Recommendations for Managing Adults With Histiocytic Neoplasms: New NCCN Guidelines

Ronald S. Go
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The histiocytoses, a group of clonal and reactive conditions, arise from monocytic macrophage or dendritic cell lineages. The current NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Histiocytic Neoplasms reflect the most up-to-date, evidence-based data relating to the evaluation and management of this disease. Specifically, the guidelines focus on adult Langerhans cell histiocytosis, Erdheim-Chester disease, and Rosai-Dorfman disease. Because these disorders are rare, challenges have arisen regarding clinical suspicion, histologic diagnosis, treatment, and molecular subtyping. Future versions of the NCCN Guidelines will address the diagnosis and management of pediatric patients, as well as malignant histiocytosis.

Disclosures: Dr. Go has disclosed having no relevant financial relationships.

Correspondence: Ronald S. Go, MD, Mayo Clinic Cancer Center, 200 First Street SW, Rochester, MN 55905. Email:
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    Go RS, Jacobsen E, Baiocchi R, et al. NCCN Clinical Practice Guidelines in Oncology: Histiocytic Neoplasms. Version 2. 2021. Accessed September 21, 2021. To view the most recent version, visit

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