Epithelioid Hemangioendothelioma of the Thoracic Aorta Resulting in Aortic Obstruction and Congestive Heart Failure
1999; Lippincott Williams & Wilkins; Volume: 100; Issue: 5 Linguagem: Inglês
10.1161/01.cir.100.5.564
ISSN1524-4539
AutoresJay H. Traverse, John R. Lesser, Björn P. Flygenring, Thomas H. Bracken, Olga Olevsky, Demétre M. Nicoloff, Thomas F. Flavin, Charles A. Horwitz, Robert G. Hauser,
Tópico(s)Vascular Malformations and Hemangiomas
ResumoHomeCirculationVol. 100, No. 5Epithelioid Hemangioendothelioma of the Thoracic Aorta Resulting in Aortic Obstruction and Congestive Heart Failure Free AccessOtherPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessOtherPDF/EPUBEpithelioid Hemangioendothelioma of the Thoracic Aorta Resulting in Aortic Obstruction and Congestive Heart Failure Jay H. Traverse, John R. Lesser, Bjorn P. Flygenring, Thomas H. Bracken, Olga M. Olevsky, Demetre M. Nicoloff, Thomas Flavin, Charles A. Horwitz and Robert G. Hauser Jay H. TraverseJay H. Traverse From the Minneapolis Heart Institute at Abbott Northwestern Hospital (J.H.T., J.R.L., B.P.F., D.M.N., T.F., R.G.H.), the Department of Internal Medicine (O.M.O.), the Department of Pathology (C.A.H.), and the Cardiovascular Division (J.H.T.), University of Minnesota Medical School, Minneapolis, and Mille Lacs Health System, Onamia, Minn (T.H.B.). , John R. LesserJohn R. Lesser From the Minneapolis Heart Institute at Abbott Northwestern Hospital (J.H.T., J.R.L., B.P.F., D.M.N., T.F., R.G.H.), the Department of Internal Medicine (O.M.O.), the Department of Pathology (C.A.H.), and the Cardiovascular Division (J.H.T.), University of Minnesota Medical School, Minneapolis, and Mille Lacs Health System, Onamia, Minn (T.H.B.). , Bjorn P. FlygenringBjorn P. Flygenring From the Minneapolis Heart Institute at Abbott Northwestern Hospital (J.H.T., J.R.L., B.P.F., D.M.N., T.F., R.G.H.), the Department of Internal Medicine (O.M.O.), the Department of Pathology (C.A.H.), and the Cardiovascular Division (J.H.T.), University of Minnesota Medical School, Minneapolis, and Mille Lacs Health System, Onamia, Minn (T.H.B.). , Thomas H. BrackenThomas H. Bracken From the Minneapolis Heart Institute at Abbott Northwestern Hospital (J.H.T., J.R.L., B.P.F., D.M.N., T.F., R.G.H.), the Department of Internal Medicine (O.M.O.), the Department of Pathology (C.A.H.), and the Cardiovascular Division (J.H.T.), University of Minnesota Medical School, Minneapolis, and Mille Lacs Health System, Onamia, Minn (T.H.B.). , Olga M. OlevskyOlga M. Olevsky From the Minneapolis Heart Institute at Abbott Northwestern Hospital (J.H.T., J.R.L., B.P.F., D.M.N., T.F., R.G.H.), the Department of Internal Medicine (O.M.O.), the Department of Pathology (C.A.H.), and the Cardiovascular Division (J.H.T.), University of Minnesota Medical School, Minneapolis, and Mille Lacs Health System, Onamia, Minn (T.H.B.). , Demetre M. NicoloffDemetre M. Nicoloff From the Minneapolis Heart Institute at Abbott Northwestern Hospital (J.H.T., J.R.L., B.P.F., D.M.N., T.F., R.G.H.), the Department of Internal Medicine (O.M.O.), the Department of Pathology (C.A.H.), and the Cardiovascular Division (J.H.T.), University of Minnesota Medical School, Minneapolis, and Mille Lacs Health System, Onamia, Minn (T.H.B.). , Thomas FlavinThomas Flavin From the Minneapolis Heart Institute at Abbott Northwestern Hospital (J.H.T., J.R.L., B.P.F., D.M.N., T.F., R.G.H.), the Department of Internal Medicine (O.M.O.), the Department of Pathology (C.A.H.), and the Cardiovascular Division (J.H.T.), University of Minnesota Medical School, Minneapolis, and Mille Lacs Health System, Onamia, Minn (T.H.B.). , Charles A. HorwitzCharles A. Horwitz From the Minneapolis Heart Institute at Abbott Northwestern Hospital (J.H.T., J.R.L., B.P.F., D.M.N., T.F., R.G.H.), the Department of Internal Medicine (O.M.O.), the Department of Pathology (C.A.H.), and the Cardiovascular Division (J.H.T.), University of Minnesota Medical School, Minneapolis, and Mille Lacs Health System, Onamia, Minn (T.H.B.). and Robert G. HauserRobert G. Hauser From the Minneapolis Heart Institute at Abbott Northwestern Hospital (J.H.T., J.R.L., B.P.F., D.M.N., T.F., R.G.H.), the Department of Internal Medicine (O.M.O.), the Department of Pathology (C.A.H.), and the Cardiovascular Division (J.H.T.), University of Minnesota Medical School, Minneapolis, and Mille Lacs Health System, Onamia, Minn (T.H.B.). Originally published3 Aug 1999https://doi.org/10.1161/01.CIR.100.5.564Circulation. 1999;100:564–565A54-year-old white woman was referred for cardiac consultation because of increased dyspnea with exertion and leg pain. The patient's past medical history was significant for smoking and recent onset of hypertension. On physical examination, she was found to have a blood pressure of 180/90 mm Hg and a heart rate of 110 bpm. The lungs were clear to auscultation. The carotid upstrokes were bounding, and there were bilateral carotid bruits. The central venous pressure was not elevated. The cardiac examination was significant for a loud, harsh systolic murmur (II to III/VI) across the precordium with radiation to the back. There was a left ventricular lift and a third heart sound. The abdominal examination was normal, and stool guaiac was negative. There was no peripheral edema. The femoral pulses were diminished.The ECG showed normal sinus rhythm, left atrial enlargement, incomplete right bundle-branch block, and voltage and T-wave changes suggestive of left ventricular hypertrophy. An echocardiogram showed a global decrease in left ventricular function, with an ejection fraction of 25%. There was mild left ventricular hypertrophy and mild mitral regurgitation. The chest radiograph showed moderate cardiomegaly with clear lung fields and normal pulmonary vasculature. Significant laboratory findings included a hemoglobin of 10.1 (mean corpuscular volume=90) and an elevated calcium of 11.6. The patient was referred for bilateral heart catheterization and coronary angiography.At angiography, her femoral pulses were diminished. A guidewire could not be passed beyond the thoracic aorta; therefore, angiography was performed with a left brachial approach. The proximal aortic pressure was 160/73 mm Hg, and the femoral arterial pressure measured through a 6F sheath was 61/51 mm Hg. Coronary angiography showed only mild to moderate atherosclerotic changes. The left ventricular pressure was 162/8, 33 mm Hg. An aortogram revealed a severe obstruction in the thoracic aorta beyond the left subclavian artery takeoff (Figure 1). A chest magnetic resonance angiogram showed a normal-size aorta without calcification. There was a 10-cm segment of marked circumferential narrowing of the mid descending thoracic aorta, with a lumen of 8 to 10 mm at its narrowest portion (Figure 2). A CT scan of the abdomen and pelvis showed small to moderate pleural effusions. There was borderline enlargement of the hemiazygous and periaortic nodes. A 3-cm lytic lesion involved the left femoral neck, and numerous small lytic lesions involved the iliac bones and left proximal femur and ischium (Figure 3).The patient underwent successful surgical resection of the thoracic aorta and needle biopsy of the femur. Tissue pathology revealed an epithelioid hemangioendothelioma in both specimens (Figure 4). An echocardiogram performed 5 days after resection of the aortic obstruction showed significant improvement in left ventricular function, with an ejection fraction of 45% to 50%. The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke's Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.Circulation encourages readers to submit cardiovascular images to Dr Hugh A. McAllister, Jr, St Luke's Episcopal Hospital and Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.Download figureDownload PowerPoint Figure 1. Aortogram of descending thoracic aorta demonstrating severe aortic obstruction found at coronary angiography.Download figureDownload PowerPoint Figure 2. Cine magnetic resonance angiogram of thorax demonstrating marked circumferential narrowing of descending thoracic aorta beginning 7 cm distal to origin of left subclavian artery.Download figureDownload PowerPoint Figure 3. CT scan of pelvis showing numerous lytic lesions involving iliac bones, left proximal femur, and ischium. A 3-cm lytic lesion involves left femoral neck (arrow).Download figureDownload PowerPoint Figure 4. Characteristic clusters of atypical epithelioid endothelial cells in a myxoid background. Tumor cells show strong positive staining in immunohistochemical studies with both CD31 and CD34. Biopsies of both aortic mass and bone lesions had similar appearances.FootnotesCorrespondence to Jay H. Traverse, MD, Minneapolis Heart Institute at Abbott Northwestern Hospital, Minneapolis Cardiology Associates, 920 E 28th St, Suite 300, Minneapolis, MN 55407. E-mail [email protected] Previous Back to top Next FiguresReferencesRelatedDetailsCited ByJung R, Janardhan H, Dresser K, Cotton J, Hutchinson L, Mao J and Trivedi C (2021) Response by Jung et al to Letter Regarding Article, "Sustained Activation of Endothelial YAP1 Causes Epithelioid Hemangioendothelioma", Arteriosclerosis, Thrombosis, and Vascular Biology, 41:10, (e493-e495), Online publication date: 1-Oct-2021. Ono M, Kasuga Y, Uehara T and Oda Y (2017) Epithelioid hemangioendothelioma of the thyroid: a case report, Surgical Case Reports, 10.1186/s40792-017-0294-2, 3:1, Online publication date: 1-Dec-2017. Ciliberti M, Caponio R, Pascali A, Matichecchia G and Lioce M (2015) A rare case of intravascular epithelioid hemangioendothelioma of the cephalic vein treated with surgery and postoperative radiation therapy: a case report and review of the literature, Journal of Medical Case Reports, 10.1186/s13256-015-0565-0, 9:1, Online publication date: 1-Dec-2015. Rusthoven C, Liu A, Bui M, Schefter T, Elias A, Lu X and Gonzalez R (2014) Sarcomas of the Aorta: A Systematic Review and Pooled Analysis of Published Reports, Annals of Vascular Surgery, 10.1016/j.avsg.2013.07.012, 28:2, (515-525), Online publication date: 1-Feb-2014. 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Posligua L, Anatelli F, Dehner L and Pfeifer J (2016) Primary Peritoneal Epithelioid Hemangioendothelioma, International Journal of Surgical Pathology, 10.1177/1066896906290682, 14:3, (257-267), Online publication date: 1-Jul-2006. Thalheimer A, Fein M, Geissinger E and Franke S (2004) Intimal angiosarcoma of the aorta: Report of a case and review of the literature, Journal of Vascular Surgery, 10.1016/j.jvs.2004.06.035, 40:3, (548-553), Online publication date: 1-Sep-2004. Chiche L, Mongrédien B, Brocheriou I and Kieffer E (2003) Primary Tumors of the Thoracoabdominal Aorta: Surgical Treatment of 5 Patients and Review of the Literature, Annals of Vascular Surgery, 10.1007/s10016-003-0018-x, 17:4, (354-364), Online publication date: 1-Jul-2003. Charette S, Nehler M, Whitehill T, Gibbs P, Foulk D and Krupski W (2001) Epithelioid hemangioendothelioma of the common femoral vein: Case report and review of the literature, Journal of Vascular Surgery, 10.1067/mva.2001.111993, 33:5, (1100-1103), Online publication date: 1-May-2001. Herrmann H, Chang G, Klugherz B and Mahoney P (2000) Hemodynamic Effects of Sildenafil in Men with Severe Coronary Artery Disease, New England Journal of Medicine, 10.1056/NEJM200006013422201, 342:22, (1622-1626), Online publication date: 1-Jun-2000. August 3, 1999Vol 100, Issue 5 Advertisement Article InformationMetrics Copyright © 1999 by American Heart Associationhttps://doi.org/10.1161/01.CIR.100.5.564 Originally publishedAugust 3, 1999 PDF download Advertisement
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