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

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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:

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E. Reference

  1. Robbins Pathologic Basis of Disease, 6th 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