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Related Experiment Video

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Multimodal Study of Murine Cardiovascular Remodeling: Four-Dimensional Ultrasound and Mass Spectrometry Imaging
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Multimodality Imaging in a Patient with Erdheim Chester Disease with Cardiovascular Involvement.

Priya Jagia1, Vineeta Ojha1, Gautam Sharma1

  • 1All India Institute of Medical Sciences, New Delhi, India.

Acta Medica Lituanica
|February 9, 2026
PubMed
Summary

This case study details a rare Erdheim Chester disease diagnosis in a woman with chest pain. Multimodality imaging was crucial for identifying the widespread xanthogranulomatous infiltration across multiple organs.

Keywords:
Erdheim Chester diseasemultimodality imaging

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Area of Science:

  • Cardiology
  • Oncology
  • Radiology
  • Pathology

Background:

  • Erdheim Chester disease is a rare non-Langerhans cell histiocytosis.
  • The condition can affect multiple organ systems, leading to diverse clinical presentations.

Purpose of the Study:

  • To report a case of Erdheim Chester disease presenting with cardiac and pineal gland involvement.
  • To emphasize the role of multimodality imaging in diagnosing this rare systemic disease.

Main Methods:

  • Cardiac MRI to assess cardiac involvement.
  • Biopsy of a pineal gland tumor for histopathological analysis.
  • CD 68 and CD1a staining for differential diagnosis.

Main Results:

  • A 40-year-old woman presented with chest pain and recurrent pericardial effusion.
  • Cardiac MRI showed a diffuse sheath-like mass in the right heart chambers.
  • Pineal gland tumor biopsy revealed xanthogranulomatous infiltration diagnostic of Erdheim Chester disease, with CD 68 positive and CD1a negative foamy histiocytes.
  • Bony and perirenal involvement were also noted.

Conclusions:

  • Erdheim Chester disease requires a high index of suspicion due to its rarity and varied manifestations.
  • Multimodality imaging is essential for comprehensive evaluation and timely diagnosis of Erdheim Chester disease.
  • Prompt diagnosis facilitates appropriate management and monitoring of systemic involvement.