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Identification and clinical course of 166 pediatric cardiac tumors

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Abstract

The aim of this study was to investigate the pathological classifications, clinical features, and natural history of pediatric cardiac tumors to provide a basis for the selection of an appropriate therapeutic method. The medical records of in- or outpatients with cardiac tumors at four hospitals were classified to analyze various types of tumor growth locations, clinical manifestations, surgical indications, and long-term follow-up results. There were 166 patients, including 158 with primary cardiac tumors, six with metastatic cardiac tumors, and two with unclassified cardiac tumors. Among the 158 cases of primary cardiac tumor, 150 were benign and eight were malignant. The rhabdomyoma, fibroma, and myxoma are the most common types of benign cardiac tumors. The major clinical manifestations of cardiac tumors include outflow tract obstruction, arrhythmia, dyspnea, pericardial effusion, heart failure, and seizures. Among the 59 patients who underwent surgery, 49 had primary benign cardiac tumors, eight had primary malignant tumors, and two had malignant metastatic tumors. Post-surgery, nine of the patients had residual tumor tissues that did not significantly affect their hemodynamics. Following surgery, there were two cases of recurrence and nine deaths, including four of benign and five of malignant tumors with mortality rates of 8.2 and 50.0 %, respectively. Of the remaining 107 cases of patients who did not undergo surgery, five (4.7 %) died.

Conclusion: The primary benign cardiac tumors are the predominant pediatric cardiac tumors, of which rhabdomyoma, fibroma, and myxoma are the most common types. If severe symptoms are nonexistent and the hemodynamics is unaffected, most of the patients can survive in the long term despite the tumors.

What is known:

• Pediatric cardiac tumors are rare and are predominantly primary and benign.

• The symptoms of heart failure, arrhythmia, and outflow obstruction are the most severe complications of cardiac tumors.

What is new:

• The rhabdomyoma, fibroma, and myxoma are the most common types of primary benign cardiac tumors.

• If severe symptoms are not present and the hemodynamics are unaffected, most of the patients can survive in the long term despite the tumors.

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Abbreviations

2DA:

Two-dimensional echocardiography

CMR:

Cardiac magnetic resonance

CT:

Computed tomography

LA:

Left atrium

LV:

Left ventricle

MPA:

Main pulmonary artery

MV:

Mitral valve

RA:

Right atrium

RV:

Right ventricle

TV:

Tricuspid valve

VS:

Ventricular septum

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Authors’ Contributions

Lin Shi collected medical data and wrote the paper. Lanping Wu performed echocardiography of the patients. Huijuan Fang was in charge of the patients of Shandong Provincial Hospital. Bo Han was in charge of the patients of Shandong Provincial Hospital. Jialun Yang was in charge of the patients of Children’s Hospital, Fudan University. Xiaojin Ma was in charge of the patients of Children’s Hospital, Fudan University. Fang Liu is the Chief Pediatrician of Children’s Hospital, Fudan University. Yongwei Zhang was in charge of the patients of Shanghai Children’s Hospital, Medical Institute, Shanghai Jiaotong University. Tingting Xiao was in charge of the patients of Shanghai Children’s Hospital, Medical Institute, Shanghai Jiaotong University. Min Huang is the Chief Pediatrician of Shanghai Children’s Hospital, Medical Institute Shanghai Jiaotong University. Meirong Huang is the Chief Pediatrician of Shanghai Children’s Medical Center, Medical Institute, Shanghai Jiaotong University and was in charge of the paper writing.

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Authors and Affiliations

  1. Shanghai Children’s Medical Center, Medical Institute, Shanghai Jiaotong University, Shanghai, China

    Lin Shi, Lanping Wu & Meirong Huang

  2. Shandong Provincial Hospital, Jinan, People’s Republic of China

    Huijuan Fang & Bo Han

  3. Children’s Hospital, Fudan University, Shanghai, China

    Jialun Yang, Xiaojin Ma & Fang Liu

  4. Shanghai Children’s Hospital, Medical Institute, Shanghai Jiaotong University, Shanghai, China

    Yongwei Zhang, Tingting Xiao & Min Huang

  5. Department of Pediatric Cardiology, Shanghai Children’s Medical Center, Medical Institute, Shanghai Jiao Tong University, 1678, Dongfang Road, Shanghai, 200127, China

    Meirong Huang

Authors
  1. Lin Shi
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  2. Lanping Wu
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  7. Fang Liu
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Corresponding author

Correspondence to Meirong Huang.

Ethics declarations

This study was retrospective without any funding support.

Conflict of interestAll the authors declare that they have no conflict of interest.Ethics approval and consent to participateAll procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. Informed consent was obtained from all individual participants included in the study.

Additional information

Communicated by Jaan Toelen

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Cite this article

Shi, L., Wu, L., Fang, H. et al. Identification and clinical course of 166 pediatric cardiac tumors. Eur J Pediatr 176, 253–260 (2017). https://doi.org/10.1007/s00431-016-2833-4

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  • DOI: https://doi.org/10.1007/s00431-016-2833-4

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