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Yang et al. Hepatoma Res 2016;2:300-4 Hepatoma Research
DOI: 10.20517/2394-5079.2016.10
www.hrjournal.net
Case Report Open Access

Aggressive primary hepatic histiocytic

sarcoma: case report and literature review

Guang Yang , Jeremy Deisch , Haixia Qin , Craig Zuppan , Anwar S. Raza 1
1
2
1
1
1 Department of Pathology, Loma Linda University Medical Center, Loma Linda, CA 92354, USA
2 Department of Medicine, Loma Linda University Medical Center, Loma Linda, CA 92354, USA
Correspondence to: Dr. Anwar S. Raza, Department of Pathology, Loma Linda University Medical Center, Loma Linda, CA 92354, USA.
E-mail: ARaza@llu.edu
How to cite this article: Yang G, Deisch J, Qin H, Zuppan C, Raza AS. Aggressive primary hepatic histiocytic sarcoma: case report and literature
review. Hepatoma Res 2016;2:300-4.
ABSTRACT
Article history: Histiocytic sarcoma is an uncommon non-Langerhans histiocyte disorder of mature tissue
Received: 07-04-2016 histiocytes. The authors presented an example of this rare tumor in a 14-year-old girl who
Accepted: 14-10-2016 presented with left upper quadrant pain, loss of appetite, and weight loss. A large 18 cm × 10 cm
Published: 07-11-2016 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
Key words: of histiocytic sarcoma was made. Chemotherapy was initiated, but the tumor did not respond
Histiocytic sarcoma, well, and she died about 7 weeks following initial diagnosis with multi-organ failure. At autopsy,
hepatic neoplasms, the tumor showed extensive necrosis, with no evidence of metastatic spread. In conclusion, the
liver 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.

INTRODUCTION large B-cell lymphoma and anaplastic large cell
lymphoma. [2]
Histiocytic sarcoma (HS) is an extremely uncommon
neoplasm with morphologic and immunophenotypic HS is an extremely aggressive neoplasm and
characteristics of mature histiocytes. It is believed responds poorly to regular therapy; most patients die of
that HS originates from monocytes/macrophages, progressive disease, in part related to the high clinical
which are critical in the processing and presentation of
antigens to T cells or B cells. [1] stage (stage III/IV) at presentation in the majority of
patients. The most common affected site of HS is in
[3]
Before the application of immunohistochemical lymph nodes, followed by different extranodal locations
techniques and the availability of molecular genetic such as the gastrointestinal tract, spleen, soft tissue
tools, HS was occasionally diagnosed. It is now and skin.
generally recognized however that the majority of the
previously diagnosed HS cases are actually examples Herein, we describe a child with an aggressive primary
of non-Hodgkin lymphomas, most of which are diffuse hepatic histiocytic sarcoma who responded poorly to
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