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Yang et al.                                                                                                                                                           Aggressive primary hepatic histiocytic sarcoma

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           Figure 3: (A) Fine needle aspiration showed pleomorphic nuclei   Figure 4: (A) CD163 immunostain; (B) CD68 immunostain. Tumor
           with prominent nucleoli (Papanicolaou, ×40); (B) Cell block of the   cells are strongly positive for CD163, with less distinct staining for
           tumor showed a poorly differentiated neoplasm with epithelioid and   CD68 (×10)
           spindle cell features in a background of fibrin and blood (HE, ×40)
                                                              hemodialysis. Positron emission tomography CT scan
           vimentin, CD34, chromogranin, synaptophysin, CD56,   suggested development of  possible metastases at
           inhibin, and CD1a. Many lesional cells were strongly   multiple  sites. She developed  abdominal  distension
           positive for  CD163, with less distinct staining for   and  multi-organ  failure,  and  passed  away  7 weeks
           CD68  [Figure 4].  Her bone marrow biopsy showed   following initial diagnosis.
           a mildly hypercellular marrow for age, with trilineage
           hematopoiesis,  a left shift of the granulocytes,  a   Autopsy findings
           moderate erythroid hyperplasia,  and plasmacytosis.   At autopsy, there was single large (20 cm × 17 cm ×
           No morphologic  or immunophenotypic  evidence of   9 cm) tumor arising from the left lobe of the liver, with
           malignancy  was  identified.  A  diagnosis  of  HS  was   extensive necrosis. No evidence of metastatic tumor
           made based on the combination of  morphology  and   was  identified  at  any  of  the  other  suspected  sites.
           immunophenotype, and this diagnosis was supported   Microscopic examination of the tumor was complicated
           by outside expert consultation.                    by moderate autolysis with extensive necrosis, but
                                                              otherwise  showed similar  morphology  to the original
           Clinical course                                    biopsies.
           Because of the large size of this tumor and the non-
           availability of liver donor at that time, complete tumor   DISCUSSION
           resection and liver transplantation were not performed.
           The patient received two rounds of  chemotherapy   HS is a malignant  proliferation  of cells showing
           (1st round ifosfamide and doxorubicin, and 2nd round   morphologic  and immunophenotypic  features of
           thalidomide)  and intensive supportive  measures.   mature tissue histiocytes.  In 1970, “histiocytic
                                                                                       [7]
           However, she  gradually  developed  worsening  multi-  sarcoma” was introduced for the first time to describe
           organ  failure  requiring  mechanical  ventilation  and   a collection  of malignant  tumor cells with histologic
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