Yan Epelboym1, Dawn R Engelkemier1, Frederic Thomas-Chausse1, Ahmad I Alomari2, Alyaa Al-Ibraheemi3, Cameron C Trenor4, Denise M Adams4, Gulraiz Chaudry5. 1. Division of Vascular and Interventional Radiology, Boston Children's Hospital and Harvard Medical School, United States of America. 2. Division of Vascular and Interventional Radiology, Boston Children's Hospital and Harvard Medical School, United States of America; Vascular Anomalies Center, Boston Children's Hospital and Harvard Medical School, United States of America. 3. Department of Pathology, Boston Children's Hospital and Harvard Medical School, United States of America; Vascular Anomalies Center, Boston Children's Hospital and Harvard Medical School, United States of America. 4. Division of Hematology/Oncology, Boston Children's Hospital and Harvard Medical School, United States of America; Vascular Anomalies Center, Boston Children's Hospital and Harvard Medical School, United States of America. 5. Division of Vascular and Interventional Radiology, Boston Children's Hospital and Harvard Medical School, United States of America; Vascular Anomalies Center, Boston Children's Hospital and Harvard Medical School, United States of America. Electronic address: gulraiz.chaudry@childrens.harvard.edu.
Abstract
PURPOSE- OBJECTIVE: Epithelioid hemangioendothelioma (EHE) is a rare vascular malignancy with varying biologic behavior. The purpose of this study was to identify imaging findings most characteristic of EHE. METHODS: Retrospective review of clinical and imaging records in patients referred to our Vascular Anomalies Center over a 17 year period with biopsy proven EHE. RESULTS: We evaluated 29 patients (17 F) with median age of 16 years (range 2-76 y). The most common presenting symptoms were pain (n = 13) and palpable mass (n = 7). 22 (70%) had multifocal disease. Most common sites of involvement were lung (n = 25), liver (n = 16), bone (n = 12), soft tissue (n = 3) and lymph nodes (n = 1). Of patients with single site disease, 3 had lung, 3 liver, and 1 had bone lesions. In 18/25 with lung disease, there were multiple nodules of varying sizes and characteristics. In 14/16 with hepatic disease there were multiple nodules with predominantly peripheral distribution. Subcapsular retraction was seen in 10/16 and a "lollipop" sign (hepatic or portal vein tapering at the edge of a well-defined hypoenhancing lesion) identified in 5/16. Of 12 osseous lesions, 11 were lytic, 8 involved vertebrae and 9 involved the axial skeleton. CONCLUSION: EHE has varied imaging findings. The most common sites are lungs, liver, and bone, with multi-organ involvement seen in most. Lung disease is most commonly characterized by multiple nodules. Hepatic lesions demonstrate the most distinctive findings, with peripheral distribution, lack of early enhancement, subcapsular retraction and "lollipop" sign. Osseous lesions are commonly lytic and more prevalent in the axial skeleton.
PURPOSE- OBJECTIVE:Epithelioid hemangioendothelioma (EHE) is a rare vascular malignancy with varying biologic behavior. The purpose of this study was to identify imaging findings most characteristic of EHE. METHODS: Retrospective review of clinical and imaging records in patients referred to our Vascular Anomalies Center over a 17 year period with biopsy proven EHE. RESULTS: We evaluated 29 patients (17 F) with median age of 16 years (range 2-76 y). The most common presenting symptoms were pain (n = 13) and palpable mass (n = 7). 22 (70%) had multifocal disease. Most common sites of involvement were lung (n = 25), liver (n = 16), bone (n = 12), soft tissue (n = 3) and lymph nodes (n = 1). Of patients with single site disease, 3 had lung, 3 liver, and 1 had bone lesions. In 18/25 with lung disease, there were multiple nodules of varying sizes and characteristics. In 14/16 with hepatic disease there were multiple nodules with predominantly peripheral distribution. Subcapsular retraction was seen in 10/16 and a "lollipop" sign (hepatic or portal vein tapering at the edge of a well-defined hypoenhancing lesion) identified in 5/16. Of 12 osseous lesions, 11 were lytic, 8 involved vertebrae and 9 involved the axial skeleton. CONCLUSION: EHE has varied imaging findings. The most common sites are lungs, liver, and bone, with multi-organ involvement seen in most. Lung disease is most commonly characterized by multiple nodules. Hepatic lesions demonstrate the most distinctive findings, with peripheral distribution, lack of early enhancement, subcapsular retraction and "lollipop" sign. Osseous lesions are commonly lytic and more prevalent in the axial skeleton.