Primary Cardiac Paraganglioma Management and Resection With Rare Recurrent Metastasis

Q: What is the clinical evidence from this study?

Study design: Case Report. Population: Primary cardiac paraganglioma (n=1). Intervention: Cardiac autotransplantation and tumor resection.

Q: What does this research mean for the field?

Primary cardiac paragangliomas carry metastatic potential requiring multidisciplinary management and long-term surveillance, with targeted systemic therapies offering options for distant recurrence. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

Ashley B. Chuong; Marcell Szekely; María G. López-Treviño; Dale J. Hamilton; Michael J. Reardon; Marvin D. Atkins

doi:10.1016/j.jaccas.2026.109928

August 1, 2026JACC Case ReportsOpen Access

Primary Cardiac Paraganglioma Management and Resection With Rare Recurrent Metastasis

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Key result

Cardiac autotransplantation enables initial survival for massive cardiac paraganglioma despite vertebral metastasis at 1 year.

n=1

Graphical abstract published by JACC Case Reports — Graphical abstract · Published by JACC Case Reports · CC BY-NC-ND 4.0

Why the study?

Primary cardiac paragangliomas are rare tumors with nonspecific symptoms that require early recognition and multidisciplinary management due to diagnostic challenges and metastatic potential.

Key Points

To describe the complex surgical resection and subsequent oncologic management of a rare, functional primary cardiac paraganglioma with delayed metastatic recurrence.
Evaluated a 55-year-old male presenting with atrial fibrillation, pericarditis, and a 10 × 12 × 5 cm functional cardiac mass involving the left atrium.
Performed complete tumor excision and left atrial reconstruction via cardiac autotransplantation, followed by longitudinal surveillance, targeted systemic therapy, radiation, and kyphoplasty upon recurrence.
Achieved complete surgical resection of the primary mass, though the postoperative course required extracorporeal membrane oxygenation support and was complicated by a stroke.
Detected a biopsy-confirmed T9 vertebral metastasis during surveillance one year postoperatively, which was subsequently treated with belzutifan, radiotherapy, and kyphoplasty.

Study Design

Type

Case Report (n=1)

Structured PICO

P

Population

A 55-year-old man presenting with a 10 x 12 x 5 cm primary cardiac paraganglioma involving the left atrium, followed for over 1 year.

I

Intervention

Cardiac autotransplantation with complete tumor resection and left atrial reconstruction, followed by surveillance and subsequent Belzutifan, radiation, and kyphoplasty for vertebral metastasis.

Cardiac paragangliomas are complex tumors that may require advanced surgical techniques like autotransplantation and necessitate long-term surveillance due to the potential for distant recurrence.

Implication

Alerts clinicians to paraganglioma in atypical pericarditis with AF; leaves open standardized protocols pending larger series.

Abstract

BACKGROUND: Primary cardiac paragangliomas are rare tumors that present with nonspecific sympathomimetic symptoms. Early recognition and multidisciplinary planning are essential. CASE SUMMARY: A 55-year-old man presented with atrial fibrillation and pericarditis after weeks of dizziness, presyncope, and dyspnea. The patient underwent biopsy via thoracotomy, which was negative for malignancy. Workup revealed a 10 × 12 × 5 cm functional paraganglioma with left atrial involvement. He underwent cardiac autotransplantation with complete tumor resection and left atrial reconstruction. Postoperative recovery was complicated by extracorporeal membrane oxygenation requirement and stroke. One year later, surveillance identified a T9 vertebral metastasis, confirmed on biopsy, and later treated with Belzutifan, radiation, and kyphoplasty. DISCUSSION: Cardiac paragangliomas often require complex surgical approaches. This case highlights diagnostic difficulty, metastatic potential, and evolving systemic therapies of interest to cardiovascular and oncology specialists. TAKE-HOME MESSAGES: Cardiac paragangliomas demand multidisciplinary management and long-term surveillance. Newer targeted therapies expand options for metastatic disease.

Discussion

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Cite This Study

Chuong et al. (2026) conducted a case report in Primary cardiac paraganglioma (n=1). Cardiac autotransplantation and tumor resection was evaluated. Cardiac autotransplantation with complete tumor resection for a 10x12x5 cm cardiac paraganglioma allowed for initial survival, though a vertebral metastasis occurred at 1 year.

synapsesocial.com/papers/6ad0fcbf15427f6a84040314 https://doi.org/10.1016/j.jaccas.2026.109928

Topics

Atrial fibrillation

Persistent AF management

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Primary Cardiac Paraganglioma Management and Resection With Rare Recurrent Metastasis

Key Points

Study Design

Structured PICO

Implication

Abstract

Discussion

Cite This Study

Topics

Primary Cardiac Paraganglioma Management and Resection With Rare Recurrent Metastasis

Key Points

Study Design

Structured PICO

Implication

Abstract

Discussion

Cite This Study

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Topics

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