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,...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.展开更多
文摘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.
