TriangleHEALTH PROFESSIONAL · SOURCE READING
Clinical presentation and diagnostic evaluation
Source: Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 21, 2025 · Captured 2026-09-09.
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Context: Treatment of Vascular Tumors / Epithelioid Hemangioendothelioma NOS
Common sites of involvement are liver alone (21%), liver plus lung (18%), lung alone (12%), and bone alone (14%).[4,10,11] Clinical presentation depends on the site of involvement, as follows:
Liver: Hepatic nodules have central vascularity on ultrasound, contrast-enhancing lesions by computed tomography, and low T1 signal and moderate T2 signal on magnetic resonance imaging. These may be incidental findings in asymptomatic patients, but most patients commonly present with signs or symptoms of cholestasis, including pruritus, jaundice, or scleral icterus.
Lung: Pulmonary epithelioid hemangioendothelioma may be an asymptomatic finding on chest x-ray or be associated with pleuritic pain, hemoptysis, anemia, and fibrosis.
Bone: Bone metastasis may be associated with pathological fracture. On x-rays, they are well-defined osteolytic lesions and can be multiple or solitary.
Soft tissue: Thirty percent of soft tissue cases are associated with metastases. When present, metastatic disease can be very aggressive and have a limited response to chemotherapy.
Skin: Cutaneous lesions can be raised and nodular or can be warm, red-brown plaques.
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