CAMTA-1 Expression in 24 Cases of Hepatic Epithelioid Hemangioendothelioma in a Single Institute: Diagnostic Utility for Differential Diagnosis from Hepatic Angiosarcoma

In Vivo. 2019 Nov-Dec;33(6):2293-2297. doi: 10.21873/invivo.11736.


Background/aim: Epithelioid hemangio-endothelioma (EHE) of the liver is an uncommon vascular tumor with variable clinical courses ranging from stable disease to fatal outcome. EHE can mimic epithelioid angiosarcoma, which has a more aggressive behavior, especially in a small biopsy sample. EHEs are known to have the WWTR1-CAMTA1 fusion gene, and nuclear expression of CAMTA1 by immunohistochemistry (IHC) has been reported in about 90% of EHEs in multiple organs. Our study aimed to validate the diagnostic utility of CAMTA1 expression in EHEs, especially in the liver.

Patients and methods: IHC was performed using anti-CAMTA1 antibody in 34 tumors (24 hepatic EHEs and 10 angiosarcomas). In CAMTA1-negative EHEs, TFE3 IHC was performed.

Results: Of the 24 hepatic EHEs, 22 (91.6%) showed nuclear staining for CAMTA1. One of two CAMTA1-negative cases showed TFE3 positivity. The other case was negative for TFE3. Meanwhile, all 10 angiosarcoma cases had no CAMTA1 expression.

Conclusion: CAMTA1 is a highly sensitive and specific marker for diagnosis of hepatic EHE. It is helpful for differentiation of hepatic EHE and angiosarcoma, especially in small biopsy samples.

Keywords: CAMTA1; TFE3; angiosarcoma; cancer; epithelioid hemangioendothelioma; liver; prognosis.

MeSH terms

  • Adult
  • Aged
  • Biomarkers, Tumor*
  • Calcium-Binding Proteins / genetics*
  • Diagnosis, Differential
  • Disease Management
  • Female
  • Gene Expression*
  • Hemangioendothelioma, Epithelioid / diagnosis*
  • Hemangioendothelioma, Epithelioid / genetics*
  • Hemangioendothelioma, Epithelioid / mortality
  • Hemangioendothelioma, Epithelioid / therapy
  • Hemangiosarcoma / diagnosis
  • Humans
  • Immunohistochemistry
  • In Situ Hybridization, Fluorescence
  • Liver Neoplasms / diagnosis*
  • Liver Neoplasms / genetics*
  • Liver Neoplasms / mortality
  • Liver Neoplasms / therapy
  • Male
  • Middle Aged
  • Trans-Activators / genetics*
  • Treatment Outcome
  • Young Adult


  • Biomarkers, Tumor
  • CAMTA1 protein, human
  • Calcium-Binding Proteins
  • Trans-Activators