Epithelioid Hemangioendothelioma
Key Facts
Terminology
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Epithelioid hemangioendothelioma (EH)
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Synonym: Intravascular bronchioloalveolar tumor (IVBT)
Clinical Issues
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Incidence
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Unusual tumor in lung
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Appears to be more common in young adults; age range: 7-72 years
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More common in women
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Symptoms
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Cough
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Dyspnea
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Pleuritic pain
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Asymptomatic
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Presentation
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Bilateral pulmonary infiltrates
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Treatment
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There is no specific treatment
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Top Differential Diagnoses
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Angiosarcoma
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Usually shows high mitotic count
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May also show areas of necrosis
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Shows more nuclear atypia
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Metastatic carcinoma
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Shows positive staining for epithelial markers (keratins and EMA/MUC1)
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Negative for vascular markers (FVIIIRAg, CD31, and CD34)
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Metastatic chordoma
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Also negative for vascular markers (CD31, CD34)
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Negative history of chordoma elsewhere
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Classical presentation of epithelioid hemangioendothelioma in the lung. Note the presence of a nodule replacing lung parenchyma and infiltrating alveolar spaces. |
High magnification of epithelioid hemangioendothelioma shows a chondromyxoid background with scattered epithelioid cells embedded in the stroma. |
TERMINOLOGY
Abbreviations
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Epithelioid hemangioendothelioma (EH)
Synonyms
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Intravascular bronchioloalveolar tumor (IVBT)
Definitions
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Vascular neoplasm of low- to intermediate-grade malignancy
ETIOLOGY/PATHOGENESIS
Etiology
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Vascular origin from precursor mesenchymal cells
CLINICAL ISSUES
Epidemiology
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Incidence
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Tumor is unusual in lung
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Age
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Appears to be more common in young adults; however, age may range from 7-72 years old
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Gender
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More common in women
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Site
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Usually bilateral pulmonary infiltrates
Presentation
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Cough
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Shortness of breath
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Pleuritic pain
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Asymptomatic
Treatment
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There is no specific treatment
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Radiation and chemotherapy can be attempted
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Steroids have been tried
Prognosis
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Unpredictable
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Spontaneous regression can occur
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Protracted course may follow
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Aggressive course with fatal outcome may occur
IMAGE FINDINGS
General Features
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Bilateral pulmonary infiltrates
MACROSCOPIC FEATURES
General Features
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Multiple nodules replacing normal lung parenchyma
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Nodules are soft or may be mucoid in consistency
Size
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Nodules may measure from a few mm to 2-3 cm
MICROSCOPIC PATHOLOGY
Histologic Features
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Multiple pulmonary nodules
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Nodules in different phases of development
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Prominent chondromyxoid background
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Epithelioid cells embedded in chondromyxoid background
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Low mitotic count
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Signet ring cell-like cellular appearance
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Cluster of tumor cells in polypoid intraalveolar pattern
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Extravasated red cells
Predominant Pattern/Injury Type
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Chondromyxoid
Predominant Cell/Compartment Type
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Epithelioid
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