ライフサイエンスコーパス: hemangioblastoma

1 pography, suggests a developmental origin of hemangioblastoma.

2 forme and is overexpressed in human cerebral hemangioblastoma.

3 lar tumors, including central nervous system hemangioblastomas.

4 specifically clear cell renal carcinomas and hemangioblastomas.

5 oradic renal cell carcinomas (RCC) and brain hemangioblastomas.

6 d with sporadic renal cell carcinoma and CNS hemangioblastomas.

7 renal carcinomas and central nervous system hemangioblastomas.

8 n Hippel-Lindau disease patients with 22 CNS hemangioblastomas (11 spinal cord; 11 cerebellar) that w

9 with important roles in the pathogenesis of hemangioblastoma and CC-RCC.

10 neoplasms, including central nervous system hemangioblastoma and clear cell renal cell carcinoma (CC

11 Loss of VHL expression predisposes to hemangioblastoma and clear cell renal cell carcinoma, wh

12 ations predisposing to renal cell carcinoma, hemangioblastoma and pheochromocytoma.

13 nificantly increased incidence of cerebellar hemangioblastoma and RCC (hazard ratios 2.3 and 4.0, res

14 that HIF deregulation plays a causal role in hemangioblastoma and renal carcinoma, and raises the pos

15 ight one day play a role in the treatment of hemangioblastoma and renal cell carcinoma.

16 A specific ELISA showed that hemangioblastomas and CC-RCCs expressed SDF-1alpha prote

17 In hemangioblastomas and CC-RCCs, HIF-1alpha is constitutiv

18 ha was detected in tumor cell nuclei of both hemangioblastomas and CC-RCCs.

19 within tumor cells and endothelial cells of hemangioblastomas and CC-RCCs.

20 l cell-derived factor-1alpha (SDF-1alpha) in hemangioblastomas and CC-RCCs.

21 The genetic hallmark of hemangioblastomas and clear cell-renal cell carcinomas (

22 e been linked to the development of sporadic hemangioblastomas and clear-cell renal carcinomas.

23 Despite their benign pathology, hemangioblastomas and ELSTs are a frequent cause of morb

24 lar pathobiology and clinical course between hemangioblastomas and ELSTs, the optimal management stra

25 the tumor cell of origin for VHL-associated hemangioblastomas and indicate that it is also the proge

26 s, seven lipid-poor angiomyolipomas, and one hemangioblastoma) and 68 malignant masses (including 41

27 ma, pheochromocytoma/paraganglioma, cerebral hemangioblastoma, and endolymphatic sac tumors.

28 , ectopia lentis, neurofibromatosis, retinal hemangioblastomas, and familial exudative vitreoretinopa

29 ssor gene predispose people to renal cancer, hemangioblastomas, and pheochromocytomas in an allele-sp

30 would predict that children who present with hemangioblastoma are likely to harbor germline mutation

31 ures and blood vessels within VHL-associated hemangioblastomas are a result of tumor-derived vasculog

32 Hemangioblastomas are central nervous system (CNS) tumor

33 Hemangioblastomas are composed of neoplastic "stromal" c

34 kD complex was selectively downregulated in hemangioblastoma as compared to glioblastoma multiforme.

35 Hemangioblastoma-associated cyst wall histomorphological

36 wly and progressively evolved into enlarging hemangioblastoma-associated cysts in all tumors (mean fo

37 angiomesenchymal tumorlets, which resembled hemangioblastoma, but which also consistently showed dis

38 r include retinal and central nervous system hemangioblastomas, clear cell renal carcinomas and pheoc

39 Thirteen (59%) hemangioblastoma-cysts became symptomatic (mean time to

40 riety of autocrine loops may be initiated in hemangioblastomas, depending on the differentiation stat

41 Four children with solitary hemangioblastoma did not have a detectable germline dele

42 creened 6 pediatric patients with cerebellar hemangioblastoma for germline or somatic mutations of th

43 alysed two renal cell carcinomas and one CNS hemangioblastoma from three unrelated patients for genet

44 ing, we analyzed five central nervous system hemangioblastomas from three patients of a single VHL ge

45 Individuals with ocular hemangioblastomas had a significantly increased incidenc

46 nset renal cell carcinoma and </= 40% of CNS hemangioblastoma harbor germline VHL mutations without a

47 ents develop multiple central nervous system hemangioblastomas (HB).

48 variety of tumor types that include retinal hemangioblastomas, hemangioblastomas of the central nerv

49 To better understand hemangioblastoma histogenesis, we analyzed postmortem CN

50 Solitary cerebellar hemangioblastoma in children does not predict a germline

51 The tumorigenesis of hemangioblastoma in younger patients may differ from tha

52 distribution of central nervous system (CNS) hemangioblastomas in the von Hippel-Lindau (VHL) tumor s

53 eveloping central nervous system and retinal hemangioblastomas, kidney cysts and clear cell carcinoma

54 derstanding the factors underlying growth in hemangioblastoma may lead to better strategies to arrest

55 er, recent data indicate that VHL-associated hemangioblastoma neoplastic cells originate from embryol

56 nt with unresectable symptomatic suprasellar hemangioblastoma, octreotide resulted in tumor volume re

57 ons result in organ-specific tumors, such as hemangioblastoma of the central nervous system and renal

58 ypes that include retinal hemangioblastomas, hemangioblastomas of the central nervous system, renal c

59 inically by vascular tumors including benign hemangioblastomas of the cerebellum, spine, brain stem a

60 elopment of highly vascular tumors including hemangioblastomas of the retina and central nervous syst

61 e at high risk of multiple tumors (e.g., CNS hemangioblastomas, pheochromocytoma, and renal cell carc

62 including retinal and central nervous system hemangioblastomas, pheochromocytomas, and renal and panc

63 ained in von Hippel-Lindau disease patients, hemangioblastomas provide an opportunity to examine the

64 In 5 VHL patients, we resected quiescent hemangioblastomas (Q-HB) that were en-route during surgi

65 of advanced juxtapapillary retinal capillary hemangioblastomas (RCH) associated with von Hippel-Linda

66 romocytoma, polycythemia, or combinations of hemangioblastoma, renal cell carcinoma, and/or pheochrom

67 Spinocerebellar hemangioblastomas, renal carcinomas, and pheochromocytom

68 ncer syndrome predisposing to ocular and CNS hemangioblastomas, renal-cell carcinoma (RCC), and pheoc

69 Moreover, in two patients who each had two hemangioblastomas resected each tumor contained a unique

70 It is characterized by brain and spinal-cord hemangioblastomas, retinal angiomas, clear-cell renal ca

71 -route during surgical access to symptomatic hemangioblastomas (S-HB), for matched tumor analysis.

72 Retinal angiomas, cerebellar and spinal hemangioblastomas, solid organ cysts, and renal carcinom

73 Central nervous system lesions include hemangioblastomas (the most common tumor in VHL) and end

74 Von Hippel-Lindau (VHL)-associated hemangioblastomas (VHL-HB) arise in the central nervous

75 rigin of tumor vasculature in VHL-associated hemangioblastomas, we analyzed the vascular elements in

76 Further, similar to hemangioblastomas, we demonstrate that other VHL-associa

77 VHL patients with at least 1 measurable hemangioblastoma were eligible.

78 characteristic topographical distribution of hemangioblastomas within the CNS.