|LETTER TO THE EDITOR
|Year : 2015 | Volume
| Issue : 2 | Page : 493-494
Fibrolamellar hepatocellular carcinoma with extensive vascular thrombosis
Monica Bhagat1, Seema Kembhavi2, Sajid S Qureshi1
1 Department of Pediatric Surgical Oncology, Tata Memorial Centre, Mumbai, Maharashtra, India
2 Department of Radiodiagnosis, Tata Memorial Centre, Mumbai, Maharashtra, India
|Date of Web Publication||7-Jul-2015|
Sajid S Qureshi
Division of Pediatric Surgical Oncology, Department of Surgical Oncology, Tata Memorial Hospital, Ernest Borges Road, Parel, Mumbai - 400 012, Maharashtra
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Bhagat M, Kembhavi S, Qureshi SS. Fibrolamellar hepatocellular carcinoma with extensive vascular thrombosis. J Can Res Ther 2015;11:493-4
A 11-year-old boy presented with progressive jaundice of two months duration. There was history of passing high colored urine and clay colored stools and negative history of fever, vomiting, weight loss or blood transfusion. The child was deeply icteric and had enlarged irregular liver mass palpable 8 cm below the right costal margin. Laboratory investigations revealed anemia (Hb - 6.5 gm%), deranged liver function (total bilirubin of 28.7 mg/dl; normal <1 mg/dl, serum albumin level of 2.4 mg/dl; normal >3.5 mg/dl) and deranged coagulation profile. The tumor markers (alpha fetoproteins - 6.5 ng/ml) were normal.
An ultrasonography (USG) suggested a large-sized mixed echogenic mass in right and caudate lobe causing obstruction at porta with associated intrahepatic biliary dilatation and features of portal hypertension. A contrast enhanced computerized tomography (CT) revealed a large irregular heterogeneous mass (10 Χ 12 Χ 12 cms) involving segment V, VI, VII, VIII and IV with a large caudate lobe lesion [Figure 1]a. There was a thrombus in the main portal vein, which was extending to the superior mesenteric vein [Figure 1]b. The lesion was encasing the common bile duct at the porta and multiple retroperitoneal and periportal nodes were present. A CT guided biopsy was performed, which revealed a fibrolamellar hepatocellular carcinoma (FLC) [Figure 2]. In view of the extensive disease, limited treatment options, overall poor prognosis and financial constraints, the parents did not pursue further treatment.
|Figure 1: (a) Lesion in right lobe with caudate lobe involvement (arrow), (b) Large superior mesenteric vein thrombus (shown by arrow)|
Click here to view
|Figure 2: (a) Large polygonal hepatocytes with eosinophilic granular cytoplasm. The nucleus shows prominent intranuclear inclusion (arrow), (b) Immunohistochemistry (IHC) with anti CK7 antibody is positive in the tumor cells|
Click here to view
FLC is a variant of Hepatocellular carcinoma (HCC) that occurs in non-cirrhotic livers with a more favorable prognosis than the usual HCC. Jaundice is rare in FLC and was present in our patient due to the cumulative effect of large mass, involvement of common bile duct (CBD) and lymphadenopathy resulting in biliary obstruction.  Radiological feature of central scar, which is characteristic of FLC was not seen in our patient.  There have been occasional reports on thrombus in portal vein in FLC; however, there are no reports of tumor thrombus extending into the superior mesenteric vein, which is described as Type IV according to Shi et al., tumor thrombus classification for the HCC.  Consequently, our patient also had features suggestive of portal hypertension. The limited therapeutic options along with the deranged liver enzyme and coagulopathy and poor general condition were the hindrance for treatment compounded by the reluctance from the parents.
| > References|| |
Soyer P, Roche A, Levesque M. Fibrolamellar hepatocellular carcinoma presenting with obstructive jaundice. A report of two cases. Eur J Radiol 1991;13:196-8.
Ichikawa T, Federle MP, Grazioli L, Madariaga J, Nalesnik M, Marsh W, et al
. Fibrolamellar hepatocellular carcinoma: Imaging and pathologic findings in 31 recent cases. Radiology 1999;213:352-61.
Shi J, Lai EC, Li N, Guo WX, Xue J, Lau WY, et al
. Surgical treatment of hepatocellular carcinoma with portal vein tumor thrombus. Ann Surg Oncol 2010;17:2073-80.
[Figure 1], [Figure 2]