Autopsy and Case Reports
Autopsy and Case Reports
Article / Clinical Case Report

Cardiac angiosarcoma: an unexpected diagnosis

Milton Roberto Furst Crenitte; Mariana Domingues Galvão; Fabíola Del Carlo Bernardi; Leonardo Gomes da Fonseca

Downloads: 6
Views: 647


Cardiac angiosarcoma is a rare entity. The incidence through autopsy findings ranges between 0.001% and 0.03%. The disease usually presents with non-specific symptoms, although asymptomatic cases are frequent; therefore, diagnosis is unexpected and consequently delayed. The authors report the case of a middle-aged man with a recent onset cough and dyspnea. He sought medical care several times without receiving a definite diagnosis until a plain chest radiography was taken showing a mediastinal enlargement, which was the reason why he was hospitalized for clinical investigation. During the diagnostic workup, an echodopplercardiogram and a thoracic computed tomography were performed, showing a heterogeneous soft-tissue mass infiltrating the pericardium and the anterior atrial wall. Multiple and scattered pulmonary nodules were also present. A pulmonary nodule was biopsied, which revealed an angiosarcoma. The clinical features added to the radiological and histological findings permitted the diagnosis of right atrial angiosarcoma. The authors highlight the unexpected pattern in the presentation of cardiac tumors.


Hemangiosarcoma, Heart Neoplasms, Incidental Findings


Centofanti P, Di Rosa E, Deorsola Let al. Primary cardiac tumors: early and late results of surgical treatment in 91 patients. Ann Thorac Surg. 1999;68(4):1236-41. []. PMid: [PMID:10543485]

Strecker T, Rösch J, Weyand M, Agaimy A. Primary and metastatic cardiac tumors: imaging characteristics, surgical treatment, and histopathological spectrum: a 10-year-experience at a German heart center. Cardiovasc Pathol. 2012;21(5):436-43. []. PMid: [PMID:22300501]

Reynen K. Frequency of primary tumors of the heart. Am J Cardiol. 1996;77(1):107. []. PMid: [PMID:8540447]

Shapiro LM. Cardiac tumours: diagnosis and management. Heart. 2001;85(2):218-22. []. PMid: [PMID:11156679]

Burke AP, Tazelaar H, Butany JW, et al. Cardiac sarcomas. In: Travis W, Brambilla E, Muller-Hermelink HK, Harris CC, editors. Pathology and genetics of tumours of the lung, thymus, pleura and heart. Lyon: ARC Press; 2004. p. 273-81.

Burke AP, Tavora F. Sarcomas of the heart and great vessels. In: Burke AP, Tavora F. Practical cardiovascular pathology. Philadelphia: Lippincott Williams & Wilkins; 2010. p. 398-406.

Hoffmeier A, Sindermann JR, Scheld HH, Martens S. Cardiac tumors—diagnosis and surgical treatment. Dtsch Arztebl Int. 2014;111(12):205-11. PMid: [PMID:24717305].

Neragi-Miandoab S, Kim J, Vlahakes GJ. Malignant tumours of the heart: a review of tumour type, diagnosis and therapy. Clin Oncol (R Coll Radiol). 2007;19(10):748-56. []. PMid: [PMID:17693068]

Strotmann J. Cardiac tumors—clinical symptoms, diagnostic approaches, and therapeutic aspects. Med Klin (Munich). 2008;103(3):175-80. PMid: [PMID:18344068]

Antonuzzo L, Rotella V, Mazzoni Fet al. Primary cardiac angiosarcoma: a fatal disease. Case Rep Med. 2009;2009:1-4." xmlns:xlink="" target="_blank" title="">">"> []. PMid:19724650

Bellitti R, Buonocore M, De Rosa N, Covino FE, Casale B, Santè P. Primary cardiac angiosarcoma in a 25-year-old man: excision, adjuvant chemotherapy, and multikinase inhibitor therapy. Tex Heart Inst J. 2013;40(2):186-8. PMid: [PMID:23678219].

Glancy DL, Morales JB Jr, Roberts WC. Angiosarcoma of the heart. Am J Cardiol. 1968;21(3):413-9. []. PMid: [PMID:4230097]

Hoffmeier A, Schmid C, Deiters Set al. Neoplastic heart disease — the Muenster experience with 108 patients. Thorac Cardiovasc Surg. 2005;53(1):1-8. []. PMid: [PMID:15692911]

Alam M, Rosman HS, Grullon C. Transesophageal echocardiography in evaluation of atrial masses. Angiology. 1995;46(2):123-8. []. PMid: [PMID:7702196]

Riles E, Gupta S, Wang DD, Tobin K. Primary cardiac angiosarcoma: A diagnostic challenge in a young man with recurrent pericardial effusions. Exp Clin Cardiol. 2012;17(1):39-42. PMid: [PMID:23204900].

Araoz PA, Eklund HE, Welch TJ, Breen JF. CT and MR imaging of primary cardiac malignancies. Radiographics. 1999;19(6):1421-34. []. PMid: [PMID:10555666]

Iglézias JCR, Velloso LGC, Dallan LA, Benvenuti LA, Verginelli G, Stolf NAG. Angiossarcoma de átrio direito. Rev Bras Cir Cardiovasc. 2000;15(2):186-91. [].

Janigan DT, Husain A, Robinson NA. Cardiac angiosarcomas. A review and a case report. Cancer. 1986;57(4):852-9. [<852::AID-CNCR2820570428>3.0.CO;2-S]. PMid: [PMID:3510706]

O’Donnell DH, Abbara S, Chaithiraphan Vet al. Cardiac tumors: optimal cardiac MR sequences and spectrum of imaging appearances. AJR Am J Roentgenol. 2009;193(2):377-87. []. PMid: [PMID:19620434]

Sibille L, Ilonca D, Oziol Eet al. FDG PET/CT in aortic angiosarcoma. Clin Nucl Med. 2010;35(2):134-7. []. PMid: [PMID:20090470]

Barreiro M, Renilla A, Jimenez JMet al. Primary cardiac tumors: 32 years of experience from a Spanish tertiary surgical center. Cardiovasc Pathol. 2013;22(6):424-7. []. PMid: [PMID:23727543]

Silver MD, Gotlieb AI, Schoen FJ. Cardiovascular pathology. 3rd ed. Philadelphia: Churchill Livingstone; 2001. p. 399-598.

Llombart-Cussac A, Pivot X, Contesso Get al. Adjuvant chemotherapy for primary cardiac sarcomas: the IGR experience. Br J Cancer. 1998;78(12):1624-8. []. PMid: [PMID:9862574]

Nakamichi T, Fukuda T, Suzuki T, Kaneko T, Morikawa Y. Primary cardiac angiosarcoma: 53 months’ survival after multidisciplinary therapy. Ann Thorac Surg. 1997;63(4):1160-1. []. PMid: [PMID:9124928]

Kakizaki S, Takagi H, Hosaka Y. Cardiac angiosarcoma responding to multidisciplinary treatment. Int J Cardiol. 1997;62(3):273-5. []. PMid: [PMID:9476688]

Baay P, Karwande SV, Kushner JP, Olsen S, Renlund DG. Successful treatment of a cardiac angiosarcoma with combined modality therapy. J Heart Lung Transplant. 1994;13(5):923-5. PMid: [PMID:7803440].

Überfuhr P, Meiser B, Fuchs Aet al. Heart transplantation: an approach to treating primary cardiac sarcoma? J Heart Lung Transplant. 2002;21(10):1135-9. []. PMid: [PMID:12398881]

Look Hong NJ, Pandalai PK, Hornick JLet al. Cardiac angiosarcoma management and outcomes: 20-year single-institution experience. Ann Surg Oncol. 2012;19(8):2707-15. []. PMid: [PMID:22476752]

Fayette J, Martin E, Piperno-Neumann Set al. Angiosarcomas, a heterogeneous group of sarcomas with specific behavior depending on primary site: a retrospective study of 161 cases. Ann Oncol. 2007;18(12):2030-6. []. PMid: [PMID:17974557]

Meis-Kindblom JM, Kindblom LG. Angiosarcoma of soft tissue: a study of 80 cases. Am J Surg Pathol. 1998;22(6):683-97. []. PMid: [PMID:9630175]

Fata F, O’Reilly E, Ilson Det al. Paclitaxel in the treatment of patients with angiosarcoma of the scalp or face. Cancer. 1999;86(10):2034-7. [<2034::AID-CNCR21>3.0.CO;2-P]. PMid: [PMID:10570428]

Penel N, Marréaud S, Robin YM, Hohenberger P. Angiosarcoma: state of the art and perspectives. Crit Rev Oncol Hematol. 2011;80(2):257-63. []. PMid: [PMID:21055965]


Publication date:

569696b564cc8b5cda711c02 autopsy Articles

Autops Case Rep

Share this page
Page Sections