《Slide 104.》Langerhans cell histiocytosis, Soft tissue

A. Brief Descriptions：

1. Definition: Langernans cell histiocytosis is a neoplastic proliferaion of Langerhans cells, with expresion of CD1a, S-100-protein, and the presence of Birbeck granules by ultrastructural examination.

2. Synonyms: Histiocytosis X, Langerhans cell granulomatosis.

3. Clinical variants: Letterer-Siwe disease, Hand-Schuller-Christian disease, and solitary eosinophilic granuloma.

B. Gross Findings：

      略.

C. Micro Findings：

1. Langerhans cells

   • 10~15 m, grooved, folded, indented, or lobulated nuclei (kidney-shaped).

   • Fine chromatin, inconspicuous nucleoli and thin nuclear membranes.

   • Cytoplasm: moderately abundant and slightly eosinophilic.

2. Background: eosinophils, , histiocytes, neutrophils, lymphocytes. Eosinophilic  microabscess formation is noticed.

3. Focal necrosis and edema.

D. Others:

     略.

E. Reference：

1. Robbins Pathologic Basis of Disease, 6^th ed.  P.685-686.

　

 

【 Fig. 104-1 (LP)】Necrosis and microabscess formation.

　

 

 

【 Fig. 104-2 (LP)】Necrosis with predominant eosinophils infiltrates.

　

 

 

【 Fig. 104-3 (LP)】Proliferation of Langerhans cells with intermingled eosinophils, neutrophils and lymphocytes.

　

 

【 Fig. 104-4 (HP)】Eosinophilic microabscess.

 

 

【 Fig. 104-5 (HP)】Langerhans cells: kidney-shaped ,grooved, folded nuclei with vesicular appearance.Fine chromatin and  nucleoli are occasionally seen