scholarly journals Histiocytic Neoplasms, Version 2.2021, NCCN Clinical Practice Guidelines in Oncology

2021 ◽  
Vol 19 (11) ◽  
pp. 1277-1303
Author(s):  
Ronald S. Go ◽  
Eric Jacobsen ◽  
Robert Baiocchi ◽  
Ilia Buhtoiarov ◽  
Erin B. Butler ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Clinical Presentation ◽  
Optimal Management ◽  
Watch And Wait ◽  
Hematologic Disorders ◽  
Nccn Guidelines ◽  
Mild Disease ◽  
Rosai Dorfman Disease ◽  
Erdheim Chester ◽  
Chester Disease

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.

scholarly journals Recommendations for Managing Adults With Histiocytic Neoplasms: New NCCN Guidelines

2021 ◽  
Vol 19 (11.5) ◽  
pp. 1355-1357
Author(s):  
Ronald S. Go
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Pediatric Patients ◽  
Clinical Suspicion ◽  
Evidence Based ◽  
Malignant Histiocytosis ◽  
Histologic Diagnosis ◽  
Nccn Guidelines ◽  
Cell Lineages ◽  
Rosai Dorfman Disease ◽  
Erdheim Chester

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.

Erdheim?Chester disease of the breast associated with Langerhans-cell histiocytosis of the hard palate

2004 ◽  
Vol 445 (4) ◽  
pp. 405-409 ◽  
Author(s):  
V. P. Andrade ◽  
C. C. V. Nemer ◽  
A. N. L. Prezotti ◽  
W. S. L. Goulart
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Hard Palate ◽  
Langerhans Cell ◽  
Erdheim Chester Disease ◽  
Erdheim Chester ◽  
Chester Disease

scholarly journals Dramatic efficacy of dabrafenib in Erdheim-Chester disease (ECD): a pediatric patient with multiple large intracranial ECD lesions hidden by refractory Langerhans cell histiocytosis

2019 ◽  
Vol 23 (1) ◽  
pp. 48-53 ◽  
Author(s):  
Xiaolei Hao ◽  
Ruie Feng ◽  
Yalan Bi ◽  
Yuhan Liu ◽  
Chunde Li ◽  
...  
Keyword(s):  
Pediatric Patient ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Neurological Involvement ◽  
Treatment Processes ◽  
Erdheim Chester Disease ◽  
Intracranial Lesions ◽  
The Central Nervous System ◽  
Erdheim Chester ◽  
Chester Disease

Erdheim-Chester disease (ECD) is a rare non–Langerhans cell form of histiocytosis that can affect the central nervous system. ECD predominantly affects adults, and only a few pediatric cases have been reported. The co-occurrence of ECD and Langerhans cell histiocytosis (LCH) is exceedingly rare. An 11-year-old boy, who was diagnosed with LCH 7 years previously, presented with multiple giant intracranial lesions. At the time of his initial diagnosis, only one intracranial lesion was observed, and it began to enlarge. Currently, up to 7 intracranial lesions can be observed in this patient. However, the diagnosis of ECD was not confirmed until this most recent open resection. The BRAF V600E mutation was detected in both LCH and ECD lesions. Dabrafenib therapy exhibited dramatic efficacy in this pediatric patient. This case represents the first successful application of dabrafenib in a pediatric patient with intracranial ECD lesions as well as mixed ECD and LCH. In this article, the authors describe the intricate diagnosis and treatment processes in this patient. Recent studies regarding treatment with BRAF inhibitors for neurological involvement in mixed ECD and LCH are also reviewed.

scholarly journals The clinical spectrum of Erdheim-Chester disease: an observational cohort study

2017 ◽  
Vol 1 (6) ◽  
pp. 357-366 ◽  
Author(s):  
Juvianee I. Estrada-Veras ◽  
Kevin J. O’Brien ◽  
Louisa C. Boyd ◽  
Rahul H. Dave ◽  
Benjamin H. Durham ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Age Of Onset ◽  
Observational Cohort Study ◽  
World Health ◽  
Key Points ◽  
The World ◽  
Observational Cohort ◽  
Health Organization ◽  
Erdheim Chester ◽  
Chester Disease

Key Points ECD varies in terms of age of onset, clinical presentation, manifestations, organ involvement, disease severity, and survival. ECD is a neoplasm and should be adopted by the field of hematology-oncology following the World Health Organization reclassification.

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