# A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid haemangioendothelioma

Source: https://onco.cc/key-papers/paper-errani-wwtr1-camta1-ehe-gcc-2011/  
OnCo record `paper-errani-wwtr1-camta1-ehe-gcc-2011` (Key paper). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

This study identified the WWTR1-CAMTA1 gene fusion in nearly every epithelioid haemangioendothelioma from any body site, giving this rare vascular tumour a defining molecular marker.

## Summary

Molecular study identifying a t(1;3) translocation fusing WWTR1 (encoding TAZ) to CAMTA1 in epithelioid haemangioendothelioma of soft tissue, bone, liver and lung, present in almost all cases and absent from other vascular tumours.

## Fields

- Kind: Key paper
- Last checked: 2026-09-17
- Journal: Genes, chromosomes & cancer
- Year: 2011
- DOI: 10.1002/gcc.20886
- Authors: Errani C, Zhang L, Sung YS, et al.
- Findings: WWTR1-CAMTA1 fusion in the vast majority of epithelioid haemangioendotheliomas across sites.
- What it means: CAMTA1 immunohistochemistry and fusion testing confirm the diagnosis, and the TAZ-CAMTA1 fusion protein's dependence on the Hippo pathway is guiding drug development.
- Caveats: A minority of cases carry an alternative YAP1-TFE3 fusion.

## Sources

- Genes Chromosomes Cancer 2011: https://doi.org/10.1002/gcc.20886
- PubMed: https://pubmed.ncbi.nlm.nih.gov/21584898/

## Connected records

- cancers: [Epithelioid haemangioendothelioma](https://onco.cc/cancers/epithelioid-haemangioendothelioma/)
- journals: [Genes, chromosomes & cancer](https://onco.cc/journals/genes-chromosomes-and-cancer/)

---
JSON: https://onco.cc/api/v1/entities/paper-errani-wwtr1-camta1-ehe-gcc-2011.json