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Hemangioblastoma

MedGen UID:
104929
Concept ID:
C0206734
Neoplastic Process
Synonym: Hemangioblastomas
SNOMED CT: Hemangioblastoma (81201000); Angioblastoma (81201000); Hemangioblastoma (1156474009)
 
HPO: HP:0010797
Monarch Initiative: MONDO:0016748
Orphanet: ORPHA252054

Definition

A hemangioblastoma is a benign vascular neoplasm that arises almost exclusively in the central nervous system. Hemangioblastomas consist of a tightly packed cluster of small blood vessels forming a mass of up to 1 or 2 cm in diameter. [from HPO]

Professional guidelines

PubMed

Suárez C, Vieito M, Valdivia A, González M, Carles J
Med Sci (Basel) 2023 Jun 30;11(3) doi: 10.3390/medsci11030046. PMID: 37489462Free PMC Article
Gangat N, Szuber N, Tefferi A
Am J Hematol 2023 Jun;98(6):965-981. Epub 2023 Apr 3 doi: 10.1002/ajh.26920. PMID: 36966432
Wiley HE, Krivosic V, Gaudric A, Gorin MB, Shields C, Shields J, Aronow ME, Chew EY
Retina 2019 Dec;39(12):2254-2263. doi: 10.1097/IAE.0000000000002572. PMID: 31259811Free PMC Article

Recent clinical studies

Etiology

Gangat N, Szuber N, Tefferi A
Am J Hematol 2023 Jun;98(6):965-981. Epub 2023 Apr 3 doi: 10.1002/ajh.26920. PMID: 36966432
Louise M Binderup M, Smerdel M, Borgwadt L, Beck Nielsen SS, Madsen MG, Møller HU, Kiilgaard JF, Friis-Hansen L, Harbud V, Cortnum S, Owen H, Gimsing S, Friis Juhl HA, Munthe S, Geilswijk M, Rasmussen ÅK, Møldrup U, Graumann O, Donskov F, Grønbæk H, Stausbøl-Grøn B, Schaffalitzky de Muckadell O, Knigge U, Dam G, Wadt KA, Bøgeskov L, Bagi P, Lund L, Stochholm K, Ousager LB, Sunde L
Eur J Med Genet 2022 Aug;65(8):104538. Epub 2022 Jun 13 doi: 10.1016/j.ejmg.2022.104538. PMID: 35709961
Jonasch E, Donskov F, Iliopoulos O, Rathmell WK, Narayan VK, Maughan BL, Oudard S, Else T, Maranchie JK, Welsh SJ, Thamake S, Park EK, Perini RF, Linehan WM, Srinivasan R; MK-6482-004 Investigators
N Engl J Med 2021 Nov 25;385(22):2036-2046. doi: 10.1056/NEJMoa2103425. PMID: 34818478Free PMC Article
Tarade D, Ohh M
Oncogene 2018 Jan 11;37(2):139-147. Epub 2017 Sep 18 doi: 10.1038/onc.2017.338. PMID: 28925400
Sung DI, Chang CH, Harisiadis L
Cancer 1982 Feb 1;49(3):553-5. doi: 10.1002/1097-0142(19820201)49:3<553::aid-cncr2820490326>3.0.co;2-a. PMID: 7199372

Diagnosis

Timonin SY, Konovalov NA
Sovrem Tekhnologii Med 2021;13(5):83-94. Epub 2021 Oct 29 doi: 10.17691/stm2021.13.5.10. PMID: 35265353Free PMC Article
Qu L, Lv C, Ji T, Wang Y, Yu J
Int J Surg Pathol 2021 Apr;29(2):129-134. Epub 2020 Jun 14 doi: 10.1177/1066896920933998. PMID: 32538251
McGrath LA, Mudhar HS, Salvi SM
Surv Ophthalmol 2019 Mar-Apr;64(2):175-184. Epub 2018 Oct 13 doi: 10.1016/j.survophthal.2018.10.002. PMID: 30321570
Bisceglia M, Muscarella LA, Galliani CA, Zidar N, Ben-Dor D, Pasquinelli G, la Torre A, Sparaneo A, Fanburg-Smith JC, Lamovec J, Michal M, Bacchi CE
Adv Anat Pathol 2018 May;25(3):197-215. doi: 10.1097/PAP.0000000000000176. PMID: 29189208
Huang Y, Han XC, Lv GS
Int J Clin Exp Pathol 2014;7(4):1777-81. Epub 2014 Mar 15 PMID: 24817978Free PMC Article

Therapy

Palavani LB, Andreão FF, de Abreu LV, Batista S, Borges J, Oliveira LB, Bertani R, Filho JAA
J Clin Neurosci 2023 Nov;117:104-113. Epub 2023 Oct 1 doi: 10.1016/j.jocn.2023.09.021. PMID: 37788533
Gangat N, Szuber N, Tefferi A
Am J Hematol 2023 Jun;98(6):965-981. Epub 2023 Apr 3 doi: 10.1002/ajh.26920. PMID: 36966432
Albakr A, Baghdadi A, Singh R, Lama S, Sutherland GR
World Neurosurg 2022 Apr;160:e242-e249. Epub 2022 Jan 6 doi: 10.1016/j.wneu.2021.12.119. PMID: 34999009
Jonasch E, Donskov F, Iliopoulos O, Rathmell WK, Narayan VK, Maughan BL, Oudard S, Else T, Maranchie JK, Welsh SJ, Thamake S, Park EK, Perini RF, Linehan WM, Srinivasan R; MK-6482-004 Investigators
N Engl J Med 2021 Nov 25;385(22):2036-2046. doi: 10.1056/NEJMoa2103425. PMID: 34818478Free PMC Article
Hodgson TS, Nielsen SM, Lesniak MS, Lukas RV
Neurologist 2016 Sep;21(5):73-8. doi: 10.1097/NRL.0000000000000085. PMID: 27564075

Prognosis

Zhang Y, Nguyen CC, Zhang NT, Fink NS, John JD, Venkatesh OG, Roe JD, Hoffman SC, Lesniak MS, Wolinsky JP, Horbinski C, Szymaniak BM, Buerki RA, Sosman JA, Shenoy NK, Lukas RV
Neuro Oncol 2023 May 4;25(5):827-838. doi: 10.1093/neuonc/noac234. PMID: 36215167Free PMC Article
Jonasch E, Donskov F, Iliopoulos O, Rathmell WK, Narayan VK, Maughan BL, Oudard S, Else T, Maranchie JK, Welsh SJ, Thamake S, Park EK, Perini RF, Linehan WM, Srinivasan R; MK-6482-004 Investigators
N Engl J Med 2021 Nov 25;385(22):2036-2046. doi: 10.1056/NEJMoa2103425. PMID: 34818478Free PMC Article
Lu VM, Niazi TN
Pediatr Rev 2021 Sep;42(9):486-499. doi: 10.1542/pir.2020-000661. PMID: 34470868
Menon SG, Ramachandran GM, Jacob M, Hegde A, Pai K
J Cancer Res Ther 2021 Jan-Mar;17(1):279-281. doi: 10.4103/jcrt.JCRT_718_18. PMID: 33723171
Sung DI, Chang CH, Harisiadis L
Cancer 1982 Feb 1;49(3):553-5. doi: 10.1002/1097-0142(19820201)49:3<553::aid-cncr2820490326>3.0.co;2-a. PMID: 7199372

Clinical prediction guides

Vetrano IG, Gioppo A, Faragò G, Pinzi V, Pollo B, Broggi M, Schiariti M, Ferroli P, Acerbi F
Adv Exp Med Biol 2023;1405:377-403. doi: 10.1007/978-3-031-23705-8_14. PMID: 37452946
Fallah J, Brave MH, Weinstock C, Mehta GU, Bradford D, Gittleman H, Bloomquist EW, Charlab R, Hamed SS, Miller CP, Dorff SE, Chambers WA, Mixter BD, Dinin J, Pierce WF, Ricks TK, Tang S, Donoghue M, Pazdur R, Amiri-Kordestani L, Ibrahim A, Beaver JA
Clin Cancer Res 2022 Nov 14;28(22):4843-4848. doi: 10.1158/1078-0432.CCR-22-1054. PMID: 35727604Free PMC Article
Jonasch E, Donskov F, Iliopoulos O, Rathmell WK, Narayan VK, Maughan BL, Oudard S, Else T, Maranchie JK, Welsh SJ, Thamake S, Park EK, Perini RF, Linehan WM, Srinivasan R; MK-6482-004 Investigators
N Engl J Med 2021 Nov 25;385(22):2036-2046. doi: 10.1056/NEJMoa2103425. PMID: 34818478Free PMC Article
Wiley HE, Krivosic V, Gaudric A, Gorin MB, Shields C, Shields J, Aronow ME, Chew EY
Retina 2019 Dec;39(12):2254-2263. doi: 10.1097/IAE.0000000000002572. PMID: 31259811Free PMC Article
Dornbos D 3rd, Kim HJ, Butman JA, Lonser RR
JAMA Neurol 2018 May 1;75(5):620-627. doi: 10.1001/jamaneurol.2017.4469. PMID: 29379961

Recent systematic reviews

Daniels AB, Chang EY, Chew EY, Gombos DS, Gorin MB, Shields CL, Wiley HE
Ophthalmology 2024 May;131(5):622-633. Epub 2023 Dec 12 doi: 10.1016/j.ophtha.2023.12.014. PMID: 38092079
Palavani LB, Andreão FF, de Abreu LV, Batista S, Borges J, Oliveira LB, Bertani R, Filho JAA
J Clin Neurosci 2023 Nov;117:104-113. Epub 2023 Oct 1 doi: 10.1016/j.jocn.2023.09.021. PMID: 37788533
Jankovic D, Vuong K, Splavski B, Rotim K, Arnautovic KI
World Neurosurg 2023 May;173:48-62. Epub 2023 Feb 22 doi: 10.1016/j.wneu.2023.02.071. PMID: 36822402
Jankovic D, Hanissian A, Rotim K, Splavski B, Arnautovic KI
World Neurosurg 2022 Feb;158:1-10. Epub 2021 Oct 21 doi: 10.1016/j.wneu.2021.10.105. PMID: 34687932
Ampie L, Choy W, Lamano JB, Kesavabhotla K, Kaur R, Parsa AT, Bloch O
Clin Neurol Neurosurg 2016 Nov;150:143-151. Epub 2016 Sep 19 doi: 10.1016/j.clineuro.2016.09.008. PMID: 27668858

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