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pulmonary epithelioid hemangioendothelioma

Monday 2 January 2012

intravascular bronchioalveolar tumor, IVBAT, PEH, TEH

Pulmonary epithelioid hemangioendothelioma (PEH), previously known as "intravascular bronchoalveolar tumor," is a rare vascular malignancy with an unpredictable prognosis.

Treatment can vary from observation in asymptomatic patients to surgery in patients with resectable disease or chemotherapy in patients with disseminated disease.

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor. It can present either as one solitary nodule or bilateral multiple nodules, usually without calcification.

Epithelioid hemangioendothelioma (formerly known as intravascular bronchioalveolar tumor, IVBAT) is an uncommon tumor of vascular endothelial origin, with an intermediate course between hemangioma and conventional angiosarcoma.

PEH typically occurs as bilateral multiple nodules among young women. But some cases also develop as a solitary lung nodule or a single cavitary nodule.

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).


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)


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

In epithelioid angiosarcoma (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.

See also

- pulmonary vascular tumors
- epithelioid hemangioendothelioma

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]

- Pulmonary epithelioid hemangioendothelioma. Rosengarten D, Kramer MR, Amir G, Fuks L, Berkman N. Isr Med Assoc J. 2011 Nov;13(11):676-9. PMID: 22279701 (Free)

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