Aggressive primary hepatic histiocytic sarcoma: case report and literature review

Abstract

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.

Keywords

Histiocytic sarcoma / hepatic neoplasms / liver

References

[1] Takahashi E.Histiocytic sarcoma: an updated literature review based on the 2008 WHO classification..J Clin Exp Hematop2013;53:1-8

[2] Hornick JL,Fletcher CD.Extranodal histi ocytic sarcoma: clinicopathologic analysis of 14 cases of a rare epithelioid malignancy..Am J Surg Pathol2004;28:1133-44

[3] Mounajjed T,Torbenson MS.Surgical Pathology of Liver Tumors..2015;Springer360-1

[4] Llamas-Velasco M,Dominguez I,Aragües M,Arranz R.Coexistence of Langerhans cell histiocytosis, Rosai-Dorfman disease and splenic lymphoma with fatal outcome after rapid development of histiocytic sarcoma of the liver..J Cutan Pathol2012;39:1125-30

[5] Rubtsova NA,Petrov LO,Vernyuk MA.Diagnosis and treatment of primary sarcoma of the liver (case review)..Radiology2014;1:43

[6] Chun HJ,Suh CH,Song HS.Primary malignant lymphoma of true histiocytic origin of the liver: histiocytic sarcoma, kupffer cell sarcoma: a case report with immunohistochemical and ultrastructural studies..Korean J Pathol1989;23:165-80

[7] Swerdllow SH,Harris NL.WHO classification of tumours of haematopoietic and lymphoid tissues..2008;FranceIARC Press356-7

[8] Mathé G,Texier JL,Berumen L.The two varieties of lymphoid tissue "reticulosarcomas", histiocytic and histioblastic types..Br J Cancer1970;24:687-95 PMCID:PMC2008717

[9] Pakravan A,Oshima K,Fesler M,Taylor JR.Histiocytic sarcoma: the first reported case of primary esophageal involvement..Am J Gastroenterol2014;109:291-2

[10] Dalia S,Sagatys E,Sokol L.Dendritic cell and histiocytic neoplasms: biology, diagnosis, and treatment..Cancer Control2014;21:290-300

[11] Ohnishi K,Oghiso Y.Immunohistochemical detection of possible cellular origin of hepatic histiocytic sarcoma in mice..J Clin Exp Hematop2012;52:171-7

[12] Lindberg MR.Diagnostic Pathology: Soft Tissue Tumors..2015;Elsevier Health Sciences310-1

[13] Vasef MA.Diagnostic Pathology: Molecular Oncology..2016;Elsevier Health Sciences2-4