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Recent Advances in Histiocytic Neoplasms

PhD Trainee
Program: Education Program
Saturday, December 5, 2015: 2:00 PM-3:30 PM
W230, Level 2 (Orange County Convention Center)
Sunday, December 6, 2015: 9:30 AM-11:00 AM
W331, Level 3 (Orange County Convention Center)

Description:

Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD) are clinically heterogenous histiocytic neoplasms whose underlying etiology has long been unknown. Genomic analysis revealed the presence of somatic activating mutations of BRAFV600E in 50% of LCH and ECD patients, as well as mutations in MAP2K1 in 25% of BRAF-wild type LCH patients.

Dr. Donald Parsons will review the recent advances in understanding the molecular mechanisms of LCH and non-LCH histiocytic neoplasms. He will discuss the role of recurrent somatic activating MAPK pathway mutations in patients with LCH, ECD and JXG. He will propose re-definition of these disorders as myeloid neoplastic disorders driven by MAPK activation at critical stages of myelopoiesis.

Dr. Sheila Weitzman will give a clinical overview on LCH and discuss the rationale for current treatment strategies. She will discuss the possible correlation between BRAF/ARAF/MAP2K1 mutations and patient outcomes, and will review the potential benefit of treating resistant high-risk LCH cases with BRAFV600E inhibitors and the possible future role of RAF/MEK inhibitors.

Dr. Julien Haroche will discuss the clinical and diagnostic approach to ECD patients including the newly proposed ECD classification. He will review the various treatment options with Interferon-α, anticytokine-directed therapy, chemotherapy and targeted therapy in ECD with BRAF inhibitors. He will also give a clinical and treatment review on other uncommon histiocytic disorders such as Rosai-Dorfman disease (RDD) and Juvenile Xanthogranuloma (JXG) that are more commonly diagnosed in young patients.

Chair:
Oussama Abla, MD, The Hospital for Sick Children

Disclosures:
No relevant conflicts of interest to declare.

Donald Williams Parsons, MD, PhD

Texas Children's Cancer Center, Baylor College of Medicine, Houston, TX

Sheila Weitzman, MB BCh, FRCP

Pediatric Hematology-Oncology/ Universiy of Toronto, Hospital For Sick Children, Toronto, ON, Canada

Julien Haroche, MD, PhD

Institut E3M, Groupe Hospitalier Pitié-Salpêtrière, Paris, Paris, France

See more of: Education Program