title: Histiocytic sarcoma with interdigitating dendritic cell differentiation.
abstract: The authors describe a rare tumor with overlapping features of true histiocytic sarcoma and interdigitating dendritic cell sarcoma occurring in infancy. A 3-month-old boy presented with hepatosplenomegaly and abdominal distention. He presented a diagnostic challenge at initial presentation despite extensive investigations. On follow-up at age 16 months he had progressive splenomegaly, thrombocytopenia, and worsening coagulopathy. A diagnosis of histiocytic sarcoma with interdigitating dendritic cell differentiation was established after an open biopsy of liver and spleen. A repeat liver biopsy coupled with splenic biopsy is recommended in young children with unexplained hepatosplenomegaly and thrombocytopenia.
authors.affiliation: Liver Unit, Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK.
authors.forename: David W
authors.initials: DW
authors.lastname: Porter
authors.forename: Girish L
authors.initials: GL
authors.lastname: Gupte
authors.forename: Rachel M
authors.initials: RM
authors.lastname: Brown
authors.forename: Chris
authors.initials: C
authors.lastname: Spray
authors.forename: Martin W
authors.initials: MW
authors.lastname: English
authors.forename: Jean
authors.initials: J
authors.lastname: deVille DeGoyet
authors.forename: Patrick J
authors.initials: PJ
authors.lastname: McKiernan
mesh_terms: D001706:Biopsy; D002454:Cell Differentiation; D003713:Dendritic Cells; D006529:Hepatomegaly / Q000209:etiology / Q000473:pathology; D015620:Histiocytic Disorders, Malignant / Q000473:pathology*; D006801:Humans; D007223:Infant; D008297:Male; D012509:Sarcoma / Q000473:pathology*; D013163:Splenomegaly / Q000209:etiology / Q000473:pathology; D013921:Thrombocytopenia / Q000209:etiology