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Liver Epithelioid Hemangioendothelioma

MedGen UID:
473525
•Concept ID:
C2064402
•
Neoplastic Process
Synonyms: Epithelioid hemangioendothelioma of liver; Hepatic Epithelioid Hemangioendothelioma
SNOMED CT: Epithelioid hemangioendothelioma of liver (860831008)

Definition

A low-grade malignant blood vessel neoplasm that arises from the liver. It is characterized by the presence of epithelioid endothelial cells. The neoplastic cells are arranged in cords and nests, which are embedded in a myxoid to hyalinized stroma. Patients may be asymptomatic or present with an abdominal mass, ascites, and jaundice. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLiver Epithelioid Hemangioendothelioma

Professional guidelines

PubMed

The challenging surgical management of hepatic epithelioid hemangioendothelioma: a narrative review.
Giovanardi F, Larghi Laureiro Z, Meo GA, Hassan R, Lai Q
Chin Clin Oncol 2022 Aug;11(4):27. doi: 10.21037/cco-21-139. PMID: 36098098
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
Primary mesenchymal liver tumors: radiological spectrum, differential diagnosis, and pathologic correlation.
Harman M, Nart D, Acar T, Elmas N
Abdom Imaging 2015 Jun;40(5):1316-30. doi: 10.1007/s00261-014-0268-9. PMID: 25311993

Recent clinical studies

Etiology

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
Laparoscopic resection of hepatic epithelioid hemangioendothelioma: report of eleven rare cases and literature review.
Xu J, Hu S, Li S, Wang W, Zhou X, Wu Y, Su Z, Cheng X, Gao Y, Zheng Q
World J Surg Oncol 2020 Oct 29;18(1):282. doi: 10.1186/s12957-020-02034-z. PMID: 33121478Free PMC Article
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
Malignant liver tumors.
Suriawinata AA, Thung SN
Clin Liver Dis 2002 May;6(2):527-54, ix. doi: 10.1016/s1089-3261(02)00005-3. PMID: 12122869

Diagnosis

Mesenchymal Neoplasms of the Liver.
Papke DJ Jr
Surg Pathol Clin 2023 Sep;16(3):609-634. Epub 2023 Jun 11 doi: 10.1016/j.path.2023.04.013. PMID: 37536892
Intra-abdominal epithelioid angiosarcoma.
Torres Jurado MJ, López Sánchez J, Quiñones Sampedro JE, Muñoz Bellvís L
Rev Esp Enferm Dig 2022 Dec;114(12):744-745. doi: 10.17235/reed.2022.8878/2022. PMID: 35607922
Rare malignant liver tumors in children.
Chavhan GB, Siddiqui I, Ingley KM, Gupta AA
Pediatr Radiol 2019 Oct;49(11):1404-1421. Epub 2019 Oct 16 doi: 10.1007/s00247-019-04402-8. PMID: 31620842
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
Malignant liver tumors.
Suriawinata AA, Thung SN
Clin Liver Dis 2002 May;6(2):527-54, ix. doi: 10.1016/s1089-3261(02)00005-3. PMID: 12122869

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
Pleural epithelioid hemangioendothelioma.
Lee YJ, Chung MJ, Jeong KC, Hahn CH, Hong KP, Kim YJ, Kim YT
Yonsei Med J 2008 Dec 31;49(6):1036-40. doi: 10.3349/ymj.2008.49.6.1036. PMID: 19108030Free PMC Article

Prognosis

A Review of the Spectrum of Imaging Manifestations of Epithelioid Hemangioendothelioma.
Jang JK, Thomas R, Braschi-Amirfarzan M, Jagannathan JP
AJR Am J Roentgenol 2020 Nov;215(5):1290-1298. Epub 2020 Aug 25 doi: 10.2214/AJR.20.22876. PMID: 32841059
Rare malignant liver tumors in children.
Chavhan GB, Siddiqui I, Ingley KM, Gupta AA
Pediatr Radiol 2019 Oct;49(11):1404-1421. Epub 2019 Oct 16 doi: 10.1007/s00247-019-04402-8. PMID: 31620842
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
Epithelioid hemangioendotheliomas of the liver and lung in children and adolescents.
Hettmer S, Andrieux G, Hochrein J, Kurz P, Rössler J, Lassmann S, Werner M, von Bubnoff N, Peters C, Koscielniak E, Sparber-Sauer M, Niemeyer C, Mentzel T, Busch H, Boerries M
Pediatr Blood Cancer 2017 Dec;64(12) Epub 2017 Jun 9 doi: 10.1002/pbc.26675. PMID: 28598585
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

A review of hepatic epithelioid hemangioendothelioma-Analyzing patient characteristics and treatment strategies.
Ajay PS, Tsagkalidis V, Casabianca A, Burchard PR, Melucci AD, Chacon A, Goyal S, Switchenko JM, Kooby DA, Carpizo DR, Shah MM
J Surg Oncol 2022 Dec;126(8):1423-1429. Epub 2022 Aug 17 doi: 10.1002/jso.27066. PMID: 35975699Free PMC Article
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
Imaging characteristics and prognostic values of hepatic epithelioid hemangioendothelioma on (18)F-FDG PET/CT.
Wang W, Liu G, Hu P, Pang L, Gu T, Yu H, Luo R, Yang X, Shi H
Clin Exp Med 2020 Nov;20(4):557-567. Epub 2020 Aug 14 doi: 10.1007/s10238-020-00653-0. PMID: 32797371
Hepatic Epithelioid Hemangioendothelioma: A Rare Disease With Favorable Outcomes After Liver Transplantation.
Krasnodębski M, Grąt M, Morawski M, Wierzchowski M, Jastrzębski M, Remiszewski P, Zając K, Patkowski W, Zieniewicz K
Transplant Proc 2020 Oct;52(8):2447-2449. Epub 2020 Mar 23 doi: 10.1016/j.transproceed.2020.02.101. PMID: 32217012
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

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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