments may be associated with improved desmoid patient outcomes.

25 This study compared a large series of desmoid patients treated at a single institution to a pr

26 was used to analyze clinical courses of 189 desmoid patients treated at The University of Texas M.D.

27 om 1995 to 2005 as compared with 189 UTMDACC desmoid patients treated between 1965 and 1994.

28 Primary or recurrent desmoid patients were identified retrospectively from an

29 enin degradation repeats, respectively, most desmoids preferentially retain two repeats (P < 0.001, c

30 by hyperintense T2 signal is associated with desmoid progression during observation and may help dist

31 of this study was to identify predictors of desmoid progression during observation.

32 y was inversely correlated with incidence of desmoid recurrence (P < 0.01).

33 Molecular determinants of desmoid recurrence remain obscure.

34 ly three-fold increase in annualized UTMDACC desmoid referral volume with significantly higher percen

35 Desmoid specimens (195 tumors from 160 patients, 1985 to

36 1 genotyping was performed on a 138-sporadic desmoid subset.

37 ions in the largest cohort of FAP-associated desmoids to date, and combined our results with previous

38 and radiomics-derived features in assessing desmoid treatment response.

39 The novel CE-SWI enhances tumor insight and desmoid treatment-response prediction by effectively sep

40 Desmoid tumor (DT) is a rare and locally aggressive mono

41 three intermediate connective tissue tumors: desmoid tumor (DT) or aggressive fibromatosis, tenosynov

42 nown regarding the molecular determinates of desmoid tumor behavior.

43 k has focused on the role of beta-catenin in desmoid tumor biology.

44 ly induces cell death on primary FAP-related desmoid tumor cells in culture.

45 In vitro models of patient desmoid tumor cells revealed a direct effect of Tazemeto

46 plex multidisciplinary management needed for desmoid tumor control may underlie significantly increas

47 nt, and penetrant genetic Xenopus tropicalis desmoid tumor models to identify and characterize drug t

48 the treatment of 10 children for progressive desmoid tumor not amenable to standard surgical or radia

49 After the transplant, the desmoid tumor recurred in the thoracic wall twice and wa

50 the 10 recipients experienced intraabdominal desmoid tumor recurrence or developed de novo visceral a

51 ly higher percentages and numbers of primary desmoid tumor referrals to UTMDACC was observed in the m

52 The term fibromatosis or desmoid tumor refers to a group of benign fibrous growth

53 twork, the Desmoid Foundation Italy, and the Desmoid Tumor Research Foundation.

54 ospective review of 189 consecutive cases of desmoid tumor treated with surgical resection, resection

55 ith stage I, II, III, and IV intra-abdominal desmoid tumor were 95%, 100%, 89%, and 76% respectively

56 (two patients) or recurrent (eight patients) desmoid tumor were treated with VBL and MTX for 2 to 35

57 rse of a patient with Gardner's syndrome and desmoid tumor who had multiple enterectomies and gradual

58 otherapy with VBL and MTX appears to control desmoid tumor without significant acute or long-term mor

59 e on the management of patients with DT, the Desmoid Tumor Working Group convened a 1-day consensus m

60 22 specimens from 19 patients diagnosed with desmoid tumor, desmoplastic fibroma, periosteal desmoid

61 moid tumor, desmoplastic fibroma, periosteal desmoid tumor, osteofibrous dysplasia, or fibrous dyspla

62 MSC cell line derived from an FAP-associated desmoid tumor, we confirmed an expected loss in the expr

63 with short-gut syndrome caused by recurrent desmoid tumor.

64 inal crypt multiplicity as well as enhancing desmoid tumorigenesis and epidermoid cyst development.

65 Purpose Desmoid tumors (aggressive fibromatosis) arise from conn

66 l of nirogacestat in adults with progressing desmoid tumors according to the Response Evaluation Crit

67 Desmoid tumors also contained a subclass of fibrocytes l

68 A cohort of 24 desmoid tumors and 25 low-grade (LG) and 14 high-grade (

69 t certain patients with FAP at high risk for desmoid tumors and could be future targets for research.

70 Medline and Embase to identify subjects with desmoid tumors and FAP.

71 Desmoid tumors and fibrosarcomas (FS) are part of a wide

72 t mortality in patients with intra-abdominal desmoid tumors and identified additional risk factors ab

73 iate between histologically similar cases of desmoid tumors and LG-FS.

74 ndications for transplantation include large desmoid tumors and other intra-abdominal tumors with rea

75 tality rate of patients with intra-abdominal desmoid tumors and to identify prognostic factors for th

76 Desmoid tumors are a group of benign, invasive, solid tu

77 The biology and natural history of desmoid tumors are an enigma.

78 Desmoid tumors are associated with a variable and unpred

79 Desmoid tumors are locally invasive fibromatous tumors,

80 Desmoid tumors are nonmalignant neoplasms of mesenchymal

81 INTRODUCTION: Intra-abdominal desmoid tumors are one of the leading causes of death in

82 Desmoid tumors are rare mesenchymal neoplasms characteri

83 Desmoid tumors are rare, locally aggressive, highly recu

84 Desmoid tumors are soft-tissue neoplasms strictly driven

85 cases can extend beyond the fibrous capsule; desmoid tumors associated with the implants; and breast

86 not metastasize; however, locally aggressive desmoid tumors can cause severe morbidity and loss of fu

87 weighted imaging (CE-SWI) for characterizing desmoid tumors can enhance the separation between fibrou

88 To test this idea, we examined 16 desmoid tumors from FAP-associated and sporadic cases, f

89 and/or radiation and diagnosis of multifocal desmoid tumors highlight the need to develop effective s

90 There is an increased risk for desmoid tumors in individuals with APC mutations between

91 The standard management of desmoid tumors is resection, but many recur locally.

92 of mesenchymal tumors, we hypothesized that desmoid tumors may arise in patients with FAP after MSCs

93 Hedgehog and Notch signaling, suggests that desmoid tumors may respond to therapies targeting these

94 Patients and Methods Seventeen patients with desmoid tumors received PF-03084014 150 mg orally twice

95 Treatment of desmoid tumors remains a challenge, but new chemotherapy

96 Together, our findings argue that desmoid tumors result from the growth of MSCs in a wound

97 In contrast, desmoid tumors showed a normal phenotype with these mark

98 th the increase of tumor aggressiveness from desmoid tumors to LG-FS to HG-FS.

99 Desmoid tumors typically have a stable genome with beta-

100 A total of 154 patients with intra-abdominal desmoid tumors were included in the study.

101 fit in patients with refractory, progressive desmoid tumors who receive long-term treatment.

102 gut with duodenal adenomatosis and extensive desmoid tumors with pancreaticoduodenal involvement dict

103 In desmoid tumors, aggressive attempts at achieving negativ

104 Patients with atypical lipomatous tumors, desmoid tumors, dermatofibrosarcoma protuberans, angiosa

105 Given the variable clinical course of desmoid tumors, the interpretation of factors classicall

106 current lack of effective treatment against desmoid tumors, we advocate that endostatin gene therapy

107 ctively followed 206 patients with extremity desmoid tumors.

108 d quality of life in adults with progressing desmoid tumors.

109 n factor CREB3L1 as genetic dependencies for desmoid tumors.

110 ents with recurrent, refractory, progressive desmoid tumors.

111 usion, CTNNB1 mutations are highly common in desmoid tumors.

112 luding colorectal cancer, breast cancer, and desmoid tumors.

113 familial adenomatous polyposis (FAP)-related desmoid tumors.

114 and in 29% of HG-FS cases but only in 4% of desmoid tumors.

115 and trisomy 20 are nonrandom aberrations in desmoid tumors.

116 margins is the treatment of choice for most desmoid tumors.

117 levels of beta-catenin signalling levels in desmoid tumour cells.

118 inical activity in patients with progressive desmoid tumours and could be a valid treatment option in

119 Desmoid tumours are locally aggressive tumours associate

120 nrolled adults (>=18 years) with progressive desmoid tumours, normal organ function and centrally doc

121 y or combination chemotherapy in progressive desmoid tumours.

122 expression is a common feature in aggressive desmoid tumours.

123 Many women who present with desmoid-type fibromatosis (DF) have had a recent pregnan

124 r targeted therapies are used for refractory desmoid-type fibromatosis (DF), but occasionally cause s

125 this case series, patients (<25 years) with desmoid-type fibromatosis from 57 centres in eight count

126 Vbl) and methotrexate (Mtx) in children with desmoid-type fibromatosis that is recurrent or not amena

127 INTERPRETATION: In paediatric patients with desmoid-type fibromatosis, the EpSSG conservative strate

128 ur progression)-for paediatric patients with desmoid-type fibromatosis.

129 and Mtx are well tolerated in children with desmoid-type fibromatosis.

130 g 1982-2011 for primary or locally recurrent desmoids were identified from a single-institution prosp

131 Desmoids were treated surgically in 495 patients (median

132 In addition, most desmoids with two APC hits (87%, 26/30) had one mutated