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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.
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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