Histiocytic sarcoma with interdigitating dendritic cell differentiation.
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.
Porter
David W
DW
Liver Unit, Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK.
Gupte
Girish L
GL
Brown
Rachel M
RM
Spray
Chris
C
English
Martin W
MW
deVille DeGoyet
Jean
J
McKiernan
Patrick J
PJ
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