Abstract
Background
Coronary artery perfusion of cardiac tumors is rare but carries diagnostic and therapeutic implications.
Case Summary
A 43-year-old incarcerated male with SDHD gene–associated hereditary paraganglioma-pheochromocytoma syndrome presented with dyspnea and leg edema. Computed tomography and cardiac magnetic resonance imaging revealed a vascular intrapericardial mass in the aortopulmonary window encasing major vessels. Left heart catheterization showed arterial supply from the left anterior descending and right coronary arteries. Given the vascular complexity of the pericardial mass, surgery was deferred. The patient was managed medically and was discharged with plans for I-131 metaiodobenzylguanidine and chemotherapy. Advanced imaging confirmed somatostatin-avid metastases.
Discussion
This case demonstrates the value of multimodal imaging in evaluating coronary-perfused cardiac tumors. It also illustrates the systemic nature of diseases associated with SDHD gene pathology. Delayed follow-up during incarceration highlights a health equity concern, as incarcerated individuals often face barriers to specialty care.
Take-Home Messages
Cardiac tumors with coronary perfusion require advanced imaging. Addressing disparities in vulnerable populations is essential for equitable outcomes.
Key words: cancer, cardiac magnetic resonance, nuclear medicine
Graphical Abstract
History of Presentation
A 43-year-old incarcerated man presented to the hospital with shortness of breath and diffuse lower extremity edema that had been progressively worsening over the previous 4 days. His vital signs were stable on presentation, with a blood pressure of 113/78 mm Hg, heart rate of 72 beats/min, respiratory rate of 16 breaths/min, temperature of 98.7 °F, and oxygen saturation of 100% on room air. Physical examination was notable for a 2/6 systolic murmur on cardiac examination, bilateral lower extremity nonpitting edema, and diffuse end-expiratory wheezes throughout all lung fields on pulmonary examination. Computed tomography (CT) pulmonary angiogram revealed a poorly characterized soft tissue mass seen splaying and separating the aorta and pulmonary artery. A right lower lung opacity concerning for malignant effusion was also seen (Figure 1).
Take-Home Messages
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Cardiac paragangliomas with coronary artery perfusion are rare and require multimodal imaging, including cardiac magnetic resonance imaging and coronary angiography, for accurate diagnosis, risk stratification, and treatment planning.
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Delays in follow-up during incarceration highlight a critical health equity concern, emphasizing the need to improve access to timely specialty care in vulnerable populations.
Figure 1.
Computed Tomography Pulmonary Angiograms
Soft tissue mass (yellow arrow) separating the aorta and pulmonary artery as seen on (Left) coronal view and (Right) axial view.
Past Medical History
At 8 years of age, the patient frequently had severe migraines and constantly felt hot and sweaty. He also struggled to gain weight as he aged. At that time, he was found to have bilateral pheochromocytomas on his adrenal glands and subsequently underwent resection, which brought resolution to his symptoms.
In 2019, he noticed that his voice would become sore while singing in the choir. During that time, he also had episodes of dizziness associated with sweating and syncope. He was evaluated for these issues in 2021, when he was diagnosed with paragangliomas in his carotid body and vocal cords. It was then that an intrapericardial mass was seen on CT thorax during the work-up. The paragangliomas in the neck were treated with chemotherapy and radiation, with his last radiation therapy session in April 2022. Surgeons elected not to operate on the intrapericardial mass at that time, as surgery was deemed an unnecessary risk given the patient had a good response to radiation therapy. Genetic testing revealed that the patient had SDHD gene–associated hereditary paraganglioma-pheochromocytoma syndrome, which explained the occurrence of the tumors.
In 2023, the patient reported a return of weight loss, as well as feeling hot and experiencing syncopal episodes. He was incarcerated at the time and was not evaluated for these symptoms until October 2024, when he was diagnosed with a left pheochromocytoma. This was resected along with the left adrenal gland. A metaiodobenzylguanidine (MIBG) scan during this visit confirmed a 7 × 6 × 5 cm mediastinal mass.
Differential Diagnoses
Differential diagnoses for a mediastinal mass are wide, and they depend on the anatomical compartment in which the mass is located. Anterior compartment masses can be thymic lesions, lymphomas, germ cell tumors, and thyroid lesions. Middle compartment masses can be lymphadenopathy, foregut cysts, or vascular lesions. Lastly, posterior compartment masses can be neurogenic tumors, spinal lesions, or esophageal lesions.
Investigations
After the CT thorax revealed the mediastinal mass, cardiac magnetic resonance imaging was obtained for further characterization of the lesion. The scans showed a stable, solid, vascular, intrapericardial mass in the aortopulmonary window at the level of the sinotubular junction anteriorly and to the left of the aorta, above the left main coronary artery. It measured 8.3 cm anteriorly to posteriorly, 6.5 cm in width, and 5.4 cm inferiorly to superiorly. The mass also encircled the proximal ascending aorta and right pulmonary artery (Figure 2).
Figure 2.
Cardiac Magnetic Resonance Imaging
Axial-view images with T2-weighted hyperintensity revealing an intrapericardial mass (yellow arrows) encircling the aorta and pulmonary artery.
These findings prompted a consultation with the cardiothoracic surgery team, who recommended a left heart catheterization to further investigate the lesion's anatomy for possible surgical intervention. The left heart catheterization showed the left anterior descending and right coronary arteries both supplying the cardiac mass through feeding vessels and collaterals (Figure 3).
Figure 3.
Angiograms During Left Heart Catheterization
(Left) Right coronary artery and collaterals supplying the cardiac mass (yellow circle). (Right) Left coronary artery and collaterals supplying the cardiac mass (yellow circle).
Management
The patient's edema and shortness of breath were successfully treated with intravenous furosemide and nebulized ipratropium bromide/albuterol. Owing to the complexity of the pericardial mass, we decided to defer surgery and pursue medical management. The patient was evaluated by the endocrinology and oncology teams and was discharged with plans for therapeutic I-131 MIBG and subsequent chemotherapy.
Outcome and Follow-Up
One month after discharge, a positron emission tomography (PET)/CT Ga-68 dotatate scan was performed. The PET scan showed intense somatostatin overexpression in bilateral carotid body paragangliomas and in extensive mediastinal metastasis. Intense uptake was also seen in the head of the pancreas. These findings were consistent with metastatic disease of the known primary neuroendocrine tumor (Figure 4).
Figure 4.
Positron Emission Tomography Scans
(Top Left) Coronal and (Bottom Left) axial views showing intense somatostatin overexpression indicating mediastinal metastasis (yellow arrows). Intense uptake also seen in the head of the pancreas is suspected adenopathy (blue arrow). (Top Right) Coronal and (Bottom Right) axial views showing intense somatostatin overexpression in the bilateral carotid body paragangliomas (green circles).
Discussion
Cardiac paragangliomas are a rare neuroendocrine tumor that arise from paraganglionic cells adjacent to the great arteries, coronary arteries, or the atria. Common locations for cardiac paragangliomas, in order of frequency, are the left atria, interatrial septum, and the anterior surface of the heart.1 The definitive treatment for these tumors is surgical resection, however given their highly vascular nature, surgery is very complex and not always feasible. Additionally, these tumors can be secretory, and therefore if surgical resection is pursued, then medical optimization with alpha blockade followed by beta blockade must be done to avoid hemodynamic changes during surgery.2
Owing to the coronary artery involvement of these tumors, evaluation necessitates the use of multimodal imaging for proper characterization. Once the cardiac mass was identified incidentally on CT of the thorax, we decided to proceed with cardiac magnetic resonance imaging, which allowed us to assess the morphology, extension, and homogeneity of the mass, as well as its histopathological characterization, particularly the vascularity. Cardiac paragangliomas appear as a high-intensity signal on T2-weighted images.3,4 Left heart catheterization can further identify a cardiac tumor's blood supply and hemodynamic effects.5 Results from left heart catheterization in this patient confirmed coronary perfusion, which ultimately led to the decision to defer surgery given the high potential for intraoperative complications. Cardiac paragangliomas are expected to be positive on PET imaging, as it was in this case during follow-up imaging. Although the tumor was not well visualized on the transthoracic echocardiography obtained on admission, cardiac paragangliomas appear as echogenic masses with a broad base on ultrasound.4
This patient's cardiac tumor was due to SDHD gene–associated hereditary paraganglioma-pheochromocytoma syndrome. SDHD is a tumor suppressor gene responsible for producing an anchor for the succinate dehydrogenase enzyme complex to the inner mitochondrial membrane. Those with an SDHD mutation have an overall higher penetrance for symptomatic tumor development. The mechanism by which this mutation leads to tumor development is not yet understood.6
This case also highlights an important health equity issue: the impact of incarceration on continuity of care. During his incarceration, the patient experienced a delay in follow-up despite symptoms consistent with tumor recurrence. Individuals in correctional settings often face systemic barriers to timely specialty care, advanced imaging, and longitudinal surveillance, which can lead to delayed diagnosis and suboptimal outcomes.7,8 This case underscores the need for improved coordination between correctional health systems and tertiary care centers to ensure equitable access to high-quality care.
Conclusions
This case reinforces the importance of identifying and characterizing vascularized cardiac tumors. The patient's presentation illustrates the diagnostic and therapeutic complexities posed by such tumors, highlighting the utility of multimodal imaging in these cases. Although surgical intervention was deferred because of the complexity of this condition, a multidisciplinary approach incorporating advanced imaging, targeted therapies, and long-term oncological follow-up remains critical for optimizing outcomes. This case also highlights the need for improved health equity among incarcerated patients.
Funding Support and Author Disclosures
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Footnotes
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the Author Center.
References
- 1.Mandak J.S., Benoit C.H., Starkey R.H., Nassef L.A. Echocardiography in the evaluation of cardiac pheochromocytoma. Am Heart J. 1996;132(5):1063–1066. doi: 10.1016/s0002-8703(96)90027-7. [DOI] [PubMed] [Google Scholar]
- 2.Brown M.L., Zayas G.E., Abel M.D., Young W.F., Jr., Schaff H.V. Mediastinal paragangliomas: the mayo clinic experience. Ann Thorac Surg. 2008;86(3):946–951. doi: 10.1016/j.athoracsur.2008.04.105. [DOI] [PubMed] [Google Scholar]
- 3.Beroukhim R.S., Prakash A., Buechel E.R., et al. Characterization of cardiac tumors in children by cardiovascular magnetic resonance imaging: a multicenter experience. J Am Coll Cardiol. 2011;58(10):1044–1054. doi: 10.1016/j.jacc.2011.05.027. [DOI] [PubMed] [Google Scholar]
- 4.Araoz P.A., Mulvagh S.L., Tazelaar H.D., Julsrud P.R., Breen J.F. CT and MR imaging of benign primary cardiac neoplasms with echocardiographic correlation. Radiographics. 2000;20:1303–1309. doi: 10.1148/radiographics.20.5.g00se121303. [DOI] [PubMed] [Google Scholar]
- 5.Gurav A., Revaiah P.C., Tsai T.Y., et al. Coronary angiography: a review of the state of the art and the evolution of angiography in cardio therapeutics. Front Cardiovasc Med. 2024;11 doi: 10.3389/fcvm.2024.1468888. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 6.Andrews K.A., Ascher D.B., Pires D.E.V., et al. Tumour risks and genotype-phenotype correlations associated with germline variants in succinate dehydrogenase subunit genes SDHB, SDHC and SDHD. J Med Genet. 2018;55(6):384–394. doi: 10.1136/jmedgenet-2017-105127. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 7.Wilper A.P., Woolhandler S., Boyd J.W., et al. The health and health care of US prisoners: results of a nationwide survey. Am J Public Health. 2009;99(4):666–672. doi: 10.2105/AJPH.2008.144279. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 8.Maruschak L.M., Berzofsky M., Unangst J. U.S. Department of Justice, Bureau of Justice Statistics; 2015. Medical Problems of State and Federal Prisoners and Jail Inmates, 2011–12.https://bjs.ojp.gov/content/pub/pdf/mpsfpji1112.pdf NCJ 248491. [Google Scholar]