Blog
Developing & Updating Clinical Guidelines for Histiocytosis
On January 16th, 2025, I attended an Annual Meeting for the National Comprehensive Cancer Network (NCCN)’s Histiocytic Neoplasms Panel. The physicians on the panel, from across leading cancer centers in the United States, have direct and regular experience treating histiocytosis. I sit on the panel as a Patient Advocate, to represent the patient voice as they develop and update the guidelines. Once the guidelines are published, I seek input from patients and/or bring perspective based on conversations I’ve had between the team and the community.
The National Comprehensive Cancer Network® (NCCN®) – an alliance of leading cancer centers – announced the publication of new NCCN Guidelines® for histiocytosis. These clinical practice guidelines provide the latest evidence and expert-consensus for diagnosing and treating the three most common forms of histiocytosis in adults: Langerhans cell histiocytosis (LCH), Erdheim-Chester Disease (ECD,) and Rosai Dorfman Disease (RDD). Although the guidelines are focused on adult patients, there may be insight for pediatric physicians as well. The Guidelines are listed as “histiocytic neoplasms” and can be found on NCCN’s website.
In 2021, the NCCN published these clinical guidelines for adults with Langerhans cell histiocytosis (LCH), Erdheim-Chester Disease (ECD), and Rosai-Dorfman Disease (RDD). Guidelines are updated at least annually; there are also interim updates for all new FDA drug approvals, expanded FDA drug indications, and when publications/presentations invite changes to clinical practice. Over the last few years, the panel has met many times to review the guidelines and make updates based on new best practices to workup/evaluation, baseline testing, diagnosis, treatment, and monitoring.
It has always been a goal of this panel to not only develop clinical guidelines for pediatric patients, but to also add other histiocytic disorders (malignant histiocytosis, hemophagocytic lymphohistiocytosis, xanthogranuloma). Pediatric patients are treated quite differently than adults, so the guidelines that were published were a helpful guide, however new guidelines will be ever more beneficial. Malignant histiocytosis is still very challenging to diagnose and treat, so the panel hopes these guidelines can bring greater awareness to these histiocytic disorders and help with treatment coordination and planning.
The meeting on January 16th was to develop guidelines for pediatric patients and malignant histiocytosis (including histiocytic sarcoma, interdigitating cell histiocytosis, and others). Prior to the meeting, two subcommittees met virtually, one for the pediatric guidelines and the other for malignant histiocytosis.
The subcommittee for pediatric LCH met to review the current clinical practice guidelines from initial diagnosis to workup and evaluation, and treatment of single-site/single-system versus multi-system LCH. The objective was to determine whether practices would be like the adult guidelines and if not, what the changes would be. For malignant histiocytosis, the guidelines are being developed from the very beginning. The task at hand for that subcommittee included mapping out every aspect of baseline testing, diagnosis, workup and evaluation, treatment, and monitoring. Upon presenting the work of both subcommittees, the full group discussed each element and provided input and direction for the next steps.
Until the guidelines are published, all conversations and potential changes are confidential, so we will plan to share the news when the guidelines are published again this year and hope to be able to offer several webinars to walk through the guidelines with all of you, including community doctors and other providers.
We are humbled and honored to have the opportunity to sit on this panel and are endlessly grateful to the clinicians who generously give their time and expertise to develop and update these clinical guidelines. I look forward to sharing more in the coming months and in the meantime, should you have any questions about the NCCN Guidelines, a recent diagnosis, finding a doctor, or anything in between, please do not hesitate to reach out: +1-856-589-6606 or info@histio.org.
