Why the study?
Langerhans cell histiocytosis is a rare clonal myeloid neoplasm, and cardiac involvement is exceedingly uncommon in neonates.
Comprehensive staging and precise anatomical imaging can guide safe observation in isolated epicardial LCH in neonates, which may spontaneously regress.
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LCH should be considered in neonatal pericardial effusion with epicardial mass; leaves open optimal management in BRAF-negative cases.
Langerhans cell histiocytosis (LCH) is a rare clonal myeloid neoplasm. Cardiac involvement is exceedingly uncommon in neonates. We report a term male neonate with prenatally detected pericardial effusion and a postnatally identified epicardial mass adherent to the left ventricle. Multimodal imaging demonstrated confinement to the visceral pericardium without myocardial infiltration. Biopsy confirmed LCH (CD1a⁺, CD207⁺, S-100⁺, ALK⁻, BRAFV600E⁻) and staging excluded systemic disease. The patient remained hemodynamically stable and was managed conservatively after multidisciplinary evaluation. Serial imaging demonstrated complete spontaneous regression by 6 months. Comprehensive staging and precise anatomical imaging can guide safe observation in isolated epicardial LCH.
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Rocha et al. (2026) studied this question.
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