key: cord-0992387-gmxut0eo
authors: Jex, Nicholas; Farley, Jonathan; Thirunavukarasu, Sharmaine; Chowdhary, Amrit; Sengupta, Anshuman; Greenwood, John; Schlosshan, Dominik; Plein, Sven; Levelt, Eylem
title: A 30-Year-Old Man With Primary Cardiac Angiosarcoma
date: 2021-05-12
journal: JACC Case Rep
DOI: 10.1016/j.jaccas.2021.03.009
sha: 8944b03bb52ea15a52ad92a6ec25d9e9a3f9f057
doc_id: 992387
cord_uid: gmxut0eo

A previously fit and well 30-year-old man presented with palpitations, fever, and pleuritic chest pain. Multimodality imaging and histopathology confirmed the diagnosis of primary cardiac angiosarcoma. We present the details of the presentation, diagnostic process using multimodality imaging, and clinical management. (Level of Difficulty: Beginner.)

acute reduction in his exercise tolerance.

On examination, a resting tachycardia with heart rate of 110 beats/min and low-grade pyrexia of 37.8 C were detected. Oxygen saturations were 97% on room air, and blood pressure was normal. There were no signs of congestive cardiac failure or audible murmurs on pre-cordial auscultation.

The medical history was unremarkable.

Given the history of pleuritic chest pain and fever and the timing of this presentation during the COVID-19 pandemic, coronavirus infection was the primary differential diagnosis. Venous pulmonary embolism and bacterial or other viral causes of pneumonia were also considered. Blood tests including D-dimer, electrocardiogram, and plain film chest x-ray film were ordered.

To understand the 2 distinct clinical presentations of cardiac angiosarcoma and how these relate to the underlying morphological features. To review the diagnostic features of angiosarcoma on cardiovascular magnetic resonance imaging. To revisit therapeutic options and the importance of multidisciplinary care in this rare condition.

Full blood count showed a normocytic anemia.

Although C-reactive protein (158 mg/l) and D-dimer (840 ng/ml) levels were raised, troponin I level 

Because of the presence of widespread metastatic disease and the extent of regional spread and size of The prognosis of angiosarcoma is poor, with a median survival of 14 months, reducing to 6 months in metastatic disease (3) . Palliative treatment with surgical debulking and chemoradiotherapy may offer some prognostic benefit (4). Anthracyclines, ifosfamide, and taxanes are the most commonly used agents (5) . Immunotherapy with recombinant interleukin 2 has been used with some prognostic benefit (6).

In our case, the multidisciplinary team decision was for surgical debulking to provide the best option for palliative care, given the degree of RA cavity obstruction, and to allow time for chemotherapy administration.

Because of the rare incidence of cardiac angiosarcomas, there is a dearth of evidenced-based guidelines, and currently, no standardized treatment algorithm for cardiac angiosarcoma exists (3). 

MR imaging of cardiac tumors and masses: a review of methods and clinical applications

Primary cardiac angiosarcoma-a review

Primary cardiac sarcoma: 25-year Cleveland Clinic experience

Integrated approach for cardiac angiosarcoma

Primary cardiac angiosarcoma: a fatal disease

The authors have reported that they have no relationships relevant to the contents of this paper to disclose. 

KEY WORDS cardiac angiosarcoma, cardiac magnetic resonance, echocardiography, malignancy, imaging, positron emission tomography APPENDIX For supplemental videos, please see the online version of this paper.