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Cardiac Epithelioid Hemangioendothelioma

MedGen UID:
232024
•Concept ID:
C1332846
•
Neoplastic Process
Synonyms: Cardiac Epithelioid Angioendothelioma; Epithelioid Angioendothelioma of Heart; Epithelioid Angioendothelioma of the Heart; Epithelioid Hemangioendothelioma of Heart; Epithelioid Hemangioendothelioma of the Heart; Heart Epithelioid Angioendothelioma; Heart Epithelioid Hemangioendothelioma

Definition

A low-grade malignant blood vessel neoplasm, arising from the heart. IIt 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. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Irreversible Electroporation as a Valid Treatment Option for Hepatic Epithelioid Hemangioendothelioma: An International Multicenter Experience.
Narayanan G, Spano A, Gentile NT, Shnayder-Adams MM, Gurusamy V, Levi DM, Wilky BA, Mora RA, Noman R, Peddu P, Dijkstra M
Cardiovasc Intervent Radiol 2024 Jul;47(7):883-890. Epub 2024 Jun 6 doi: 10.1007/s00270-024-03770-5. PMID: 38844684Free PMC Article

Recent clinical studies

Etiology

Cardiac epithelioid hemangioendothelioma with 8-year follow-up.
Safirstein J, Aksenov S, Smith F
Cardiovasc Pathol 2007 May-Jun;16(3):183-6. Epub 2006 Dec 18 doi: 10.1016/j.carpath.2006.10.003. PMID: 17502249
Atypical primary epithelioid hemangioendothelioma of the heart.
Moulai N, Chavanon O, Guillou L, Noirclerc M, Blin D, Brambilla E, Lantuejoul S
J Thorac Oncol 2006 Feb;1(2):188-9. PMID: 17409853

Diagnosis

A case report of right atrial epithelioid hemangioendothelioma with multiple pulmonary metastases.
Zhou X, Li P, Gu X, Zheng F, Zhao J, Zhao L
Clin Respir J 2020 Feb;14(2):173-178. Epub 2019 Dec 17 doi: 10.1111/crj.13121. PMID: 31794151
Atypical primary epithelioid hemangioendothelioma of the heart.
Moulai N, Chavanon O, Guillou L, Noirclerc M, Blin D, Brambilla E, Lantuejoul S
J Thorac Oncol 2006 Feb;1(2):188-9. PMID: 17409853
Epithelioid hemangioendothelioma of the heart with distant metastases. A case report and literature review.
Marchiano D, Fisher F, Hofstetter S
J Cardiovasc Surg (Torino) 1993 Dec;34(6):529-33. PMID: 8300722

Prognosis

Cardiac epithelioid hemangioendothelioma with 8-year follow-up.
Safirstein J, Aksenov S, Smith F
Cardiovasc Pathol 2007 May-Jun;16(3):183-6. Epub 2006 Dec 18 doi: 10.1016/j.carpath.2006.10.003. PMID: 17502249
Atypical primary epithelioid hemangioendothelioma of the heart.
Moulai N, Chavanon O, Guillou L, Noirclerc M, Blin D, Brambilla E, Lantuejoul S
J Thorac Oncol 2006 Feb;1(2):188-9. PMID: 17409853

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