XXXX : a review of past, current and future therapies.

@entity1 @entity66 @entity75 @entity1222 @entity149 @entity134 @entity2010
The goal of therapy in @entity1222 ( @entity1222 ) is to decrease the activity and proliferation of histiocytes, lymphocytes and macrophages that cause the disease. @entity1 with disease that is localized to skin, bone and lymph node (defined as "nonrisk" organs) generally have a good prognosis and require minimal treatment. However, @entity1 with lesions in "risk" organs (liver, spleen, lung, bone marrow) have a worse overall prognosis regarding mortality and morbidity. Likewise, @entity1 with @entity149 (CNS), vertebrae, facial bones or bones of the anterior or middle cranial fossa are at higher risk for morbidity and recurrent disease. @entity1222 in the orbit, mastoid or temporal skull regions are classified as "CNS risk" because of an increased frequency of developing @entity2010 or parenchymal @entity75 . Outcomes of @entity1 with @entity1222 in only one bone in "nonrisk" locations are generally benign, and the disease responds well to several treatment modalities including observation, surgical excision, @entity134 injection or radiation therapy. The last is generally reserved for a single @entity75 or when a risk of pathologic fracture exists in the greater trochanter. The greatest challenge in treatment of @entity1222 is @entity1 with @entity66 . @entity1 with persistent or worsening disease in risk organs by the end of the first 6-12 weeks of therapy have significantly decreased overall survival regardless of treatment. Additionally, optimal treatment for @entity1 with late-onset CNS symptoms and adults with @entity1222 remain to be defined. In this article we will review the evolution of multicenter and international treatment studies as well as the current Histiocyte Society research treatment protocol, @entity1222 -III. We also review experiences with a variety of agents that have been used to treat @entity1222 outside of clinical trials. Since @entity1222 is a rare disease in @entity1 and adults, these @entity1 should be enrolled on clinical trials whenever possible to advance our knowledge of the optimal therapeutic interventions and long-term outcomes.
