Histiocytic Sarcoma: A Challenging Dilemma between Diagnosis and Treatment: Review of Articles

Abstract

Histiocytic sarcoma (HS) is rare malignant disorder. It mainly affects lymph node, and can affect also extra nodal tissue. HS diagnosis is based on combination of histologically and immunohistochemically feature of histiocyte lineage with expression of CD68 and CD163. Usually, HS cases presents with advanced disease in a high mortality state. Most patients die from disseminated disease within two years. There is no consensus regarding treatment of HS. Patient with low-staged disease can be cured by a complete surgical resection while it is highly difficult to treat in advanced staged disease. HDCT followed by ASCT is an effective choice for those with relapsed HS as reported by literature. Great improvement to HS patient with Alemtuzumab therapy was observed. Further evaluation of immunosuppressive medication like Alemtuzumab for treatment of HS is needed as well as continued publishing case reports and case series since it is considered the primary source of information due to rarity of the disease.