Prisoner of the Heart - Primary Unresectable Cardiac Angiosarcoma in an African American Man

Document Type

Conference Proceeding

Publication Date

10-1-2021

Publication Title

Cancer Clin Trials

Abstract

Background: One-quarter of cardiac tumors are malignant [Centofanti P, et al Ann Thorac Surg 1999] and present with metastasis [Patel S, et al Med Sci Mon 2014]. The most common malignant primary cardiac tumor is sarcoma, of which 30% is angiosarcoma [Patel S, et al Med Sci Mon 2014]. From all histologic subtypes, primary cardiac angiosarcoma (PCA) has the statistically lowest medial overall survival of 7 months with no associated improvement over the last decade [Yin K, et al J thorac cardiovasc Surg 2019]. Given this, the outcomes of interventions are controversial. Eleven percent of PCAs are found in African- Americans [Yin K, et al J thorac cardiovasc Surg 2019]. We contribute a report of unresectable PCA in an African-American imprisoned man who faced numerous obstacles, resulting in partial completion of radiotherapy. Objectives: • Cardiac angiosarcoma has a higher incidence within the White population. • The presenting symptoms include dyspnea and hemorrhagic pericardial effusion. • Delay in diagnosis prolongs hemodynamic instability and patient discomfort. • This case offers value for future male African American epidemiologic studies. • There are no radiotherapy guidelines in treating patients diagnosed with any cardiac sarcoma • Radiation therapy provided symptom free in a metastatic setting • The COVID-19 pandemic complicates disposition, especially relating to prisons. Methods: Case Description A 42-year-old imprisoned African-American man with no relevant history presented to an outside hospital with chest pain, dyspnea, tachycardia, muffled heart sounds and jugular venous distention without hypotension secondary to a large hemorrhagic pericardial effusion with tamponade. The subxiphoid approach pericardiocentesis was unsuccessful. The subsequent pericardial window via left mini thoracotomy with pericardial biopsy removed 1500 cc of bloody pericardial effusion status post chest tube placement, resulting in symptomatic improvement. Transthoracic echocardiogram (TTE) revealed 62% ejection fraction, pericardial effusion and hyperdynamic biventricular systolic functions with right ventricular diastolic collapse. The pericardial biopsy showed poorly differentiated malignant epithelial neoplasm. Computed tomography (CT) chest showed a 6 x 5.8 x 8 cm mass of the right heart border extending into both right atrial and ventricular lumens with abutment into the superior vena cava, lymphadenopathy, and 14 mm pulmonary right lower lobe nodule suspicious for metastasis (Fig. 1). The patient was transferred to our hospital. Repeat CT chest revealed an interval decrease in pericardial effusion and new left pleural effusion, and Pleur-x catheter was placed. Increased dyspnea prompted CTPE, which was negative, with repeat TTE showing reaccumulation of pericardial effusion. CT abdomen pelvis was negative for metastasis. Cardiac MRI revealed a 7 x 7.8 cm mass originating from the atrioventricular groove extending into the left atrium, the lateral atrial recess, the right atrial posterior and lateral free walls, and the pericardium with right coronary artery encasement. Another 2.5 x 3 cm heterogeneous mass was found involving the right superior and inferior pulmonary veins. Cardiac biopsy pathology revealed positivity for ERG and CD31 stains (Fig. 2), consistent with angiosarcoma. His course was complicated by an unstable arrhythmia and a new 1400 cc hemorrhagic effusion requiring thoracentesis. TTE demonstrated interval decrease to 4.9 x 4.1 cm after six of the 30 fractions prescribed to 60 Gray (Gy). The prevalence of COVID-19 in both our hospital and Michigan prisons complicated disposition. Thus, he enrolled in hospice after 12 fractions of radiotherapy and expired in jail 5 months from initial symptom onset. Results: N/A Conclusions: The rarity of primary cardiac tumors [Reynen K, Am J Cardol 1996] makes randomized trials investigating outcomes difficult. An evaluation of primary cardiac sarcomas within the United States, not exclusively PCA, using the SEER database found 24% of patients received radio herapy with no association to better outcomes [Yin K, et al J thorac cardiovasc Surg 2019]. It is unclear what percentage of patients with the angiosarcoma histology (43% of their study population) received radiotherapy. Additionally, the database did not include the timing of interventions. Our patient's tumor decreased by half after receiving a total of 12 Gy with moderate symptom relief. There was no additional interval imaging to trend tumor size after receiving 24 Gy. Currently, there are no radiotherapy guidelines in treating patients diagnosed with any cardiac sarcoma. The risk of contracting COVID-19 while admitted was high. Thus, he only received 24 Gy, enrolled into hospice and transferred back to his jail hospital and expired. Did the partial radiation provide benefit? The radiotherapy he did receive had no effect on his overall survival given he expired within the expected timeframe, however he did not complete his prescription. Isambert et al found that radiotherapy was associated with improved progression-free survival, retrospectively [Isambert N, et al Journal of General Internal Medicine 2017] . However, our patient is not wellrepresented in their study cohort encompassing all histologies and only patients with non-metastatic disease received radiotherapy. For the entire cohort, about 19% received radiotherapy as monotherapy. The mean age of PCA diagnosis is 47.2 years old [Yin K, et al J thorac cardiovasc Surg 2019], which aligns closely to that of our patient's. PCAs arise in the right atrium [6] causing cardiac encasement, pericardial involvement, pericardial effusion, and cardiac tamponade [Reynen K, Am J Cardol 1996]. The most common site of metastasis is the lungs [Patel S, et al Med Sci Mon 2014, Abdel-Rahman Z et al Journal of General Internal Medicine 2017], as most likely seen here. All of the above factors made achieving disease control or patient comfort a challenge. PCA must be in the differential for pericardial effusion. The need to further evaluate the role of radiotherapy is greatly needed. Given the rarity of PCA, it is unlikely that clinical trials will investigate the role of radiotherapy or other interventions. This report chronicles interval tumor size decrease and symptom relief. Lastly, more investigations on PCA in African-American men [Galven D, et al Oncoscience 2018] are needed. (Figure Presented).

Volume

44

Issue

10

First Page

S128

Last Page

S130

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