Erdheim-Chester disease in a female cardiac surgery patient

H R Mahoozi1, A Zittermann, K Hakim Meibodi

  • 1Clinic for Thoracic and Cardiovascular Surgery, Heart Center NRW, Bad Oeynhausen, Germany.

Insights

Erdheim-Chester disease (ECD) can present with isolated cardiac involvement. Surgical strategies for cardiac tumors should consider ECD, especially those with infiltrative growth patterns.

Area of Science:

  • Cardiology
  • Oncology
  • Pathology

Background:

  • Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis.
  • Cardiac involvement in ECD is uncommon and often associated with extracardiac manifestations.

Observation:

  • A 74-year-old female presented with superior vena cava syndrome.
  • PET-CT revealed obstructing and non-obstructing hypermetabolic cardiac lesions in the right and left atria, respectively.
  • No extracardiac disease was detected.

Findings:

  • Surgical exploration identified an infiltrative right atrial tumor.
  • Histological and immunohistochemical analysis confirmed Erdheim-Chester disease.
  • The tumor had invaded the pericardium.

Implications:

  • Isolated cardiac ECD is a potential diagnosis for cardiac tumors with infiltrative growth.
  • Cardiac ECD should be considered in the differential diagnosis of cardiac masses.
  • This case highlights the importance of considering rare diagnoses in cardiac oncology.

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