Histiocytic neoplasm

A histiocytic neoplasm is a rare clonal hematologic malignancy characterized by the abnormal proliferation and infiltration of mature cells belonging to the mononuclear phagocyte system, including monocytes, macrophages, and dendritic cells

A histiocytic neoplasm is a rare clonal hematologic malignancy characterized by the abnormal proliferation and infiltration of mature cells belonging to the mononuclear phagocyte system, including monocytes, macrophages, and dendritic cells These neoplasms can present at any age, affecting pediatric and adult populations alike, and may arise as solitary lesions or as widespread multiorgan disease involving the bones, skin, central nervous system, lungs, liver, spleen, and lymph nodes The clinica...

These neoplasms can present at any age, affecting pediatric and adult populations alike, and may arise as solitary lesions or as widespread multiorgan disease involving the bones, skin, central nervous system, lungs, liver, spleen, and lymph nodes

These neoplasms can present at any age, affecting pediatric and adult populations alike, and may arise as solitary lesions or as widespread multiorgan disease involving the bones, skin, central nervous system, lungs, liver, spleen, and lymph nodes

The clinical spectrum ranges from indolent, localized conditions such as juvenile xanthogranuloma to aggressive, systemic diseases including Erdheim-Chester disease, Rosai-Dorfman disease, Langerhans cell histiocytosis, and histiocytic sarcoma Pathophysiologically, these disorders are frequently driven by mutually exclusive somatic activating mutations within the MAPK signaling pathway, most notably *BRAF* and *MAP2K1* mutations, leading to constitutive cellular proliferation and tissue inflamma...

Pathophysiologically, these disorders are frequently driven by mutually exclusive somatic activating mutations within the MAPK signaling pathway, most notably *BRAF* and *MAP2K1* mutations, leading to constitutive cellular proliferation and tissue inflammation