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Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

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Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
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Proton Therapy Delivery and Its Clinical Application in Select Solid Tumor Malignancies
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Published on: February 6, 2019

Cardiac tumours in children.

Orhan Uzun1, Dirk G Wilson, Gordon M Vujanic

  • 1Department of Paediatric Cardiology, University Hospital Of Wales, Heath Park Cardiff, CF14 4XW, Wales, UK. orhan.uzun@cardiffandvale.wales.nhs.uk

Orphanet Journal of Rare Diseases
|March 3, 2007
PubMed
Summary

Cardiac tumors are rare, with rhabdomyoma most common in children and myxoma in adults. While benign tumors have favorable outcomes, malignant cardiac tumors, especially metastatic ones, have a poor prognosis despite treatment.

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Area of Science:

  • Cardiology
  • Oncology
  • Pathology

Background:

  • Cardiac tumors, encompassing benign or malignant neoplasms, can be primary or metastatic, affecting the heart's inner lining, muscle, or pericardium.
  • Primary cardiac tumors are rare in pediatric practice (0.0017-0.28% autopsy series) but more common in fetal life (approx. 0.14%).
  • In children, most primary cardiac tumors are benign (rhabdomyoma being most common), whereas in adults, myxomas are most frequent among benign tumors, and sarcomas constitute the majority of malignant cardiac masses.

Purpose of the Study:

  • To provide a comprehensive overview of cardiac tumors, including their prevalence, types, diagnostic modalities, and treatment strategies.
  • To differentiate the characteristics and prognoses of cardiac tumors in pediatric versus adult populations.
  • To highlight the diagnostic gold standard and therapeutic considerations for both benign and malignant cardiac neoplasms.

Main Methods:

  • Review of existing literature on cardiac tumors, focusing on prevalence, pathology, and clinical presentation.
  • Analysis of diagnostic tools such as echocardiography, computed tomography (CT), and magnetic resonance imaging (MRI).
  • Evaluation of treatment outcomes for surgical resection, chemotherapy, and radiotherapy in primary and metastatic cardiac tumors.

Main Results:

  • Rhabdomyoma is the predominant cardiac tumor in fetal life and childhood, accounting for over 60% of primary pediatric tumors.
  • Adults exhibit a different spectrum, with 75% benign (myxomas most common) and 25% malignant tumors (sarcomas prevalent).
  • Malignant cardiac tumors, particularly metastatic ones, have an extremely poor prognosis, with palliative surgery offering limited benefit.

Conclusions:

  • Accurate diagnosis of cardiac tumors relies on non-invasive imaging and histological assessment via biopsy.
  • Surgical resection is recommended for symptomatic benign tumors, offering favorable outcomes.
  • While treatment options exist for malignant cardiac tumors, including surgery, chemotherapy, and radiotherapy, the overall prognosis remains poor, especially for metastatic disease.