Case report
Excision of a cardiac paraganglioma
We present a case report detailing the surgical management of a cardiac paraganglioma in a 57-year-old man with a succinate dehydrogenase subunit B mutation and family history of paragangliomas. These neuroendocrine tumours arise from chromaffin cells and account for less than 3% of cardiac tumours. Succinate dehydrogenase subunit B mutations are associated with aggressive disease patterns and require a multidisciplinary approach for optimal management. The patient had multifocal disease involving the neck, abdomen and heart, yet was asymptomatic aside from a palpable neck mass. Positron emission tomography with fluorodeoxyglucose, echocardiography and coronary angiography identified a mass within the right atrial wall and concurrent significant narrowing of the left anterior descending coronary artery. The video tutorial demonstrates excision of the cardiac paraganglioma via median sternotomy, establishment of cardiopulmonary bypass, and right atrial exploration. No tumour was visualized within the chamber, and further inspection revealed a firm lesion in the right atrioventricular groove, infiltrating the myocardium and encasing the right coronary artery. The artery was transected and bypassed using a saphenous vein graft, allowing for complete tumour resection. A graft to the left anterior descending artery was also performed. Postoperative recovery was uncomplicated, and histopathology confirmed a cardiac paraganglioma with clear margins.
Paraganglioma (PG) is a neuroendocrine tumour arising from chromaffin cells [1] and can be found in various anatomical locations. The majority of PGs (60–70%) occur in the head and neck region, particularly in the carotid body and jugulotympanic areas, while thoracic PG accounts for approximately 5–7% of cases. Intracardiac PG is exceedingly rare, accounting for 0.6–2.5% of all PGs [6, 7], and approximately 1–3% of all cardiac and pericardial neoplasms in imaging and autopsy series [2, 3].
Succinate dehydrogenase subunit A (SDHA), SDHB, SDHC and SDHD genes encode the four subunits of SDH, a crucial mitochondrial enzyme that functions in both the Krebs cycle and the electron transport chain [1]. Mutations resulting in loss of SDH function have been identified as a key cause of hereditary PGs and pheochromocytomas, highlighting the enzyme’s role in tumour suppression [4]. Among these, SDHB mutations are particularly associated with a higher risk of malignancy, and PGs linked to this mutation are more likely to be extra-adrenal, exhibiting aggressive behaviour [4].
In this video tutorial we present a case report featuring a 57-year-old man with cardiac PG.
A 57-year-old man was referred to cardiac surgery after being diagnosed with the tumour in the right atrium. His past medical history included hypertension, hypercholesterolaemia, obstructive sleep apnoea, asthma and obesity. A strong family history of PGs was noted: his brother had a large abdominal PG and tested positive for the SDHB mutation, while his nephew had multiple PGs with complex abdominal complications.
Genetic testing confirmed the SDHB mutation in the patient while further imaging revealed multiple PGs in the cardiac, cervical and abdominal regions. Despite negative plasma metanephrines, his only symptom was a palpable right-sided neck mass. Transthoracic echocardiography (TTE) identified a tumour of the right atrium adjacent to the tricuspid valve, and coronary angiography showed severe left anterior descending (LAD) coronary artery stenosis and moderate right coronary artery (RCA) disease. The patient was scheduled for excision of the cardiac tumour and simultaneous coronary artery bypass grafting (CABG).
1 - Introduction and background (0:13)
PGs are rare neuroendocrine tumours arising from chromaffin tissue. While most occur in the head and neck, cardiac involvement is exceptionally rare, constituting less than 3% of all cardiac tumours. These tumours are often driven by mutations in the SDH complex, particularly SDHB, which is associated with aggressive and hereditary forms. Early identification and a multidisciplinary approach are vital, as these tumours are often asymptomatic and discovered incidentally.
2 - Patient presentation and diagnosis (1:16)
A 57-year-old man with a known family history of PG was referred after imaging revealed a right-sided cardiac mass. Although he harboured tumours in the neck, heart and abdomen, his only symptom was a palpable neck lesion. He had multiple comorbidities, including hypertension and obesity. Genetic testing confirmed an SDHB mutation, and interestingly, plasma and urinary metanephrines were within normal limits—emphasizing the non-secretory nature of many cardiac PGs.
3 - Imaging (2:06)
Multimodal imaging was essential for localization and surgical planning. Positron emission tomography with fluorodeoxyglucose (FDG-PET)/computed tomography (CT) revealed a hypermetabolic intracardiac mass along the right atrial wall with intense FDG uptake. TTE showed a well-defined, echogenic lesion without evidence of flow obstruction. However, due to poor acoustic windows on TTE, transoesophageal echocardiography (TOE) was performed, which more clearly demonstrated a 1.8 × 2.5 cm mass abutting the anterior tricuspid valve leaflet, with trivial regurgitation. Coronary angiography revealed severe LAD artery stenosis and a moderate RCA stenosis, with a clear vascularized mass at the distal portion, associated with the post right atrial wall. Retrograde filling of the posterior descending artery was visualized, suggesting a tumour-associated altered coronary haemodynamic.
4 - Surgical procedure (5:02)
A median sternotomy was performed, and cardiopulmonary bypass was established with central aortic and bicaval cannulation. The heart was arrested using antegrade cardioplegia. Upon opening the right atrium, no tumour was seen intraluminally, confirming an intramural location.
5 - Tumour localization (5:26)
Careful inspection of the external surface of the heart revealed a firm, well-circumscribed mass in the right atrioventricular groove. The lesion infiltrated both the right atrial and right ventricular walls (Figure 1), confirming its intramural nature.
Figure 1: Intramural tumour identified in the atrioventricular groove involving the right atrium and right ventricle.
6 - RCA transection (6:35)
The tumour was found to be tightly adherent to the mid-portion of the RCA. To safely mobilize the lesion without compromising coronary perfusion, the RCA was transected proximally and distally (Figure 2). This manoeuvre allowed complete circumferential dissection of the mass and prevented inadequate oncological resection. A saphenous vein graft was then anastomosed from the aorta to the distal RCA to restore flow.
Figure 2: Tumour encompassing the RCA.
7 - Tumour resection and operative conclusion (8:01)
The tumour was carefully excised from the infiltrated myocardium, with attention to maintaining clear surgical planes and minimizing injury to adjacent structures. The mass measured approximately 1.8 × 2.5 cm in its greatest dimensions (Figure 3). The resection bed was irrigated and inspected to ensure complete excision and haemostasis. The tricuspid valve was thoroughly assessed and found to be structurally and functionally intact, with no evidence of leaflet distortion or regurgitation. The right atrium was closed in standard fashion using a two-layer running suture technique. To address concomitant LAD stenosis, a left internal mammary artery (LIMA)-to-LAD bypass graft was completed. The patient was weaned from bypass without inotropic support.
Figure 3: Intramural tumour completely excised with clear surgical margins.
8 - Postoperative outcomes and follow-up (8:43)
Postoperative recovery was uneventful, and the patient was discharged on postoperative Day 4. Histopathology confirmed a cardiac PG with negative margins and positive SDHB mutation. The patient later underwent resection of an abdominal PG a year later, and is currently being evaluated for stereotactic radiosurgery of a large right jugular bulb lesion. This case exemplifies the importance of genetic screening, cross-specialty collaboration and flexible intraoperative planning in managing rare, high-risk tumours.
Cardiac PG is a rare neuroendocrine tumour that can cause life-threatening cardiovascular complications if undiagnosed. They are often mistaken for other cardiac masses, making awareness critical for early detection and appropriate management [8]. Reporting rare cases of cardiac PG helps expand limited clinical knowledge, to improve diagnostic accuracy and enhance surgical approaches, particularly in rare conditions. Understanding these rare tumours and the approach to its management is crucial in refining protocols, and providing evidence plus experience-based examples for other centres to use if encountered [9].
A case series of 15 patients reported an average age of 41.9 years at diagnosis [1], while a larger series of 128 patients found a slightly lower average age of 39.7 years [5]. Although most patients in the smaller series exhibited symptoms of catecholamine excess, they did not present with typical cardiac-related symptoms, such as chest pain or shortness of breath, as in our case. This suggests that the tumour location may influence symptom manifestation. Notably, one patient in their study – similar to the case presented here – had normal metanephrine levels, further reinforcing the variability in clinical presentation [1].
During diagnostic workup, 77% of these patients were associated with underlying mutations in SDHB, SDHC and SDHD, highlighting the crucial role of genetic evaluation. Identifying these mutations is essential for appropriate follow-up care and for providing genetic counselling to family members who may also be at risk [1].
Diagnostic imaging plays a vital role in evaluating the tumour’s relationship with cardiac structures, helping to prevent surgical complications [8]. Echocardiography was the primary imaging modality used for tumour visualization in the literature; however, it proved inaccurate in determining the exact tumour location in our case, leading to discrepancies between preoperative imaging and intraoperative findings. This limitation underscores the need for complementary imaging techniques to enhance the diagnostic accuracy. Previous studies have indicated that TTE is less sensitive than TOE in detecting cardiac PG. This reduced sensitivity is particularly problematic when the tumour is situated in the posterior aspect of the pericardium, as it may result in a false-negative diagnosis [5].
CT provides detailed anatomical visualization, enabling better assessment of tumour size, location and relationship with surrounding structures. However, CT lacks functional imaging capabilities and exposes patients to ionizing radiation. Magnetic resonance imaging (MRI) offers superior soft-tissue contrast, making it particularly useful for distinguishing cardiac tumours from thrombi and evaluating vascular invasion. The main drawback of MRI is availability, longer scan time, contraindications with metallic implants, and higher costs. For functional imaging, nuclear medicine techniques such as 68Ga-DOTATATE PET/CT and 123I-MIBG scintigraphy provide enhanced detection of catecholamine-secreting PGs, particularly those with metastatic potential. However, again the drawback is the limited availability and high costs compared with other conventional imaging techniques [10]. Given these considerations, a multimodal imaging approach is often recommended to optimize preoperative assessment, improve surgical planning and minimize diagnostic uncertainty.
In a large review of 158 patients diagnosed with cardiac PGs, the majority of tumours were intrapericardial, originating either from the epicardium or the root of great vessels. The other common locations include right atrium, right ventricle and atrioventricular groove, as in this particular case [15].
The study (158 patients) found that most of these tumours derived their blood supply from the coronary arteries, highlighting the critical role of vascular assessment in their diagnosis and management. Specifically, among 91 evaluated cases, 85 tumours (93.4%) exhibited a coronary artery blood supply, and coronary angiography proved highly valuable in delineating the tumour’s extent and feeding vessels, aiding in both diagnostic accuracy and surgical planning [11]. Although multiple tumours were fairly common within this series, metastases were rare, suggesting that with appropriate surgical management, patients with cardiac PG can have successful outcomes.
Surgical resection remains the gold-standard treatment for cardiac PGs, offering the best chance for long-term survival and symptom resolution. However, due to the vascularity and intrapericardial location of these tumours, surgery presents significant challenges that require careful preoperative planning. Intraoperative considerations include meticulous dissection of the tumour from the surrounding cardiac structures and adequate haemodynamic monitoring, as catecholamine-secreting tumours can cause severe intraoperative blood pressure fluctuations [10].
While complete surgical excision is the goal, cases where the tumour is highly invasive may necessitate partial resection or adjunctive therapies [10]. Minimally invasive approaches are being explored, but their feasibility depends on tumour location and size [12].
Postoperative outcomes largely depend on completeness of resection and genetic factors, as mutations in SDHB, SDHC or SDHD may increase the risk of recurrence or metastasis [4]. In our case, the patient had complete resection of the tumour with clear margins confirmed by histopathology, and went on to have an excision of an abdominal PG the year after and is currently scheduled for stereotactic radiosurgery of a 9-mm right jugular bulb PG due to complex anatomy.
Ongoing monitoring is essential due to risk of recurrence and catecholamine-related complications. A multidisciplinary approach is required between surgeons, oncologists, anaesthesiologists and endocrinologists to optimize patient outcomes and surgical intervention [12].
The prognosis of PG varies widely, with survival ranging from near-normal life expectancy to a 5-year survival rate of only 11.8% in some cases. Without treatment and surgical intervention, these tumours can cause severe complications, such as arrhythmias, heart failure and sudden cardiac death. Surgical excision significantly improves prognosis, with some studies suggesting life-expectancy close to that of age-matched controls [13].
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Authors
Muslim Mustaev, Laith Kayyal, Minji Ho, Kamran Baig & Christopher Young
Affiliations
Department of Adult Cardiac Surgery, Guy’s and St Thomas’ NHS Foundation Trust, London, UK
Corresponding Author
Laith Kayyal
Department of Adult Cardiac Surgery,
Guy’s and St Thomas’ NHS Foundation Trust,
London, UK
Email: laith.kayyal@gstt.nhs.uk
Keywords
© The Author 2025. Published by MMCTS on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
