Sarcomatoid Morphology in Pediatric Langerhans Cell Neoplasm Does Not Always Predict Aggressive Clinical Course

Authors

Sam Sirotnikov, Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, GA, USA.
Louis P. Dehner, Department of Pathology and Immunology, Washington University School of Medicine in St. Louis, St. Louis, MO, USA.
José E. Velázquez Vega, Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, GA, USA.
Jinjun Cheng, Department of Pathology and Laboratory Medicine, Children's National Hospital, Washington, DC, USA.

Document Type

Journal Article

Publication Date

9-28-2024

Journal

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society

DOI

10.1177/10935266241281517

Keywords

BRAF V600E; LCH; LCS; diagnostic criteria; histiocytosis; malignancy

Abstract

Langerhans cell sarcoma (LCS), a rare malignant neoplasm in the general category of myeloid neoplasms characterized by overtly malignant Langerhans cells (LC) with conspicuous mitotic activity including atypical forms. Although most cases occur in adults, rare examples of LCS have been reported in children with variable clinical outcome. We present 2 childhood cases of Langerhans cell neoplasm with high grade sarcomatous features and OSBPL9::BRAF fusion and BRAF V600E mutation.

APA Citation

Sirotnikov, Sam; Dehner, Louis P.; Velázquez Vega, José E.; and Cheng, Jinjun, "Sarcomatoid Morphology in Pediatric Langerhans Cell Neoplasm Does Not Always Predict Aggressive Clinical Course" (2024). GW Authored Works. Paper 5574.
https://hsrc.himmelfarb.gwu.edu/gwhpubs/5574

Department

Pathology