Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Pulmonary epithelioid hemangioendothelioma
- Radiologic Findings
- Fig 1-4. Chest radiograph and chst CT images show numerous well-defined tiny (<10mm) noncalcified nodules of random distribution scattered throughout the lungs.
Fig 5. The 18F-FDG PET/CT scan shows nodules without significant hypermetabolism.
- Brief Review
- Pulmonary epithelioid hemangioendothelioma is a rare vascular neoplasm, with an intermediate course between hemangioma and conventional angiosarcoma. It is often diagnosed asymptomatically in middle-aged patients and tends to be more common in female.
The most common CT finding of pulmonary epitheloid hemangioendothelioma is the presence of multiple discrete nodules. The nodules range in size up to 2cm but most are less than 1cm in diameter. On serial CT examinations, these lesions have little or no growth. Other findings include multiple reticulonodular opacities mimicking hematolymphangitic metastasis and diffuse infiltrative pleural thickening mimicking malignant pleural mesothelioma or diffuse pleural carcinomatosis.
Unilateral single nodules may be surgically removed. Patients with diffuse lung lesions are mainly treated with chemotherapy, although no single chemotherapy agent has demonstrated efficiency in treating pulmonary epitheloid hemangioendothelioma.
The prognosis varies with spontaneous regression, and some have blood vessels, lymphatics, pleural cavities, and distant hematogenous spreads. The poor prognostic factors of PEH include the presence of respiratory symptoms or pleural effusion revealed by chest radiography on presentation; extensive intravascular, endobronchial or interstitial tumor spreading, hepatic metastases; peripheral lymphadenopathy; the presence of spindle cells in the tumor; and, particularly, pleural hemorrhagic effusions and hemoptysis. The 5-year survival probability is 60%.
- References
- 1. Mesquita RD, Sousa M, Trinidad C, Pinto E, Badiola IA. New insights about pulmonary epithelioid hemangioendothelioma: review of the literature and two case reports. Case Rep Radiol 2017;2017:5972940
2. KS Lee, J Han, Chung MP, YJ Jeong. Radiology Illustrated Chest Radiology 2014 Springer p.190
3. Ye B, Li W, Feng J, Shi JX, Chen Y, Han BH. Treatment of pulmonary epithelioid hemangioendothelioma with combination chemotherapy: Report of three cases and review of the literature. Oncol Lett. 2013 May;5(5):1491-1496.
- Keywords