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Case Report




          Combined sarcomatoid hepatocellular and
          Combined sarcomatoid hepatocellular and
          cholangiocarcinoma: a case report and literature review
          cholangiocarcinoma: a case report and literature review


          Julian Shun Tsang , Kenneth Siu Ho Chok , Rex Kwok Him Au-Yeung , Chung Mau Lo                 1
                                                        1
                                                                                       2
                               1
          1 Department of Surgery, Queen Mary Hospital, The University of Hong Kong, Hong Kong, China
          2 Department of Pathology, Queen Mary Hospital, The University of Hong Kong, Hong Kong, China


               ABSTRACT
               Hepatic sarcomatoid carcinomas are very rare. The majority of cases contain sarcomatoid features with either hepatocellular
               carcinoma (HCC) or cholangiocarcinoma (CC) elements alone. These are aggressive tumors and carry an unfavorable
               prognosis. We describe an extremely rare tumor sub-type of combined sarcomatoid HCC and CC in a hepatitis B virus carrier
               presenting with abdominal pain. Pre-operative imaging suggested a segment VI hepatocellular cancer with no metastatic
               spread. En bloc surgical resection with the right adrenal gland, Gerota’s fascia and right hemidiaphragm was performed. The
               patient suffered early peritoneal tumor recurrence and lymph node metastasis. Pre-operative diagnosis of such sarcomatoid
               tumors is diffi cult. Current evidence for adjuvant treatment is also limited. Prognosis of these patients remains extremely
               poor, and surgery appears to be the only curative option in cases of early disease. It is essential that clinicians carry a high
               index of suspicion and awareness of this rare pathological entity to improve patient outcome.

               Key words: Cholangiocarcinoma; hepatocellular carcinoma; sarcomatoid; spindle cell sarcoma


          Address for correspondence:
          Dr. Kenneth Siu Ho Chok, Division of Hepatobiliary and Pancreatic Surgery and Liver Transplantation, Department of Surgery, Queen Mary
          Hospital, The University of Hong Kong, Hong Kong, China. E-mail: kennethchok@gmail.com
          Received: 18-01-2015, Accepted: 13-03-2015


          INTRODUCTION                                        A sarcomatous tumor with both HCC and CC components is
                                                              extremely rare. These tumors have an aggressive behavior and
          Hepatic sarcomatoid carcinomas are very rare. The reported   are associated with poor prognosis. Management options are
          incidence is < 10% with most cases only identified at   currently unclear and limited. We report a case of combined
          autopsy. Diagnosis after surgical resection has been found   sarcomatoid HCC and CC with a literature review of reported
          in 1.8% of cases.  Most patients are reported as individual   cases based on their characteristics and treatment options.
                        [1]
          case reports or small series only. There is a confusing and
          varied pathological terminology including carcinosarcoma,   CASE REPORT
          sarcomatoid carcinoma and spindle cell carcinoma, [1-3]  all likely
          to describe the same pathology. The majority of cases contain   A 69-year-old Asian man presented with right-sided
          sarcomatoid features together with either hepatocellular   abdominal pain. He was a known hepatitis B virus (HBV)
          carcinoma (HCC) or cholangiocarcinoma (CC) elements alone.   carrier but otherwise had no significant past medical
                                                              history. He had no regular follow-up and was not on
                                                              anti-viral therapy. Physical examination was unremarkable.
                          Access this article online
                                             Quick Response Code  Laboratory investigations were consistent with Child’s A
           Website:                                           liver cirrhosis. Liver function tests were normal apart from a
           http://www.hrjournal.net/                          raised alkaline phosphatase level at 168 μmol/L. Alpha-feto
                                                              protein (AFP) level was raised to 21 ng/mL. Antibody to

           DOI:                                               hepatitis e antigen (HBeAg) was positive whereas HBeAg
           10.4103/2394-5079.154331                           and anti-hepatitis C virus were negative. HBV DNA level
                                                              was 178 IU/mL. Computed tomography (CT) identified a



               Hepatoma Research | Volume 1 | Issue 1 | April 15, 2015                                       41
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