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Table 1: Case reports of combined sarcomatoid hepatocellular carcinoma and cholangiocarcinoma
Study Age, gender Symptoms Hepatitis serology Treatment Follow-up and prognosis
Nakajima et al. [4] 74 years, male Right upper quadrant pain HBsAb TACE Lung metastasis; death at 17 months;
diagnosis at autopsy
Papotti et al. [5] 59 years, male Lumbar pain Negative Hepatectomy Biliary fi stula; death at 4 months
Jeong et al. [6] 60 years, female Right upper quadrant pain/mass NA Hepatectomy Tumor recurrence; death at 12 months
Kim et al. [7] 67 years, male Synchronous colon tumor NA NA NA
Boonsakan et al. [8] 28 years, male Fever HBsAg Palliative resection Lost to follow-up
Pua et al. [9] 71 years, male Fever, weight loss, anorexia Negative Hepatectomy Lung and pleural metastasis; death
at 6 weeks
NA: not available; HBsAb: hepatitis B surface antibody; HBsAg: hepatitis B surface antigen; TACE: trans-arterial chemoembolization

ordinary HCC with positive hepatitis serology, cirrhosis and were divided according to the percentage of CC. Sarcomatous
raised AFP levels. Liver function parameters may or may not patients were found to have significantly higher chance of
be unstable depending on the size of the tumor and degree of vascular invasion, lymph node metastasis as well as high
underlying cirrhosis. The liver was grossly cirrhotic [Figure 4] CC percentage group, and were associated with a much
in our patient; however, his child’s score was only Class A. This shorter survival compared to those without a sarcomatous
might be attributed to his relatively large liver volume reserve and lower CC component (P < 0.0048). Such aggressive
compensating for cirrhosis. Some authors have reported lesions make the diagnosis and treatment difficult. Patients
high fever and abdominal pain as frequent symptoms of are either diagnosed only at post-mortem or at an advanced
sarcomatoid HCC. [13,16] Although these were not present in unresectable stage. There is currently no recommended
this patient, the suspicion of liver abscess based on these treatment strategy for sarcomatoid carcinomas, and surgical
symptoms may delay and make the diagnosis more difficult. resection appears to be the only curative option, albeit only
In a patient described by Inoue et al., abscess drainage at the early stage of tumor formation. There is yet no report
[13]
followed by biopsy were performed before the diagnosis was of successful adjuvant treatment with chemotherapy or
made. At 3 weeks after admission, there was already pleural radiotherapy described in the literature to prolong survival
metastasis. Despite a trial of chemotherapy, the patient died in patients with this aggressive tumor.
of multiorgan failure on day 27. Radiologically, our patient’s
tumor was also suggestive of HCC with arterial enhancement In conclusion, a case of hepatic combined sarcomatoid HCC
and portovenous contrast washout. Honda et al. described and CC is reported here. This is an extremely rare tumor with
[17]
delayed or prolonged contrast enhancement as a feature an aggressive behavior. The prognosis is extremely poor, and
of sarcomatoid HCC. This finding may be attributed to the survival has been limited to within a few months in most
presence of active cancerous tissue with fibrous stroma. cases. Pre-operative diagnosis and distinction from ordinary
The aggressiveness of this type of tumor is highlighted by HCC is difficult. Reports in the literature suggest surgery as
the high incidence of local invasion and metastatic spread the treatment of choice at present. Due to the rarity of the
either at the time of surgery or early recurrence. Indeed, disease, adjuvant chemotherapy or radiotherapy has not
there was invasion into the right hemidiaphragm of > 50% shown any promise. Early diagnosis with a high index of
in our patient. Both intra and extrahepatic metastasis as well suspicion appears to give the best chance of cure.
as lymphadenopathy is common. Metastasis to multiple
[16]
organs including the lungs, hepatic hilar lymph nodes, greater
omentum, stomach and diaphragm as seen in our patient REFERENCES
have been reported. [4]
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Maeda T, Adachi E, Kajiyama K, Takenaka K, Sugimachi K,
survival rarely exceeded 12 months. This is exemplified Tsuneyoshi M. Spindle cell hepatocellular carcinoma. A clinicopathologic
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and peritoneal nodules were already identified 1-month 4. Nakajima T, Kubosawa H, Kondo Y, Konno A, Iwama S. Combined
post-surgical resection. In a study reported by Aishima hepatocellular-cholangiocarcinoma with variable sarcomatous
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[18]
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44 Hepatoma Research | Volume 1 | Issue 1 | April 15, 2015