Adult: Cardiac Tumor
Management of primary cardiac paraganglioma

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Abstract

Objective

Cardiac paraganglioma is a rare tumor that most surgeons have limited experience treating. The objective of this study is to examine the management and outcomes for cardiac paraganglioma treatment when cared for by a multidisciplinary cardiac tumor team.

Methods

We reviewed our institutionally approved cardiac tumor database from March 2004 to June 2020 for cardiac paraganglioma. These prospectively collected data were retrospectively reviewed. Patient characteristics were presented for individual patients and as summary statistics. Demographic and clinical data were also reported as median and interquartile range for continuous variables and frequencies and proportions for categoric variables. Kaplan–Meier curves were used to depict the patient survival from surgery.

Results

There were 21 cases of primary cardiac paraganglioma, 19 of whom had surgical resection with 3 refusing offered surgery. Of 19 resected tumors, 13 originated from the left atrium and 6 originated from the roots of the pulmonary artery and the aorta. Complex procedures were required, including aortic and pulmonary root replacement and 8 autotransplants. All tumors had complete gross resection with no identifiable disease left behind, but 4 of these had microscopically positive margins. None of the patients had local recurrence of disease. There was 1 case of metastatic paraganglioma with death at 4 years postsurgery. Operative mortality was 10.6%. Survival from surgery was 88.2%, 71.8%, and 71.8% and 1, 5, and 10 years, respectively.

Conclusions

Cardiac paraganglioma presents a surgical challenge. Mortality and long-term survival after surgical resection are acceptable but may require complex resection and reconstruction.

Section snippets

Materials and Methods

Analysis of our cardiac tumor database identified 21 cases of PCP, with 19 undergoing attempted complete surgical resection between March 2004 and June 2020 and 3 refusing the offered surgery. All consecutive patients referred for evaluation were considered for the study, and no patients were excluded except those who refused surgery and consent into the study. Treatment planning was done with a multidisciplinary cardiac tumor board at Houston Methodist Hospital and MD Anderson Cancer Center,

Results

A total of 19 patients underwent attempted complete surgical resection of a cardiac paraganglioma. Baseline patient demographics are presented in Table 1. Individual patient presentations are listed in Table E1, with shortness of breath as the most common symptom. Unlike patients with pheochromocytoma, only 1 patient with cardiac paraganglioma presented with hypertensive or hyperadrenergic episodes. Median age at the time of surgery was 53.4 years (IQR, 35.8-62.8). Eleven patients (57.9%) were

Discussion

The primary finding of this study shows that operative and 30-day hospital mortality are 10.6% in this series. Operative morbidity was substantial in these complex cases, including transfusion in 14 of 19 cases (73.7%) with a median of 5 units of packed red blood cells transfused, cardiac tamponade, mechanical cardiac support including IABP and RVAD, and prolonged respiratory failure requiring tracheostomy. Survival from surgery at 1, 5, and 10 years was 88.2%, 71.8%, and 71.8%, respectively,

Conclusions

Cardiac paragangliomas are rare and surgically challenging tumors because of their vascularity and proximity to critical surrounding structures. Complete surgical excision is the treatment of choice and may benefit from management at experienced centers. Operative mortality is reasonable for this complex disease, and long-term outcomes are favorable in patients with resection having negative or only microscopically positive margins. A multidisciplinary heart tumor team is helpful in our

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  • EY Chan, MacGillivray TE, Reardon MJ. Surgical management of cardiac paraganglioma. Op Tech Thorac Cardiovasc Surg....
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    Approved by the Houston Methodist Hospital Institutional Review Board.

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