Histiocytic Neoplasms, Version 2.2021, NCCN Clinical Practice Guidelines in Oncology
Ronald S. Go, Eric D. Jacobsen, Robert A. Baiocchi, Ilia N. Buhtoiarov, Erin Butler, Patrick Campbell, Donald W. Coulter, Eli L. Diamond, Aron Flagg, Aaron M. Goodman, Gaurav Goyal, Dita Gratzinger, Paul C. Hendrie, Meghan A. Higman, Michael D. Hogarty, Filip Jankú, Reem Karmali, David Morgan, Anne C. Raldow, Alexandra Stefanovic, Srinivas K. Tantravahi, Kelly Walkovich, Ling Zhang, Mary Anne Bergman, Susan Darlow
Abstract
Histiocytic neoplasms are rare hematologic disorders accounting for less than 1% of cancers of the soft tissue and lymph nodes. Clinical presentation and prognosis of these disorders can be highly variable, leading to challenges for diagnosis and optimal management of these patients. Treatment often consists of systemic therapy, and recent studies support use of targeted therapies for patients with these disorders. Observation ("watch and wait") may be sufficient for select patients with mild disease. These NCCN Guidelines for Histiocytic Neoplasms include recommendations for diagnosis and treatment of adults with the most common histiocytic disorders: Langerhans cell histiocytosis, Erdheim-Chester disease, and Rosai-Dorfman disease.