Histiocytic sarcoma (HS) is a rare neoplasm of uncertain etiology. Most recently, the diagnostic criteria for this entity have been revised with inclusion of diagnostic modalities such as immunohistochemical and cytogentic techniques. HS tends to have an aggressive clinical course and presents with systemic symptoms of fever, weight loss, adenopathy, hepatosplenomegly, rash, and pancytopenia. There is no standard treatment and no comparative studies to the rarity of this disease. Thalidomide is a promising agent that may exert a therapeutic benefit but thus far it is supported only by case reports. A 2006 Health Technology Assessment (HTA): Usefulness of thalidomide for the management of sarcomas concluded that evidence supporting it in other sarcomas and even in Kaposi’s, where it has been studied the most, is anecdotal.
Muneer H. Abidi et al Thalidomide for the treatment of histiocytic sarcoma after hematopoietic stem cell transplant American Journal of Hematology Volume 82 Issue 10, Pages 932 – 933