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Epithelioid hemangioendothelioma

MedGen UID:
569676
•Concept ID:
C0334538
•
Neoplastic Process
Synonym: Epithelioid hemangioendothelioma, malignant
SNOMED CT: Epithelioid hemangioendothelioma (54124005); Epithelioid hemangioendothelioma, malignant (54124005)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0015523
Orphanet: ORPHA157791

Definition

A rare vascular tumor characterized by a solitary lesion in the superficial or deep soft tissue of the extremities, most often originating from a small vein as a fusiform intravascular mass also infiltrating surrounding tissues. It is composed of epithelioid endothelial cells arranged in short cords and nests in a myxohyaline stroma. Patients present with an often painful nodule which may be associated with edema or thrombophlebitis. In classic epithelioid hemangioendothelioma lacking atypical histological features metastatic rate and mortality are low. [from ORDO]

Professional guidelines

PubMed

Epithelioid Vascular Lesions: The Differential Diagnosis and Approach in Cytology and Small Biopsies.
Tabish N, Monaco SE
Adv Anat Pathol 2022 Nov 1;29(6):389-400. Epub 2022 Aug 22 doi: 10.1097/PAP.0000000000000358. PMID: 35993506
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
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

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

Recent clinical studies

Etiology

Vascular tumors of intermediate malignancy: An update.
Folpe AL
Hum Pathol 2024 May;147:114-128. Epub 2024 Feb 13 doi: 10.1016/j.humpath.2024.01.014. PMID: 38360216
Pulmonary Epithelioid Hemangioendothelioma: Imaging and Clinical Features.
Liu H, Wang J, Lang J, Zhang X
J Comput Assist Tomogr 2021 Sep-Oct 01;45(5):788-794. doi: 10.1097/RCT.0000000000001194. PMID: 34546683
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
Liver transplantation for malignancies.
Eghtesad B, Aucejo F
J Gastrointest Cancer 2014 Sep;45(3):353-62. doi: 10.1007/s12029-014-9590-2. PMID: 24604263
Epithelioid hemangioendothelioma and related lesions.
Weiss SW, Ishak KG, Dail DH, Sweet DE, Enzinger FM
Semin Diagn Pathol 1986 Nov;3(4):259-87. PMID: 3303234

Diagnosis

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
Epithelioid Vascular Tumors: A Review.
Shon W, Billings SD
Adv Anat Pathol 2019 May;26(3):186-197. doi: 10.1097/PAP.0000000000000228. PMID: 30762622
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
Congenital Vascular Tumors.
Goss JA, Greene AK
Otolaryngol Clin North Am 2018 Feb;51(1):89-97. doi: 10.1016/j.otc.2017.09.008. PMID: 29217070
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

Therapy

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
Liver Transplant for Nonhepatocellular Carcinoma Malignancy.
Haberal Reyhan N
Exp Clin Transplant 2017 Mar;15(Suppl 2):69-73. doi: 10.6002/ect.TOND16.L18. PMID: 28302003
Liver transplantation for malignancies.
Eghtesad B, Aucejo F
J Gastrointest Cancer 2014 Sep;45(3):353-62. doi: 10.1007/s12029-014-9590-2. PMID: 24604263
Vascular sarcomas.
Ravi V, Patel S
Curr Oncol Rep 2013 Aug;15(4):347-55. doi: 10.1007/s11912-013-0328-2. PMID: 23852636
Molecular pathways: ROS1 fusion proteins in cancer.
Davies KD, Doebele RC
Clin Cancer Res 2013 Aug 1;19(15):4040-5. Epub 2013 May 29 doi: 10.1158/1078-0432.CCR-12-2851. PMID: 23719267Free PMC Article

Prognosis

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
Vascular sarcomas.
Ravi V, Patel S
Curr Oncol Rep 2013 Aug;15(4):347-55. doi: 10.1007/s11912-013-0328-2. PMID: 23852636

Clinical prediction guides

Hepatic epithelioid hemangioendothelioma: A clinocopathological correlation.
Mundada AD, Deodhar K, Ramadwar M, Bal M, Kumar R
Indian J Pathol Microbiol 2022 Jan-Mar;65(1):133-136. doi: 10.4103/ijpm.ijpm_350_21. PMID: 35074978
Prognostic stratification of clinical and molecular epithelioid hemangioendothelioma subsets.
Rosenbaum E, Jadeja B, Xu B, Zhang L, Agaram NP, Travis W, Singer S, Tap WD, Antonescu CR
Mod Pathol 2020 Apr;33(4):591-602. Epub 2019 Sep 19 doi: 10.1038/s41379-019-0368-8. PMID: 31537895Free 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
Pulmonary epithelioid hemangioendothelioma.
Mizuno Y, Iwata H, Shirahashi K, Hirose Y, Takemura H
Gen Thorac Cardiovasc Surg 2011 Apr;59(4):297-300. Epub 2011 Apr 12 doi: 10.1007/s11748-010-0651-6. PMID: 21484560

Recent systematic reviews

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
Recurrence of Epithelioid Hemangioendothelioma during Pregnancy: Case Report and Systematic Review.
Mcculloch M, Russin M, Nachat A
Perm J 2016 Summer;20(3):15-152. Epub 2016 Jul 25 doi: 10.7812/TPP/15-152. PMID: 27479949Free PMC Article
Novel rescue procedure for inferior vena cava reconstruction in living-donor liver transplantation using a vascular graft recovered 25 h after donors' circulatory death and systematic review.
Palma AF, Oberkofler CE, Raptis DA, Eshmuminov D, de Rougemont O, Schnyder A, Dimitroulis D, Lesurtel M, Dutkowski P, Clavien PA
Transpl Int 2014 Feb;27(2):204-10. Epub 2013 Dec 2 doi: 10.1111/tri.12238. PMID: 24289717
Liver transplantation for benign hepatic tumors: a systematic review.
Ercolani G, Grazi GL, Pinna AD
Dig Surg 2010;27(1):68-75. Epub 2010 Apr 1 doi: 10.1159/000268628. PMID: 20357454

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    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

    Reviews

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