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Hemangioendothelioma

MedGen UID:
42384
•Concept ID:
C0018915
•
Neoplastic Process
Synonyms: Endothelioma, Vascular; Endotheliomas, Vascular; Hemangio Endothelioma; Hemangio-Endothelioma; Hemangio-Endotheliomas; Hemangioendotheliomas; Vascular Endothelioma; Vascular Endotheliomas
SNOMED CT: Hemangioendothelioma (66229009); Angioendothelioma (66229009); Hemangioendothelioma (403980002)
 
Monarch Initiative: MONDO:0021121

Definition

A vascular proliferation characterized by the presence of prominent endothelial cells and the formation of vascular channels. Hemangioendotheliomas may display borderline or low grade characteristics. [from NCI]

Professional guidelines

PubMed

Management of Vascular Sarcoma.
Subramaniam A, Giani C, Napolitano A, Ravi V, Frezza AM, Jones RL
Surg Oncol Clin N Am 2022 Jul;31(3):485-510. doi: 10.1016/j.soc.2022.03.014. PMID: 35715146
Treatment modalities and long-term outcomes of hepatic hemangioendothelioma in the United States.
Kaltenmeier C, Stacchiotti S, Gronchi A, Sapisochin G, Liu H, Ashwat E, Gunabushanam V, Reddy D, Thompson A, Geller D, Tohme S, Zureikat A, Molinari M
HPB (Oxford) 2022 Oct;24(10):1688-1696. Epub 2022 Apr 14 doi: 10.1016/j.hpb.2022.03.013. PMID: 35504832Free PMC Article
Epithelioid Hemangioendothelioma: Update on Diagnosis and Treatment.
Rosenberg A, Agulnik M
Curr Treat Options Oncol 2018 Mar 15;19(4):19. doi: 10.1007/s11864-018-0536-y. PMID: 29546487

Recent clinical studies

Etiology

The genetics of vascular tumours: an update.
Torrence D, Antonescu CR
Histopathology 2022 Jan;80(1):19-32. doi: 10.1111/his.14458. PMID: 34958509Free PMC Article
Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts.
Stacchiotti S, Miah AB, Frezza AM, Messiou C, Morosi C, Caraceni A, Antonescu CR, Bajpai J, Baldini E, Bauer S, Biagini R, Bielack S, Blay JY, Bonvalot S, Boukovinas I, Bovee JVMG, Boye K, Brodowicz T, Callegaro D, De Alava E, Deoras-Sutliff M, Dufresne A, Eriksson M, Errani C, Fedenko A, Ferraresi V, Ferrari A, Fletcher CDM, Garcia Del Muro X, Gelderblom H, Gladdy RA, Gouin F, Grignani G, Gutkovich J, Haas R, Hindi N, Hohenberger P, Huang P, Joensuu H, Jones RL, Jungels C, Kasper B, Kawai A, Le Cesne A, Le Grange F, Leithner A, Leonard H, Lopez Pousa A, Martin Broto J, Merimsky O, Merriam P, Miceli R, Mir O, Molinari M, Montemurro M, Oldani G, Palmerini E, Pantaleo MA, Patel S, Piperno-Neumann S, Raut CP, Ravi V, Razak ARA, Reichardt P, Rubin BP, Rutkowski P, Safwat AA, Sangalli C, Sapisochin G, Sbaraglia M, Scheipl S, Schöffski P, Strauss D, Strauss SJ, Sundby Hall K, Tap WD, Trama A, Tweddle A, van der Graaf WTA, Van De Sande MAJ, Van Houdt W, van Oortmerssen G, Wagner AJ, Wartenberg M, Wood J, Zaffaroni N, Zimmermann C, Casali PG, Dei Tos AP, Gronchi A
ESMO Open 2021 Jun;6(3):100170. Epub 2021 Jun 2 doi: 10.1016/j.esmoop.2021.100170. PMID: 34090171Free PMC Article
Kaposiform hemangioendothelioma: current knowledge and future perspectives.
Ji Y, Chen S, Yang K, Xia C, Li L
Orphanet J Rare Dis 2020 Feb 3;15(1):39. doi: 10.1186/s13023-020-1320-1. PMID: 32014025Free PMC Article
Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences.
Ji Y, Chen S, Peng S, Xia C, Li L
Orphanet J Rare Dis 2019 Jul 5;14(1):165. doi: 10.1186/s13023-019-1147-9. PMID: 31277673Free PMC Article
Benign esophageal tumors.
Ha C, Regan J, Cetindag IB, Ali A, Mellinger JD
Surg Clin North Am 2015 Jun;95(3):491-514. Epub 2015 Mar 21 doi: 10.1016/j.suc.2015.02.005. PMID: 25965126

Diagnosis

Pseudomyogenic Hemangioendothelioma.
Al-Qaderi A, Mansour AT
Arch Pathol Lab Med 2019 Jun;143(6):763-767. Epub 2018 Dec 21 doi: 10.5858/arpa.2017-0430-RS. PMID: 30576238
Epithelioid Hemangioendothelioma: Update on Diagnosis and Treatment.
Rosenberg A, Agulnik M
Curr Treat Options Oncol 2018 Mar 15;19(4):19. doi: 10.1007/s11864-018-0536-y. PMID: 29546487
Pseudomyogenic hemangioendothelioma.
Horan NA, DiMaio DJ
Cutis 2017 Dec;100(6):E13-E16. PMID: 29360904
Cutaneous Malignant Vascular Neoplasms.
Shon W, Billings SD
Clin Lab Med 2017 Sep;37(3):633-646. Epub 2017 Jul 13 doi: 10.1016/j.cll.2017.06.004. PMID: 28802504
Malignant vascular tumors--an update.
Antonescu C
Mod Pathol 2014 Jan;27 Suppl 1:S30-8. doi: 10.1038/modpathol.2013.176. PMID: 24384851

Therapy

Sirolimus as initial therapy for kaposiform hemangioendothelioma and tufted angioma.
Wang H, Guo X, Duan Y, Zheng B, Gao Y
Pediatr Dermatol 2018 Sep;35(5):635-638. Epub 2018 Jul 12 doi: 10.1111/pde.13600. PMID: 29999213
Epithelioid Hemangioendothelioma: Update on Diagnosis and Treatment.
Rosenberg A, Agulnik M
Curr Treat Options Oncol 2018 Mar 15;19(4):19. doi: 10.1007/s11864-018-0536-y. PMID: 29546487
Treatment of kaposiform hemangioendothelioma and tufted angioma.
Liu XH, Li JY, Qu XH, Yan WL, Zhang L, Yang C, Zheng JW
Int J Cancer 2016 Oct 1;139(7):1658-66. Epub 2016 Jun 28 doi: 10.1002/ijc.30216. PMID: 27252149
Vascular sarcomas.
Ravi V, Patel S
Curr Oncol Rep 2013 Aug;15(4):347-55. doi: 10.1007/s11912-013-0328-2. PMID: 23852636
Kasabach-Merritt phenomenon.
Kelly M
Pediatr Clin North Am 2010 Oct;57(5):1085-9. Epub 2010 Aug 21 doi: 10.1016/j.pcl.2010.07.006. PMID: 20888459

Prognosis

Incidence, demographics, and survival of malignant hemangioendothelioma in the United States.
Paulson KG, Ravi V, Rubin BP, Park M, Loggers ET, Cranmer LD, Wagner MJ
Cancer Med 2023 Jul;12(14):15101-15106. Epub 2023 Jun 1 doi: 10.1002/cam4.6181. PMID: 37260142Free PMC Article
Pseudomyogenic Hemangioendothelioma (Epithelioid Sarcoma-Like Hemangioendothelioma).
Caballero GA, Roitman PD
Arch Pathol Lab Med 2020 Apr;144(4):529-533. Epub 2019 Apr 24 doi: 10.5858/arpa.2018-0395-RS. PMID: 31017450
Pseudomyogenic Hemangioendothelioma.
Al-Qaderi A, Mansour AT
Arch Pathol Lab Med 2019 Jun;143(6):763-767. Epub 2018 Dec 21 doi: 10.5858/arpa.2017-0430-RS. PMID: 30576238
Hepatic Epithelioid Hemangioendothelioma.
Studer LL, Selby DM
Arch Pathol Lab Med 2018 Feb;142(2):263-267. doi: 10.5858/arpa.2016-0171-RS. PMID: 29372848
Cutaneous Malignant Vascular Neoplasms.
Shon W, Billings SD
Clin Lab Med 2017 Sep;37(3):633-646. Epub 2017 Jul 13 doi: 10.1016/j.cll.2017.06.004. PMID: 28802504

Clinical prediction guides

Incidence, demographics, and survival of malignant hemangioendothelioma in the United States.
Paulson KG, Ravi V, Rubin BP, Park M, Loggers ET, Cranmer LD, Wagner MJ
Cancer Med 2023 Jul;12(14):15101-15106. Epub 2023 Jun 1 doi: 10.1002/cam4.6181. PMID: 37260142Free PMC Article
A Phase II Study of Epacadostat and Pembrolizumab in Patients with Advanced Sarcoma.
Kelly CM, Qin LX, Whiting KA, Richards AL, Avutu V, Chan JE, Chi P, Dickson MA, Gounder MM, Keohan ML, Movva S, Nacev BA, Rosenbaum E, Adamson T, Singer S, Bartlett EK, Crago AM, Yoon SS, Hwang S, Erinjeri JP, Antonescu CR, Tap WD, D'Angelo SP
Clin Cancer Res 2023 Jun 1;29(11):2043-2051. doi: 10.1158/1078-0432.CCR-22-3911. PMID: 36971773Free PMC Article
Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences.
Ji Y, Chen S, Peng S, Xia C, Li L
Orphanet J Rare Dis 2019 Jul 5;14(1):165. doi: 10.1186/s13023-019-1147-9. PMID: 31277673Free PMC Article
Malignant vascular tumors--an update.
Antonescu C
Mod Pathol 2014 Jan;27 Suppl 1:S30-8. doi: 10.1038/modpathol.2013.176. PMID: 24384851
Vascular sarcomas.
Ravi V, Patel S
Curr Oncol Rep 2013 Aug;15(4):347-55. doi: 10.1007/s11912-013-0328-2. PMID: 23852636

Recent systematic reviews

The use of mTOR inhibitors for the treatment of kaposiform hemangioendothelioma. A systematic review.
Maza-Morales M, Valdés-Loperena S, Durán-McKinster LC, García-Romero MT
Pediatr Dermatol 2023 May-Jun;40(3):440-445. Epub 2023 Jan 30 doi: 10.1111/pde.15262. PMID: 36716766
Pseudomyogenic hemangioendothelioma with bone and soft tissue involvement with favorable response to pamidronate: a case report and systematic review of the literature.
Brance ML, Cóccaro NM, Roitman P, Castiglioni A, Agostinis F, Spense M, Scheitlin B, Rene N, Brun LR
Arch Osteoporos 2022 Feb 1;17(1):28. doi: 10.1007/s11657-022-01062-4. PMID: 35106633
Adult primary liver sarcoma: systematic review.
Martins ACA, Costa Neto DCD, Silva JDDE, Moraes YM, LeÃo CS, Martins C
Rev Col Bras Cir 2020;47:e20202647. Epub 2020 Nov 30 doi: 10.1590/0100-6991e-20202647. PMID: 33263653
Treatment of kaposiform hemangioendothelioma and tufted angioma.
Liu XH, Li JY, Qu XH, Yan WL, Zhang L, Yang C, Zheng JW
Int J Cancer 2016 Oct 1;139(7):1658-66. Epub 2016 Jun 28 doi: 10.1002/ijc.30216. PMID: 27252149
Liver transplantation for hepatic tumors: a systematic review.
Ravaioli M, Ercolani G, Neri F, Cescon M, Stacchini G, Del Gaudio M, Cucchetti A, Pinna AD
World J Gastroenterol 2014 May 14;20(18):5345-52. doi: 10.3748/wjg.v20.i18.5345. PMID: 24833864Free PMC Article

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