Aggressive primary hepatic histiocytic sarcoma: case report and literature review

摘要

Histiocytic sarcoma is an uncommon non-Langerhans histiocyte disorder of mature tissue histiocytes.The authors presented an example of this rare tumor in a 14-year-old girl who presented with left upper quadrant pain,loss of appetite,and weight loss.A large 18 cm×10 cm heterogeneous solid and cystic enhancing mass was found in the left lobe of the liver.Based on the histomorphology and positivity for histiocyte-specific markers in a needle biopsy,a diagnosis of histiocytic sarcoma was made.Chemotherapy was initiated,but the tumor did not respond well,and she died about 7 weeks following initial diagnosis with multi-organ failure.At autopsy,the tumor showed extensive necrosis,with no evidence of metastatic spread.In conclusion,the diagnosis of histiocytic sarcoma is challenging,and requires a high index of suspicion,with an appropriate panel of confirmatory immunohistochemical stains.Recognition of this rare tumor is important because of its poor response to chemotherapy and high mortality.