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mammary Erdheim-chester disease

Friday 21 May 2010

Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis of unknown etiology, the commonest sites of involvement being the long bones, skin, orbit, pituitary and retroperitoneum. Breast involvement is rare. (20216377)

SYnopsis

- diffuse xanthomatous infiltrate with scattered Touton-type giant cells
- patchy lymphocytic infiltrate.
- The cells are CD68 positive, and negative for S100, CD1a and a broad panel of cytokeratins.

Perivascular infiltration is also a rare but recognized presentation of Erdheim-Chester disease.

Histiocytic proliferations including ECD can mimic breast carcinoma clinically, radiologically, and histologically, and should be considered in the differential diagnosis of breast mass lesions. (20216377)

See also

- Erdheim-Chester disease

References

- Erdheim-chester disease presenting as bilateral clinically malignant breast masses. Provenzano E, Barter SJ, Wright PA, Forouhi P, Allibone R, Ellis IO. Am J Surg Pathol. 2010 Apr;34(4):584-8. PMID: 20216377