What is new in endothelial neoplasia?

Virchows Arch. 2020 Jan;476(1):17-28. doi: 10.1007/s00428-019-02651-4. Epub 2019 Aug 28.

Abstract

The classification of vascular neoplasms continues to evolve as we accumulate more genetic and clinical data, particularly for rare tumor types. Because of tumor rarity, changes to classification schema, overlapping histologic features, and in some cases, lack of morphologic evidence of vasoformation, vascular neoplasms present a diagnostic challenge. Here, we discuss recent developments in our understanding of vascular tumors, with a detailed discussion of epithelioid hemangioma, tufted angioma, kaposiform hemangioendothelioma, composite hemangioendothelioma, pseudomyogenic hemangioendothelioma, epithelioid hemangioendothelioma, and angiosarcoma.

Keywords: Angiosarcoma; Gene fusion; Hemangioendothelioma; Hemangioma; Immunohistochemistry.

Publication types

  • Review

MeSH terms

  • Calcium-Binding Proteins / genetics
  • Hemangioendothelioma / genetics
  • Hemangioendothelioma / pathology
  • Hemangioendothelioma, Epithelioid / genetics
  • Hemangioendothelioma, Epithelioid / pathology
  • Hemangioma / genetics
  • Hemangioma / pathology
  • Hemangiosarcoma / genetics
  • Hemangiosarcoma / pathology
  • Humans
  • Kasabach-Merritt Syndrome / genetics
  • Kasabach-Merritt Syndrome / pathology
  • Proto-Oncogene Proteins c-myc / analysis
  • Sarcoma, Kaposi / genetics
  • Sarcoma, Kaposi / pathology
  • Skin Neoplasms / genetics
  • Skin Neoplasms / pathology
  • Trans-Activators / genetics
  • Transcriptional Coactivator with PDZ-Binding Motif Proteins
  • Vascular Neoplasms / genetics
  • Vascular Neoplasms / pathology*

Substances

  • CAMTA1 protein, human
  • Calcium-Binding Proteins
  • MYC protein, human
  • Proto-Oncogene Proteins c-myc
  • Trans-Activators
  • Transcriptional Coactivator with PDZ-Binding Motif Proteins
  • WWTR1 protein, human

Supplementary concepts

  • Kaposiform Hemangioendothelioma
  • Tufted angioma