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Published on: July 18, 2014
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.
Abstract:
We report a case of Erdheim-Chester disease (ECD) with isolated cardiac involvement in a 74-year-old female patient. The patient initially presented with superior vena cava syndrome and PET-CT imaging demonstrating an obstructing hypermetabolic lesion in the right atrium, and a distinct nonobstructing hypermetabolic lesion in the left atrium, expected to be malignant. There was no evidence of extracardiac disease. At surgical exploration, consistent with malignancy, the right atrial tumor was found to have grown into the pericardium and was resected to address symptoms and for histological diagnosis which revealed ECD on immunohistochemistry. We conclude that isolated cardiac ECD should be included in the surgical strategy for cardiac tumors showing infiltrative growth.
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