309:
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64:
29:
224:
280:
Hemangioblastoma are among the rarest central nervous system tumors, accounting for less than 2%. Hemangioblastomas usually occur in adults, yet tumors may appear in VHL syndrome at much younger ages. Men and women are approximately at the same risk. Although they can occur in any section of the
271:
The outcome for hemangioblastoma is very good if surgical extraction of the tumor can be achieved; excision is possible in most cases and permanent neurologic deficit is uncommon and can be avoided altogether if the tumor is diagnosed and treated early. Persons with VHL syndrome have a bleaker
498:
Silva, Danilo; Grabowski, Mathew M.; Juthani, Rupa; Sharma, Mayur; Angelov, Lilyana; Vogelbaum, Michael A.; Chao, Samuel; Suh, John; Mohammadi, Alireza; Barnett, Gene H. (September 2016). "Gamma Knife radiosurgery for intracranial hemangioblastoma".
617:
1211:
1111:
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742:
255:
of the tumor. Although usually straightforward to carry out, recurrence of the tumor or more tumors at a different site develop in approximately 20% of patients.
227:
Contrast enhanced MRI of the conus medullaris and cauda equina of patient with VHL depicts a hemangioblastoma of the conus with extension into the nerve roots.
1074:
1195:
1119:
1043:
184:
881:
735:
187:(pVHL) is dysfunctional, usually due to mutation and/or gene silencing. In normal circumstances, pVHL is involved in the inhibition of
389:
359:
1069:
212:
116:
728:
594:
876:
1048:
921:
1246:
260:
199:. In these dysfunctional cells pVHL cannot degrade HIF-1α, causing it to accumulate. HIF-1α causes the production of
263:
have also been employed to successfully treat recurrence and control tumor growth of cerebellar hemangioblastomas.
1133:
446:
Kaelin, William G (2005). "von Hippel–Lindau-associated malignancies: Mechanisms and therapeutic opportunities".
132:
972:
967:
759:
643:
272:
prognosis than those who have sporadic tumors since those with VHL syndrome usually have more than one lesion.
751:
236:
160:
708:
1223:
Note: Not all brain tumors are of nervous tissue, and not all nervous tissue tumors are in the brain (see
891:
886:
820:
282:
85:
28:
1188:
1147:
871:
544:"Haemangioblastoma of the central nervous system in von Hippel-Lindau disease. French VHL Study Group"
223:
1152:
962:
995:
235:
scan (CT scan). On a scan, hemangioblastoma shows as a well-defined, low attenuation region in the
232:
63:
1159:
1101:
573:
524:
1172:
916:
781:
654:
565:
516:
428:
385:
355:
252:
172:
52:
407:"Cerebellar hemangioblastoma presenting as secondary erythrocytosis and aspiration pneumonia"
1224:
990:
555:
508:
455:
418:
1064:
908:
89:
1033:
1024:
954:
637:
423:
406:
208:
156:
81:
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92:, usually during middle age. Sometimes, these tumors occur in other sites such as the
1240:
1142:
1028:
944:
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543:
120:
577:
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1179:
1167:
896:
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149:
101:
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1038:
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697:
459:
294:
256:
93:
659:
512:
1183:
1096:
939:
626:
290:
286:
196:
124:
109:
105:
40:
36:
858:
806:
689:
665:
192:
176:
128:
44:
520:
569:
432:
1088:
931:
703:
57:
153:
609:
1016:
849:
621:
240:
204:
97:
350:
Lindsay, Kenneth W; Ian Bone; Robin
Callander; J. van Gijn (1991).
222:
188:
112:
670:
542:
Richard S, Campello C, Taillandier L, Parker F, Resche F (1998).
200:
119:(VHL syndrome). Hemangioblastomas are most commonly composed of
724:
1212:
WHO classification of the tumors of the central nervous system
131:
or spinal cord. They are classed as grade I tumors under the
595:
Stereotactic
Radiosurgery for Hemangioblastomas of the Brain
150:
abnormally high number of red blood cells in the bloodstream
215:, which act to stimulate growth of cells within the tumor.
382:
WHO Classification of Tumors of the
Central Nervous System
239:
with an enhancing nodule on the wall. Sometimes multiple
326:
Micrograph of cerebellar hemangioblastoma. HPS stain.
314:
Micrograph of cerebellar hemangioblastoma. HPS stain.
100:. They may be associated with other diseases such as
599:
1204:
1132:
1110:
1087:
1057:
1013:
983:
953:
930:
907:
857:
848:
828:
819:
795:
767:
758:
680:
603:
51:
21:
123:in small blood vessels and usually occur in the
736:
8:
1075:Embryonal tumour with multilayered rosettes
285:, they usually occur in either side of the
854:
845:
825:
764:
743:
729:
721:
600:
345:
343:
341:
62:
27:
18:
559:
422:
448:Drug Discovery Today: Disease Mechanisms
375:
373:
371:
354:. United States: Churchill Livingstone.
1196:Malignant peripheral nerve sheath tumor
1120:Primary central nervous system lymphoma
1044:Dysembryoplastic neuroepithelial tumour
337:
304:
352:Neurology and Neurosurgery Illustrated
251:The treatment for hemangioblastoma is
231:The primary diagnosis is made with a
7:
478:The Lecturio Medical Concept Library
882:Subependymal giant cell astrocytoma
201:vascular endothelial growth factor
171:Hemangioblastomas are composed of
14:
152:due to ectopic production of the
1070:Atypical teratoid rhabdoid tumor
561:10.1046/j.1365-2796.1998.00337.x
501:Journal of Clinical Neuroscience
319:
307:
213:transforming growth factor alpha
205:platelet derived growth factor B
411:The Western Journal of Medicine
148:Hemangioblastomas can cause an
1164:Cranial and paraspinal nerves
1:
877:Pleomorphic xanthoastrocytoma
752:Tumours of the nervous system
405:Farrukh, HM (February 1996).
922:Anaplastic oligodendroglioma
189:hypoxia-inducible factor 1 α
460:10.1016/j.ddmec.2005.04.003
1263:
513:10.1016/j.jocn.2016.03.008
135:'s classification system.
117:Von Hippel–Lindau syndrome
1220:
185:von Hippel-Lindau protein
133:World Health Organization
35:
26:
1049:Lhermitte–Duclos disease
973:Choroid plexus carcinoma
968:Choroid plexus papilloma
257:Gamma Knife Radiosurgery
88:that originate from the
380:Louis, David N (1991).
197:proteosomal degradation
161:paraneoplastic syndrome
892:Anaplastic astrocytoma
887:Fibrillary astrocytoma
283:central nervous system
228:
179:and stromal cells. In
86:central nervous system
1148:Esthesioneuroblastoma
872:Pilocytic astrocytoma
226:
1153:Ganglioneuroblastoma
1058:CNS embryonal tumors
963:Choroid plexus tumor
996:Gliomatosis cerebri
233:computed tomography
1247:Vascular neoplasia
1160:Nerve sheath tumor
1102:Hemangiopericytoma
681:External resources
474:"Hemangioblastoma"
229:
78:haemangioblastomas
43:hemangioblastoma.
1234:
1233:
1173:Neurofibromatosis
1128:
1127:
1083:
1082:
1009:
1008:
917:Oligodendroglioma
815:
814:
782:Craniopharyngioma
718:
717:
301:Additional images
253:surgical excision
173:endothelial cells
74:Hemangioblastomas
71:
70:
16:Medical condition
1254:
1225:brain metastasis
1189:Acoustic neuroma
991:Oligoastrocytoma
984:Multiple/unknown
855:
846:
826:
800:
775:
765:
745:
738:
731:
722:
601:
582:
581:
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67:
66:
31:
22:Hemangioblastoma
19:
1262:
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1251:
1237:
1236:
1235:
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1200:
1124:
1106:
1079:
1065:Medulloblastoma
1053:
1015:
1005:
979:
949:
926:
909:Oligodendrocyte
903:
838:
832:
830:Neuroepithelial
811:
796:
791:
768:
754:
749:
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327:
324:
315:
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303:
278:
269:
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237:posterior fossa
221:
169:
146:
141:
90:vascular system
82:vascular tumors
61:
17:
12:
11:
5:
1260:
1258:
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1091:
1085:
1084:
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1080:
1078:
1077:
1072:
1067:
1061:
1059:
1055:
1054:
1052:
1051:
1046:
1041:
1036:
1034:Retinoblastoma
1031:
1025:Ganglioneuroma
1021:
1019:
1011:
1010:
1007:
1006:
1004:
1003:
998:
993:
987:
985:
981:
980:
978:
977:
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959:
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955:Choroid plexus
951:
950:
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942:
936:
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928:
927:
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651:
640:
629:
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608:
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605:
604:Classification
598:
597:
590:
589:External links
587:
584:
583:
548:J. Intern. Med
534:
490:
465:
454:(2): 225–231.
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318:
316:
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306:
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209:erythropoietin
168:
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157:erythropoietin
145:
142:
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69:
68:
55:
49:
48:
33:
32:
24:
23:
15:
13:
10:
9:
6:
4:
3:
2:
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1166:
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1144:
1143:Neuroblastoma
1141:
1140:
1138:
1135:
1131:
1121:
1118:
1117:
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1113:
1112:Hematopoietic
1109:
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1060:
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1042:
1040:
1037:
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1032:
1030:
1029:Ganglioglioma
1026:
1023:
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1002:
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994:
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986:
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974:
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946:
945:Subependymoma
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847:
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840:spinal tumors
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831:
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554:(6): 547–53.
553:
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522:
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514:
510:
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502:
494:
491:
479:
475:
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453:
449:
442:
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434:
430:
425:
420:
417:(2): 169–71.
416:
412:
408:
401:
398:
393:
391:92-832-2430-2
387:
383:
376:
374:
372:
368:
363:
361:0-443-04345-0
357:
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246:
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243:are present.
242:
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202:
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182:
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144:Complications
143:
138:
136:
134:
130:
126:
122:
121:stromal cells
118:
114:
111:
107:
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99:
95:
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83:
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65:
59:
56:
54:
50:
46:
42:
38:
34:
30:
25:
20:
1222:
1180:Neurilemmoma
1168:Neurofibroma
897:Glioblastoma
835:brain tumors
797:
769:
702:
688:
664:
653:
642:
631:
616:
551:
547:
537:
504:
500:
493:
481:. Retrieved
477:
468:
451:
447:
441:
414:
410:
400:
381:
351:
279:
276:Epidemiology
270:
250:
230:
191:(HIF-1α) by
181:VHL syndrome
170:
167:Pathogenesis
147:
139:Presentation
102:polycythemia
77:
73:
72:
1039:Neurocytoma
1001:Gliosarcoma
867:Astrocytoma
787:Pituicytoma
507:: 147–151.
295:spinal cord
259:as well as
104:(increased
94:spinal cord
1184:Schwannoma
1097:Meningioma
940:Ependymoma
655:DiseasesDB
332:References
291:brain stem
287:cerebellum
125:cerebellum
110:pancreatic
106:blood cell
41:cerebellar
37:Micrograph
859:Astrocyte
807:Pinealoma
760:Endocrine
698:radio/326
690:eMedicine
666:SNOMED CT
267:Prognosis
247:Treatment
219:Diagnosis
195:mediated
193:ubiquitin
177:pericytes
129:brainstem
53:Specialty
45:HPS stain
1241:Category
1089:Meninges
932:Ependyma
704:Orphanet
695:med/2991
671:81201000
578:20823909
529:19652430
521:27422585
384:. IARC.
108:count),
58:Oncology
649:D018325
638:M9161/1
570:9681857
483:24 July
433:8775737
424:1303396
293:or the
241:lesions
154:hormone
84:of the
1017:neuron
1014:Mature
850:Glioma
798:Other:
771:Sellar
709:252054
576:
568:
527:
519:
431:
421:
388:
358:
289:, the
98:retina
80:, are
60:
1205:Other
660:31512
633:ICD-O
627:D48.1
574:S2CID
525:S2CID
261:LINAC
159:as a
113:cysts
76:, or
39:of a
644:MeSH
566:PMID
517:PMID
485:2021
429:PMID
386:ISBN
356:ISBN
211:and
183:the
115:and
96:and
1134:PNS
821:CNS
618:ICD
556:doi
552:243
509:doi
456:doi
419:PMC
415:164
1243::
1227:).
1027::
707::
693::
669::
658::
647::
636::
625::
622:10
572:.
564:.
550:.
546:.
523:.
515:.
505:31
503:.
476:.
450:.
427:.
413:.
409:.
370:^
340:^
297:.
207:,
203:,
175:,
163:.
127:,
1182:/
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837:,
833:(
773::
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737:t
730:v
620:-
610:D
580:.
558::
531:.
511::
487:.
462:.
458::
452:2
435:.
394:.
364:.
47:.
Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.
