title: Pathology of extramedullary mastocytosis.
abstract: Mastocytosis encompasses a group of clinically and pathologically heterogeneous disorders most commonly involving the skin, which typically takes the form of urticaria pigmentosa. Mastocytosis may also involve other organs, most often bone marrow, followed by gastrointestinal tract, liver, spleen, and lymph nodes. The presence of extracutaneous involvement by mastocytosis is a major diagnostic criterion for systemic disease. However, mast cell infiltrates are often subtle in skin and extracutaneous organs, and the histologic features of mastocytosis at different anatomic sites may be variable. This article reviews the pathologic features and clinical correlates of mastocytosis involving skin and other extramedullary sites.
authors.affiliation: Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, 75 Francis Street, Boston, MA 02115 USA.
authors.forename: Leona A
authors.initials: LA
authors.lastname: Doyle
authors.affiliation: Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, 75 Francis Street, Boston, MA 02115 USA; Mastocytosis Center, Brigham and Women's Hospital, 75 Francis Street, Boston, MA 02115, USA. Electronic address: jhornick@partners.org.
authors.forename: Jason L
authors.initials: JL
authors.lastname: Hornick
mesh_terms: D000328:Adult; D015703:Antigens, CD / Q000235:genetics / Q000378:metabolism; D002277:Carcinoma / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D002648:Child; D005753:Gastric Mucosa / Q000378:metabolism; D005770:Gastrointestinal Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D015870:Gene Expression; D006801:Humans; D008407:Mast Cells / Q000378:metabolism / Q000473:pathology*; D008415:Mastocytosis / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D009154:Mutation; D011379:Prognosis; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics / Q000378:metabolism; D012867:Skin / Q000378:metabolism / Q000473:pathology*; D013270:Stomach / Q000473:pathology*; D053802:Tryptases / Q000235:genetics / Q000378:metabolism
chemical_list: D015703:Antigens, CD; D019009:Proto-Oncogene Proteins c-kit; D053802:Tryptases
keywords: KIT; Mast cell sarcoma; Mast cells; Mastocytosis; Urticaria pigmentosa