Pathology of Histiocytic Disorders and Neoplasms and Related Disorders

  • Jennifer Picarsic
  • Ronald Jaffe


The pathology of histiocytic disorders and neoplasms of the macrophage-dendritic cell lineages is heterogeneous and may have overlapping features, all the while made more difficult by their rarity. The morphologic features together with immunophenotype and pattern of involvement should be taken together with the clinical and radiographic findings to make a unifying diagnosis. The “molecular microscope” of these lesions is further unraveling their biologic potential, which in some cases has helped to solidify their standing as inflammatory myeloid neoplasms. The histopathologic clues for the diagnosis of Langerhans cell histiocytosis (LCH), Erdheim-Chester Disease (ECD), the “juvenile xanthogranuloma” (JXG) family of lesions, Rosai-Dorfman disease (RDD), malignant histiocytoses including histiocytic sarcoma and Langerhans cell sarcoma, and other less common histiocytic-related disorders, including hemophagocytic lymphohistiocytosis (HLH), are reviewed in this chapter.


Langerhans cell histiocytosis (LCH) Erdheim-Chester disease (ECD) Juvenile xanthogranuloma (JXG) Rosai-Dorfman disease (RDD) Malignant histiocytoses Histiocytic sarcoma Langerhans cell sarcoma Indeterminate cell histiocytosis Hemophagocytic lymphohistiocytosis (HLH) 


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© Springer International Publishing AG 2018

Authors and Affiliations

  1. 1.Department of PathologyUniversity of Pittsburgh School of Medicine, Children’s Hospital of Pittsburgh of UPMCPittsburghUSA
  2. 2.Department of PathologyChildren’s Hospital of Pittsburgh of UPMCPittsburghUSA
  3. 3.Department of PathologyUniversity of Pittsburgh School of Medicine, Magee Women’s Hospital of UPMCPittsburghUSA

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