ライフサイエンスコーパス: neurofibroma

1 n with lymphadenopathy, one with a plexiform neurofibroma).

2 rcomas) compared to benign tumors (cutaneous neurofibromas).

3 e NF1 and a clinically significant plexiform neurofibroma.

4 xpression of many cytokines overexpressed in neurofibroma.

5 ated a patient with a debilitating plexiform neurofibroma.

6 atient with a nonresectable and debilitating neurofibroma.

7 back 11 years previously and diagnosed as a neurofibroma.

8 The most common types include schwannoma and neurofibroma.

9 and fibroblastoid cells derived from an NF1 neurofibroma.

10 se model of neurofibromatosis type 1-related neurofibroma.

11 measure the change in size of the plexiform neurofibroma.

12 yeloid leukemia, optic glioma, and plexiform neurofibroma.

13 arly relevant to medical management of these neurofibromas.

14 traits to predict the presence of paraspinal neurofibromas.

15 gain in Nf1(+/-) mast cell degranulation in neurofibromas.

16 over-expressed in MPNST compared with benign neurofibromas.

17 monitor for the malignant transformation of neurofibromas.

18 order characterized by complex tumors called neurofibromas.

19 ait spots, skinfold freckling, and cutaneous neurofibromas.

20 synthesize excessive collagen, a hallmark of neurofibromas.

21 eurofibromas or clinically obvious plexiform neurofibromas.

22 fibromatosis type 1 (NF1) -related plexiform neurofibromas.

23 ficantly altered cytokine gene expression in neurofibromas.

24 heral nerve sheath tumors (MPNST) and benign neurofibromas.

25 and the S100(+) cells from each of 9 benign neurofibromas.

26 elop benign Schwann cell (SC) tumours called neurofibromas.

27 causes abnormalities characteristic of human neurofibromas.

28 ed in 11 MPNST samples from 8 patients and 7 neurofibromas.

29 ozygous for a mutation in Nf1 do not develop neurofibromas.

30 ing the behavior of Schwann cells from human neurofibromas.

31 ne-transmitted NF1 mutation develop multiple neurofibromas.

32 X1 protein overexpression in human plexiform neurofibromas.

33 SCs and SCP in the context of mice that form neurofibromas.

34 ity of specific cell type that gives rise to neurofibromas.

35 kt expression in grade 3 PNSTs compared with neurofibromas.

36 s, suggesting that these sarcomas arise from neurofibromas.

37 s for children with orbitotemporal plexiform neurofibromas.

38 o demonstrated ciliary body involvement with neurofibromas.

39 schwannomas, 2 of 2 medulloblastomas, 1 of 1 neurofibroma, 1 of 2 neuronoglial tumors, 2 of 3 ependym

40 erized by the development of numerous benign neurofibromas, a small subset of which progress to malig

41 ng by methylation were not identified in the neurofibromas analyzed.

42 axon-glial interactions, characteristics of neurofibroma and are hypoalgesic.

43 Schwann cells had a significant increase in neurofibroma and grade 3 PNST (MPNST) formation compared

44 the cell of origin for murine Nf1 plexiform neurofibroma and leverage this finding to develop a plat

45 n useful for identifying novel genes driving neurofibroma and MPNST pathogenesis.

46 nt reduced aberrantly proliferating cells in neurofibroma and MPNST, prolonged survival of mice impla

47 Common insertion site (CIS) analysis of 269 neurofibromas and 106 MPNSTs identified 695 and 87 sites

48 roblasts are a major cellular constituent in neurofibromas and are a source of collagen that constitu

49 ncreased senescence observed in human benign neurofibromas and colon adenoma lesions in vivo.

50 e are intriguing links between the growth of neurofibromas and levels of circulating hormones: neurof

51 disorder of the nervous system resulting in neurofibromas and malignant peripheral nerve sheath tumo

52 matosis type 1 (NF1) patients develop benign neurofibromas and malignant peripheral nerve sheath tumo

53 ts are predisposed to certain tumors such as neurofibromas and may associate with vascular disorder.

54 eas loss in adulthood caused large plexiform neurofibromas and morbidity beginning 4 months after ons

55 the NF1 gene, is characterized clinically by neurofibromas and more rarely by neurofibrosarcomas.

56 activation was sustained in mouse and human neurofibromas and MPNST.

57 es transcriptome analyses of mouse and human neurofibromas and MPNSTs and identified global negative

58 nd may be an important therapeutic target in neurofibromas and MPNSTs.

59 Neurofibromas and optic pathway gliomas result from NF1

60 elopment of nervous system tumors, including neurofibromas and pilocytic astrocytomas.

61 Neurofibromas and schwannomas are benign peripheral nerv

62 Neurofibromas and schwannomas are benign Schwann cell-de

63 clinical characteristics and pathogenesis of neurofibromas and schwannomas in the neurofibromatoses.

64 Several cases of multiple isolated neurofibromas and spinal neurofibromas were reported.

65 Major superficial plexiform neurofibromas and symptomatic spinal neurofibromas were

66 f the disease are multiple and severe due to neurofibromas and their occasional malignant transformat

67 ncisional biopsy confirmed the presence of a neurofibroma, and because of the extent of the lesion, t

68 sitive Schwann cells of dermal and plexiform neurofibromas, and in endothelial cells of tumor blood v

69 Macrophages are abundant in neurofibromas, and macrophage targeted interventions may

70 e prone to optic gliomas, malignant gliomas, neurofibromas, and malignant peripheral nerve sheath tum

71 uch as schwannomatosis and multiple isolated neurofibromas, and malignant peripheral nerve sheath tum

72 ibroblasts, and tumor Schwann cells of skin, neurofibromas, and MPNSTs.

73 ipheral nerve sheath tumors (MPNSTs), benign neurofibromas, and normal Schwann cells.

74 expression is independent of the presence of neurofibromas, and thus appears to be associated with mu

75 Benign peripheral nerve tumors called neurofibromas are a major source of morbidity for patien

76 Neurofibromas are benign peripheral nerve tumors driven

77 Neurofibromas are benign tumors comprised primarily of S

78 Neurofibromas are benign tumors of the peripheral nerve

79 OPGs and orbitofacial neurofibromas are clinically diverse.

80 Plexiform neurofibromas are common NF1 tumors carrying a risk of m

81 Neurofibromas are common tumors found in neurofibromatos

82 Neurofibromas are complex tumors and composed mainly of

83 Neurofibromas are complex tumors composed of axonal proc

84 Neurofibromas are highly vascularized, and recent studie

85 Plexiform neurofibromas are one of the most common tumors encounte

86 Plexiform neurofibromas are pathognomonic of the disease and consi

87 Plexiform neurofibromas are peripheral nerve sheath tumors initiat

88 Dermal and plexiform neurofibromas are peripheral nerve sheath tumors that ar

89 Plexiform neurofibromas are slow growing benign tumors that are hi

90 Plexiform neurofibromas are slow-growing chemoradiotherapy-resista

91 ay gliomas (OPGs) and orbitofacial plexiform neurofibromas are two of the more common ophthalmic mani

92 Neurofibromas arise from the Schwann cell lineage follow

93 Multiple pharmaceuticals have shown neurofibroma arrest in vitro and are in phase II clinica

94 Dermal and plexiform neurofibromas as well as malignant peripheral nerve shea

95 se Schwann cell progenitors (SCPs) and mouse neurofibromas at the messenger RNA and protein levels.

96 rotein profiling, and show that treatment of neurofibroma-bearing mice with polyethylene glycolyated

97 elieved to be the primary pathogenic cell in neurofibromas because they harbor biallelic neurofibromi

98 fibromatosis type 1 and inoperable plexiform neurofibromas benefited from long-term dose-adjusted tre

99 Patients commonly present with plexiform neurofibromas, benign but debilitating growths that can

100 s (NF1) is characterized by the formation of neurofibromas, benign tumors composed mainly of Schwann

101 ral nerve sheath tumors (MPNSTs) from benign neurofibromas (BNFs) in patients with neurofibromatosis

102 -Ras2/TC21 displayed a delay in formation of neurofibromas but an acceleration in formation of brain

103 sis type 1 (NF1) patients are predisposed to neurofibromas but the driver(s) that contribute to neuro

104 The vein did not separate the gland from the neurofibroma, but this tumor was medial to the gland; th

105 We validated type-I interferon expression in neurofibroma by protein profiling, and show that treatme

106 tablished from 10 dermal and eight plexiform neurofibromas by selective subculture using glial growth

107 Neurofibromas can cause extensive destruction of alveola

108 demonstrate that fibro-blasts isolated from neurofibromas carried at least one normal NF1 allele and

109 lignant transformation of internal plexiform neurofibromas carries a poor prognosis, in part because

110 To determine which neurofibroma cell type has altered Ras-GTP regulation, w

111 In dissociated neurofibroma cells from NF1 patients, Ras-GTP was elevat

112 Consistently, knockdown of KANK1 in neurofibroma cells promoted cell growth.

113 signaling results in development of multiple neurofibromas, complex tumors of the peripheral nerves.

114 1 patients develop peripheral nerve tumors (neurofibromas) composed mainly of Schwann cells and fibr

115 odels have shed light on the pathogenesis of neurofibromas confirming that the Schwann cell initiates

116 Neurofibromas contained cells with Remak bundle disrupti

117 s to develop benign peripheral nerve tumors (neurofibromas) containing Schwann cells (SCs).

118 timulate the growth of human endothelial and neurofibroma-derived cells.

119 f neurofibromin-deficient ECs in response to neurofibroma-derived growth factors both in vitro and in

120 mplete loss of Pten dramatically accelerated neurofibroma development and led to the development of h

121 This paradigm is relevant to understanding neurofibroma development in neurofibromatosis type I pat

122 fic mutation to the suppression of cutaneous neurofibroma development is unknown.

123 arboring the putative locus that exacerbates neurofibroma development to 1 Mb.

124 rstanding early cellular events that dictate neurofibroma development, as well as for the development

125 is necessary but not sufficient to initiate neurofibroma development.

126 sis, asserting that tumorigenic cells in the neurofibroma do not arise and grow in isolation.

127 s present in approximately half of the total neurofibromas examined.

128 xpressed PR, whereas only a minority (5%) of neurofibromas expressed estrogen receptor.

129 The majority (75%) of neurofibromas expressed PR, whereas only a minority (5%)

130 of the Schwann cell lineage can give rise to neurofibromas following loss of NF1.

131 We examined 59 human neurofibromas for the expression of estrogen and progest

132 loss of heterozygosity is not sufficient for neurofibroma formation and Nf1 haploinsufficiency in at

133 neurofibromin-deficient SCs are involved in neurofibroma formation and, by selective subculture, pro

134 ibromas but the driver(s) that contribute to neurofibroma formation are not fully understood.

135 ferent cell types and of elevated Ras-GTP in neurofibroma formation are unclear.

136 Previous studies found that plexiform neurofibroma formation in a mouse model requires biallel

137 Our results raise the possibility that neurofibroma formation in individuals with neurofibromat

138 e approaches to develop therapies to prevent neurofibroma formation in NF1 patients.

139 lls augments angiogenesis, which may promote neurofibroma formation in NF1.Oncogene advance online pu

140 etion of Runx1 in SCs and SCPs delayed mouse neurofibroma formation in vivo.

141 ls that closely recapitulate human plexiform neurofibroma formation indicate that tumorigenesis neces

142 To understand whether neurofibroma formation is possible after birth, we induc

143 c animals have localized rather than diffuse neurofibroma formation, however, suggests that additiona

144 In mouse models of plexiform neurofibroma formation, Nf1 haploinsufficient mast cells

145 mbryonic and adult Schwann cells can lead to neurofibroma formation.

146 identify a novel molecular target to inhibit neurofibroma formation.

147 of driving complex changes characteristic of neurofibroma formation.

148 mouse, loss of Nf1 in the SC lineage causes neurofibroma formation.

149 s necessary, but not sufficient, to initiate neurofibroma formation.

150 mast cells that is likely to be important in neurofibroma formation.

151 ed the correlation between EGFR activity and neurofibroma formation.

152 s confirming that the Schwann cell initiates neurofibroma formation.

153 other neural crest derivatives contribute to neurofibroma formation.

154 Trauma has been proposed to trigger neurofibroma formation.

155 tic or epigenetic events may be required for neurofibroma formation.

156 tion cells, suggesting RUNX1 might relate to neurofibroma formation.

157 cating the hematopoietic system in plexiform neurofibroma genesis, delineate the physiology of stem c

158 a role for the microenvironment in plexiform neurofibroma genesis.

159 t progesterone may play an important role in neurofibroma growth and suggest that antiprogestins may

160 ned Ras/Raf/MEK/ERK signaling contributes to neurofibroma growth in a neurofibromatosis mouse model (

161 ole for MK as a mediator of angiogenesis and neurofibroma growth in NF1.

162 Plexiform neurofibromas have an intermediate range of levels of NF

163 naling in malignant transformation of benign neurofibromas have been described.

164 ther examine the role of these mutant SCs in neurofibroma histogenesis.

165 tal and tumour syndromes that include benign neurofibromas, hyperpigmentation of melanocytes and hama

166 ygous for the Nf1 gene promote the growth of neurofibromas in a mouse model of neurofibromatosis and

167 implanted with human MPNST cells, and shrank neurofibromas in more than 80% of mice tested.

168 On occasion, neurofibromas in neurofibromatosis type 1 may be present

169 Neurofibromas in NF1 and schwannomas in NF2 or schwannom

170 Benign plexiform neurofibromas in NF1 patients can transform spontaneousl

171 rofibrosarcomas in three patients, cutaneous neurofibromas in one patient and a plexiform neuroma in

172 ib mesylate could be used to treat plexiform neurofibromas in patients with NF1.

173 e burden of clinically significant plexiform neurofibromas in patients with NF1.

174 owth is caused by the formation of plexiform neurofibromas in the connective tissue of the gingiva.

175 characterized by the formation of cutaneous neurofibromas infiltrated with a high density of degranu

176 dominant disorder characterized by cutaneous neurofibromas infiltrated with large numbers of mast cel

177 the adult and narrow the range of potential neurofibroma-initiating cell populations.

178 cally, expression of CNP-hEGFR increased SCP/neurofibroma-initiating cell self-renewal, a surrogate f

179 comparison of microarray gene lists on human neurofibroma-initiating cells and developed neurofibroma

180 ing in Nf1+/- hematopoietic cells diminishes neurofibroma initiation and progression.

181 we identified RUNX1 overexpression in human neurofibroma initiation cells, suggesting RUNX1 might re

182 gest that Runx1 has an important role in Nf1 neurofibroma initiation, and inhibition of RUNX1 functio

183 To test if EGFR activity affects neurofibroma initiation, size, and/or number, we studied

184 CP), which have been implicated in plexiform neurofibroma initiation.

185 of tyrosine kinase expression in SCPs modify neurofibroma initiation.

186 Extent of orbitotemporal plexiform neurofibroma involvement was assessed clinically and wit

187 The management of the orbitofacial neurofibroma is primarily surgical, and the systematic s

188 sulted in the development of small plexiform neurofibromas late in life, whereas loss in adulthood ca

189 f scid mice consistently produced persistent neurofibroma-like tumors with diffuse and often extensiv

190 Orbitotemporal plexiform neurofibroma location was classified as isolated eyelid

191 iological approach to targeted therapies for neurofibromas, malignant peripheral nerve sheath tumours

192 of the prevalent heterozygous cells found in neurofibromas, mast cells and fibroblasts interact direc

193 Thus, the plexiform neurofibroma microenvironment involves a tumor/stromal i

194 cells underpin inflammation in the plexiform neurofibroma microenvironment of neurofibromatosis type

195 hematopoietic contributions to the plexiform neurofibroma microenvironment, and highlight application

196 opoietic cells and their contribution to the neurofibroma microenvironment, and highlight the applica

197 ECs as a potential therapeutic target in the neurofibroma microenvironment.

198 n for these tumors and report a non-germline neurofibroma model for preclinical drug screening to ide

199 f NF1 children with orbitotemporal plexiform neurofibromas, most commonly because of ptosis and aniso

200 mouse model that accurately models plexiform neurofibroma-MPNST progression in humans would facilitat

201 elopment of benign neurofibromas; subsequent neurofibroma-MPNST progression is caused by aberrant gro

202 0)-GGFbeta3 mice represent a robust model of neurofibroma-MPNST progression useful for identifying no

203 er P(0)-GGFbeta3 mice accurately model human neurofibroma-MPNST progression, cohorts of these animals

204 1 (NF1) are predisposed to develop multiple neurofibromas (NFs) and are at risk for transformation o

205 peripheral nerve sheath tumors (MPNSTs) and neurofibromas (NFs).

206 peripheral nerve sheath tumors (MPNSTs) and neurofibromas (NFs).

207 ither externally visible cutaneous/plexiform neurofibromas nor other tumors.

208 Genetic studies demonstrate that in neurofibromas, nullizygous loss of Nf1 in Schwann cells

209 FR mice versus heterozygous littermates, and neurofibroma number and size increased when CNP-hEGFR wa

210 Neurofibroma number increased in homozygous CNP-hEGFR mi

211 ng in Nf1(fl/fl);DhhCre;Wa2/+ mice decreased neurofibroma number.

212 In contrast, neurofibromas occur with high penetrance in mice in whic

213 Neurofibromas occur with high penetrance in mice in whic

214 fibromas and levels of circulating hormones: neurofibromas often first appear around the time of pube

215 al structures (orbital-periorbital plexiform neurofibroma [OPPN]) can result in significant visual lo

216 Paraffin-embedded neurofibroma or MPNST blocks were assembled in a tissue

217 ons affecting genes not previously linked to neurofibroma or MPNST pathogenesis.

218 delAAT (p.990delM) mutation but no cutaneous neurofibromas or clinically obvious plexiform neurofibro

219 potential therapeutic treatment strategy for neurofibroma patients.

220 meningiomas, ependymomas, astrocytomas, and neurofibromas), peripheral neuropathy, ophthalmological

221 n genetic disorder characterized by multiple neurofibromas, peripheral nerve tumors containing mainly

222 t-tissue tumors that occur in this syndrome (neurofibromas, plexiform neuromas and neurofibrosarcomas

223 Although plexiform neurofibroma (PN) is thought to represent a benign neopl

224 athognomonic finding of NF1 is the plexiform neurofibroma (PN), a benign, likely congenital tumor tha

225 Plexiform neurofibromas (PNs) involving the eyelid, orbit, periorb

226 Within neurofibromas, PR was expressed by non-neoplastic tumor-

227 These neurofibromas presumably arise from NF1 inactivation in

228 umor microenvironment is sufficient to allow neurofibroma progression in the context of Schwann cell

229 ways are major tumor-suppressive barriers to neurofibroma progression.

230 f1+/- fibroblasts and fibroblasts from human neurofibromas proliferate and synthesize excessive colla

231 c and genetic implications, the diagnosis of neurofibroma requires appropriate medical referral.

232 The development of neurofibromas requires loss of both Nf1 alleles in Schwa

233 We verified that neurofibroma SC conditioned medium contains macrophage c

234 tect full-length neurofibromin in any of the neurofibroma SC cultures, indicating that neurofibromin-

235 wed elevated Ras-GTP, unexpectedly revealing neurofibroma Schwann cell heterogeneity.

236 neurofibroma-initiating cells and developed neurofibroma Schwann cells (SCs) we identified RUNX1 ove

237 Some neurofibroma Schwann cells aberrantly express the epider

238 ctivity is detectably increased in only some neurofibroma Schwann cells and suggest that neurofibromi

239 Of the different cell types found in neurofibromas, Schwann cells usually provide between 40

240 as peripheral nervous system tumors such as neurofibromas, schwannomas, and malignant peripheral ner

241 MK was induced in human neurofibromas, schwannomas, and various nervous system t

242 F1) gene mutations lead to increased risk of neurofibromas, schwannomas, low grade, pilocytic optic p

243 Among these, multiple hybrid neurofibromas/schwannomas may also appear, not yet being

244 e 22 in almost half of all cases with hybrid neurofibromas/schwannomas of patients with multiple peri

245 re performed molecular analysis of 22 hybrid neurofibromas/schwannomas using array comparative genomi

246 44% of tumors of tested patients with hybrid neurofibromas/schwannomas.

247 tive measurement of orbitotemporal plexiform neurofibroma size, and larger volumes were associated wi

248 raction inhibitor, Ro5-3335, decreased mouse neurofibroma sphere number in vitro.

249 changes, resulting in development of benign neurofibromas; subsequent neurofibroma-MPNST progression

250 Nascent MPNSTs were identified within neurofibromas, suggesting that these sarcomas arise from

251 kable for an early age at onset of cutaneous neurofibromas, suggesting the deletion of an additional

252 Sixteen MPNSTs but none of the neurofibromas tested were found to have somatic mutation

253 quirement in formation of TGF-beta-dependent neurofibromas that arise in Nf1-deficient mice.

254 al loss results in the development of benign neurofibromas that can progress to malignancy.

255 rapeutic options for patients with plexiform neurofibromas that cannot be surgically removed because

256 atosis type 1 (NF1) develop benign plexiform neurofibromas that frequently progress to become maligna

257 sion and action of NRG-1 in human MPNSTs and neurofibromas, the benign precursor lesions from which M

258 heral nerves might underlie the formation of neurofibromas, the hallmark of neurofibromatosis.

259 oietic effector cells long known to permeate neurofibroma tissue, mediate key mitogenic signals that

260 e gain-in-functions, and mast cells permeate neurofibroma tissue.

261 The genetic evolution from a benign neurofibroma to a malignant sarcoma in patients with neu

262 rophages from wild-type and mutant nerve and neurofibroma to identify candidate pathways involved in

263 fibromatosis type 1 and inoperable plexiform neurofibromas to determine the maximum tolerated dose an

264 luence the multicellular microenvironment of neurofibromas to inhibit the development and/or progress

265 addition, malignant progression of plexiform neurofibromas to malignant peripheral nerve sheath tumor

266 g may contribute to the conversion of benign neurofibromas to MPNSTs.

267 ound that EGFR overexpression promotes mouse neurofibroma transformation to aggressive MPNST (GEM-PNS

268 can rapidly recapitulate the onset of human neurofibroma tumorigenesis and the progression to MPNSTs

269 es with enhanced susceptibility to plexiform neurofibroma tumorigenesis.

270 nts to mast cells as crucial contributors to neurofibroma tumorigenesis.

271 osis, which is characterized by formation of neurofibromas (tumors of peripheral nerves).

272 h NF1 have cutaneous, diffuse, and plexiform neurofibromas, tumors comprised primarily of Schwann cel

273 , 0%-7%) had discrete cutaneous or plexiform neurofibromas, typical NF1 osseous lesions, or symptomat

274 hildren (71%) and decreases from baseline in neurofibroma volume in 12 of 18 mice (67%).

275 with amblyopia had orbitotemporal plexiform neurofibroma volumes greater than 10 mL.

276 ia secondary to the orbitotemporal plexiform neurofibroma was present in 13 subjects (62%) and was ca

277 ase from baseline in the volume of plexiform neurofibromas) was monitored by using volumetric magneti

278 ral regulatory element also develop multiple neurofibromas, we demonstrate that the Tax trans-regulat

279 Typical immunohistochemical features of neurofibroma were not observed.

280 exiform neurofibromas and symptomatic spinal neurofibromas were more prevalent in these individuals c

281 Uveal neurofibromas were noted in all patients; anteriorly dis

282 f multiple isolated neurofibromas and spinal neurofibromas were reported.

283 Further, NF1 patients develop vascular neurofibromas where tumor vessels are invested in a dens

284 Mast cells infiltrate neurofibromas, where they secrete proteins that can remo

285 nt of benign peripheral nerve sheath tumors (neurofibromas), which can progress to malignancy.

286 f neurofibromatosis type 1-related plexiform neurofibromas, which are characterized by elevated RAS-m

287 The development of neurofibromas, which are complex tumors composed of mult

288 acterized by both malignant and nonmalignant neurofibromas, which are composed of Schwann cells, degr

289 Patients with NF1 develop numerous neurofibromas, which contain many inflammatory mast cell

290 his report, we present the case of a palatal neurofibroma with radiographic involvement in a patient

291 and were necropsied; 94% developed multiple neurofibromas, with 70% carrying smaller numbers of MPNS

292 , thereby allowing the development of benign neurofibromas without the need for direct mutations in N