학술논문
Progressive nodular histiocytosis in a 9‐year‐old boy treated with cobimetinib.
Document Type
Article
Author
Source
Subject
*ERDHEIM-Chester disease
*LANGERHANS-cell histiocytosis
*NON-langerhans-cell histiocytosis
*CHILD patients
*MUCOUS membranes
*TREATMENT effectiveness
*RAS oncogenes
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Language
ISSN
0736-8046
Abstract
Progressive nodular histiocytosis is a rare variant of non‐Langerhans cell histiocytosis that affects the skin and mucous membranes and displays a progressive clinical course and poor response to treatment. We describe a case of severe progressive nodular histiocytosis harboring a KRAS p.G12S mutation in a 9‐year‐old boy, refractory to chemotherapy, who was successfully treated with the MEK inhibitor cobimetinib. This is the first report of the use of MEK inhibition for this histiocytosis subtype in a pediatric patient. [ABSTRACT FROM AUTHOR]