Erdheim-Chester Disease with Emperipolesis: A Unique Case Involving the Heart / Journal of the Korean Cancer Association, 대한암학회지
Cancer Research and Treatment
; : 553-558, 2017.
Article
in En
| WPRIM
| ID: wpr-63848
Responsible library:
WPRO
ABSTRACT
Histiocytosis is an uncommon disease characterized by excessive accumulation of histiocytes. Here, we report a rare case of non-Langerhans-cell histiocytosis in a 51-year-old woman who presented with severe symptoms of pericardial effusion. Radiologic investigation also detected multiple bone (lower limbs, vertebrae, ribs, and ilium) lesions. Resected pericardium showed abundant mono- or multi-nucleated non-foamy histiocytes (CD68⁺/CD163⁺/S-100⁺/CD1α⁻/langerin⁻) in a fibroinflammatory background. The histiocytes demonstrated emperipolesis of lymphocytes, a hallmark feature of Rosai-Dorfman disease (RDD). However, molecular analysis revealed a BRAF V600E mutation of the proliferating histiocytes, highlighting the neoplastic features frequently observed in another non-Langerhans-cell histiocytosis known as Erdheim-Chester Disease (ECD). We consider this case to be a unique presentation of ECD harboring some RDD-like cells with emperipolesis, but not a case of RDD with a BRAF mutation concerning its clinical manifestation (involvement of the heart and bones) and neoplastic features.
Key words
Full text:
1
Index:
WPRIM
Main subject:
Pericardial Effusion
/
Pericardium
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Ribs
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Spine
/
Lymphocytes
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Histiocytosis
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Histiocytosis, Non-Langerhans-Cell
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Histiocytosis, Sinus
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Erdheim-Chester Disease
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Extremities
Limits:
Female
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Humans
Language:
En
Journal:
Cancer Research and Treatment
Year:
2017
Type:
Article