Vascular tumors of intermediate malignancy: An update

Hum Pathol. 2024 May:147:114-128. doi: 10.1016/j.humpath.2024.01.014. Epub 2024 Feb 13.


The term "hemangioendothelioma" is used for endothelial neoplasms of intermediate malignancy and describes a group of rare neoplasms having biologic behavior falling in between that of the benign hemangiomas and fully malignant angiosarcomas. The hemangioendotheliomas fall into several specific, clinicopathologically and genetically distinct entities, specifically epithelioid hemangioendothelioma, kaposiform hemangioendothelioma, papillary intralymphatic angioendothelioma and retiform hemangioendothelioma (hobnailed hemangioendothelioma), pseudomyogenic hemangioendothelioma, composite hemangioendothelioma, and YAP1::TFE3-fused hemangioendothelioma. The clinical, morphologic, immunohistochemical, and genetic features, and the differential diagnosis of each of these rare entities are discussed in this review.

Keywords: Composite hemangioendothelioma; Epithelioid hemangioendothelioma; Immunohistochemistry; Kaposiform hemangioendothelioma; Molecular genetics; Papillary intralymphatic angioendothelioma; Pseudomyogenic hemangioendothelioma; Retiform hemangioendothelioma; Sarcoma; YAP1::TFE3-Fused hemangioendothelioma.

Publication types

  • Review

MeSH terms

  • Biomarkers, Tumor* / analysis
  • Biomarkers, Tumor* / genetics
  • Diagnosis, Differential
  • Genetic Predisposition to Disease
  • Hemangioendothelioma* / diagnosis
  • Hemangioendothelioma* / genetics
  • Hemangioendothelioma* / pathology
  • Hemangioendothelioma, Epithelioid / genetics
  • Hemangioendothelioma, Epithelioid / pathology
  • Humans
  • Immunohistochemistry
  • Vascular Neoplasms / genetics
  • Vascular Neoplasms / pathology
  • YAP-Signaling Proteins


  • Biomarkers, Tumor
  • YAP-Signaling Proteins