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
en En
| WPRIM
| ID: wpr-63848
Biblioteca responsable:
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.
Palabras clave
Texto completo:
1
Índice:
WPRIM
Asunto principal:
Derrame Pericárdico
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Pericardio
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Costillas
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Columna Vertebral
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Linfocitos
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Histiocitosis
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Histiocitosis de Células no Langerhans
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Histiocitosis Sinusal
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Enfermedad de Erdheim-Chester
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Extremidades
Límite:
Female
/
Humans
Idioma:
En
Revista:
Cancer Research and Treatment
Año:
2017
Tipo del documento:
Article