histiocytic sarcoma

Synopsis

 pleomorphic histiocytic cells: medium cells, large cells and the giant cells expressing CD68, lysozyme and S100 protein.
 erythrophagocytosis (erythrophagocytic histiocytosis)
 lymphocytophagocytosis

Forms

 localized forms (localized histiocytic sarcoma)

• spleen (splenic histiocytic sarcoma)
• central nervous system (12548175)

 systemic histiocytic sarcoma or disseminated histiocytic sarcoma
 malignant histiocytosis with trisomy 9 following primary mediastinal germ cell tumor (16809892)

Immunochemistry

 CD11+
 CD13+
 HLA-DR+
 CD33+
 lysozyme+
 S-100+

Associations

 histiocytic sarcoma following allogeneic bone marrow transplantation for T-ALL (14513060)
 disseminated histiocytic sarcoma with autoimmune lymphoproliferative syndrome (ALPS) and Rosai-Dorfman disease (20216376)
 malignant histiocytosis with trisomy 9 following primary mediastinal germ cell tumor (16809892)
 true histiocytic malignancy associated with a malignant teratoma in a patient with 46XY gonadal dysgenesis (1346359)

References

 Development of disseminated histiocytic sarcoma in a patient with autoimmune lymphoproliferative syndrome and associated Rosai-Dorfman disease. Venkataraman G, McClain KL, Pittaluga S, Rao VK, Jaffe ES. Am J Surg Pathol. 2010 Apr;34(4):589-94.PMID: 20216376

 Audouin J, Vercelli-Retta J, Le Tourneau A, Adida C, Camilleri-Broet S, Molina T, Diebold J. Primary histiocytic sarcoma of the spleen associated with erythrophagocytic histiocytosis. Pathol Res Pract. 2003;199(2):107-12. PMID: 12747473

 Boisseau-Garsaud AM, Vergier B, Beylot-Barry M, Nastasel-Menini F, Dubus P, de Mascarel A, Eghbali H, Beylot C. Histiocytic sarcoma that mimics benign histiocytosis. J Cutan Pathol. 1996 Jun;23(3):275-82.
PMID: 8793665

 Koo CH, Reifel J, Kogut N, Cove JK, Rappaport H. True histiocytic malignancy associated with a malignant teratoma in a patient with 46XY gonadal dysgenesis. Am J Surg Pathol. 1992 Feb;16(2):175-83. PMID: 1346359