- Human pathology

Home > F. Pathology by regions > Thorax > thoracic epithelioid malignant vascular tumor

thoracic epithelioid malignant vascular tumor

Friday 23 January 2015

Malignant thoracic epithelioid vascular tumors are an uncommon and heterogenous group of tumors that include low-grade to intermediate-grade epithelioid hemangioendothelioma (EHE) and high-grade epithelioid angiosarcoma (EAS).


Fluorescence in situ hybridization analysis showed CAMTA1-WWTR1 fusions in 4/7 low-grade and 23/23 intermediate-grade EHE (P@<@0.001).

In EAS, CAMTA1 rearrangement was negative in all cases, whereas a WWTR1 complex abnormality was found in 1/5 cases (P@<@0.001).

This offers an objective means of differentiating intermediate-grade EHE from EAS, especially on limited biopsies.


In a series, all cases show expression of at least one vascular marker, which allows differentiation from primary thoracic epithelial malignancies, although keratin expression is a potential pitfall with 29% of EHE and 25% of EAS showing keratin expression. (25353289)

In a series, survival analysis shows that higher tumor grade for all tumors (P=0.026) as well as lung and pleural tumors only (P=0.010) and the presence of pleural involvement in lung and/or pleural tumors (P=0.042) correlate with poor prognosis. (25353289)

Open references

- Thoracic Epithelioid Malignant Vascular Tumors: A Clinicopathologic Study of 52 Cases With Emphasis on Pathologic Grading and Molecular Studies of WWTR1-CAMTA1 Fusions. Anderson T, Zhang L, Hameed M, Rusch V, Travis WD, Antonescu CR. Am J Surg Pathol. 2015 Jan;39(1):132-9. doi : 10.1097/PAS.0000000000000346 PMID: 25353289 [Free in PMC]