Journal
JOURNAL OF THE NATIONAL COMPREHENSIVE CANCER NETWORK
Volume 19, Issue 11, Pages 1277-1303Publisher
HARBORSIDE PRESS
DOI: 10.6004/jnccn.2021.0053
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Histiocytic neoplasms are rare hematologic disorders, often treated with systemic therapy. For patients with mild disease, observation may be sufficient.
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 RosaiDorfman disease.
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