A mysterious cause of chest pain.
Abdel-Rahman Z, Alkhatib Y, and Dabak V. A mysterious cause of chest pain. J Gen Intern Med 2017; 32(2):S412.
J Gen Intern Med
LEARNING OBJECTIVE #1: Recognize the clinical features of primary cardiac tumors LEARNING OBJECTIVE #2: Manage chest pain by ruling out the most serious and fatal etiologies first CASE: A 42-year-old African American male with a past medical history significant for previously treated non-medullary thyroid carcinoma presented to our hospital with acute onset pleuritic chest pain and dyspnea on exertion. Initial workup revealed a slightly elevated Troponin-I along with a diffuse ST-segment elevation on electrocardiogram. Left sided heart catheterization with coronary angiogram did not show any obstructive lesions. No evidence of pulmonary embolism was seen on computed tomography (CT) angiogram. A transthoracic echocardiogram showed a large right atrial mass, which was further evaluated by a cardiac magnetic resonance imaging (MRI) and found to be involving the right atrial wall and pericardium with evidence of central necrosis on T2 signal. Patient underwent gross total resection of the mass, pathology showed areas of spindle cells and vascular spaces which stained positive for CD31 and CD34 indicating vascular differentiation consistent with angiosarcoma. Staging CT scan of the chest, abdomen and pelvis did not show evidence of distant metastasis. Patient received adjuvant chemotherapy with adriamycin and ifosfamide, postchemotherapy cardiac MRI showed a possible evidence of persistent disease for which he received external beam radiation therapy (EBRT) to the remnant mass. Patient has been stable since then with no evidence of active disease for 2 years. IMPACT: Chest pain is a common presenting complaint in many patients, while the etiology is benign in several occasions, the clinical approach would differ for each particular case depending on several factors which should be incorporated to formulate a specific diagnostic approach and subsequent therapy plan. Younger patients with chest pain should be investigated thoroughly after ruling out deadly cardiac, pulmonary or vascular conditions. DISCUSSION: Primary cardiac tumors are very rare; based upon data from22 large autopsy series the approximate frequency is 0.02%, corresponding to 200 tumors in 1 million autopsies. Malignant tumors constitute around one quarter of these with the majority being sarcomas. Cardiac angiosarcoma typically affects middle aged males and it usually involves the right atrium with pericardial, caval and tricuspid-valve invasion. Metastases are usually found at time of presentation in up to 89% of cases with lungs being the most common site. Presentation is variable depending on the site of involvement. It can be incidentally discovered or might cause symptoms secondary to embolization, direct invasion of adjacent structures or hemodynamic instability due to obstructing cardiac flow. The mainstay of treatment for cardiac tumors remains surgical resection, however chemotherapy and in some occasions radiation therapy are essential adjuvant modalities to achieve cure.