Case Report: Primary Cardiac Angiosarcoma
- 1. Hospital Universitario de Toledo, Spain
CITATION
Pigorini AG, Boillot C, González BS, Valbuena FP, Romero LMH, et al. (2024) Case Report: Primary Cardiac Angiosarcoma. JSM Clin Case Rep 12(3): 1245.
CASE PRESENTATION
We present the case of a 55-year-old woman with a relevant medical history of migraines who was referred for evaluation from the emergency room. Upon our arrival, the patient was in poor general condition, hypotensive (70/40 mmHg), and in sinus tachycardia at 110 bpm. The patient reported precordial pain that had started the previous night, without any infectious symptoms in the preceding weeks. An emergent bedside echocardiogram was performed, revealing severe circumferential pericardial effusion with echocardiographic signs of tamponade. Intensive fluid therapy was initiated in the critical care unit, along with a norepinephrine infusion, and the patient was emergently transferred to the operating room for pericardiocentesis [1]. During the procedure, 250 cc of clear fluid was obtained, which had negative cytology for malignant cells and exudative characteristics according to Light’s criteria.
The patient was later admitted in stable condition to the cardiology unit for further evaluation. A few days later, a scheduled transthoracic echocardiogram revealed a heterogeneous echodense image in the basal portion of both atria, extending to the coronary sinus and into the right atrial cavity at the level of its opening. Given these findings, a cardiac magnetic resonance imaging was performed, showing a large heterogeneous mass (appearing vascularized) located in the left atrioventricular (AV) groove, extending along the posterior wall of the left atrium, infiltrating the inferior and inferolateral basal segment of the left ventricle, and the coronary sinus, becoming intracavitary in the right atrium. Additionally, multiple pulmonary nodules were observed, likely indicating metastases [2].
Subsequent body computerized tomography (CT) confirmed the presence of a cardiac mass centered in the left AV groove and coronary sinus, measuring approximately 51 x 38 x 30 mm (APxTxCC), with irregular and multilobulated margins, suggesting a primary cardiac mass with multiple bilateral pulmonary metastases. The study did not show intracranial metastases. Additionally, the patient was diagnosed with acute bilateral pulmonary embolism, with pulmonary infarction in the lower right lobe. PET-CT indicated that the cardiac mass was suggestive of malignancy, confirming pulmonary and bone metastases at the humerus level [3].
A CT-guided biopsy was scheduled to obtain a histological sample which confirmed the presence of an angiosarcoma (Figures 1-3).
Figure 1: Magnetic resonance imaging showing left atrial mass
Figure 2: Computed tomography showing atrial mass
Figure 3: Positron emission tomography (PET-TC) showing atrial, pulmonary nodules and humerus activity.
DISCUSSION
Primary cardiac tumors are rare and are more often benign. On the other hand, angiosarcoma is the most frequent malignant tumor of the heart and has a very poor prognosis because of infiltration of different structures of the heart and surrounding structures. The initial diagnosis is usually made with echocardiography and confirmed with other techniques such as RMC, cardiac CT and PET-TC.
There is currently no standard treatment approach. When localized, surgical resection leads to the best chance of longterm survival. However this technique can be challenging and in some patients, the rapidly progressive and aggressive nature of this tumor can contraindicate the surgery. Several case reports and case series report treatment with adjuvant radiotherapy and neoadjuvant or adjuvant chemotherapy.
