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PubMed · 15591905

Histiocytic sarcoma with interdigitating dendritic cell differentiation.

David W Porter·Girish L Gupte·Rachel M Brown·Chris Spray·Martin W English·Jean deVille DeGoyet·Patrick J McKiernan
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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.

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BiopsyCell DifferentiationDendritic CellsHepatomegalyHistiocytic Disorders, MalignantHumansInfantMaleSarcomaSplenomegalyThrombocytopeniaCase ReportsJournal Article

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David W Porter, Girish L Gupte, Rachel M Brown, Chris Spray, Martin W English, Jean deVille DeGoyet, Patrick J McKiernan. 2004. Histiocytic sarcoma with interdigitating dendritic cell differentiation.. https://pubmed.ncbi.nlm.nih.gov/15591905/

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