Robust Effect of Hepatic Arterial Infusion Chemotherapy and Radiation Therapy on Hepatocellular Carcinoma Arising from Fontan-associated Liver Disease.

Fontan-associated liver disease (FALD) caused by long-term systemic venous congestion following the Fontan procedure may eventually lead to hepatocellular carcinoma (HCC). Treatment strategies for HCC due to FALD (FALD-HCC) remain unclear. We herein report a 35-year-old man with FALD-HCC that was well controlled by 3 cycles of continuous infusion of 5-fluorouracil and low-dose cisplatin (low-dose FP therapy) combined with 60 Gy of radiation therapy. However, the patient ultimately died of extrahepatic metastases. A pathological autopsy revealed more than 90% necrosis in the primary HCC lesion. This case suggests that low-dose FP therapy might be effective in FALD-HCC.

Usefulness of Tumor Tissue Biopsy for Predicting the Biological Behavior of Hepatocellular Carcinoma.

BACKGROUND/AIM: Assessment of the biological behavior of tumors is important for choosing an appropriate cancer therapy. In hepatocellular carcinoma (HCC), the biological behaviour can be assessed by tumor morphology and molecular biology. This study investigated the usefulness of tumor tissue biopsy for predicting the biological behavior of HCC. PATIENTS AND METHODS: We studied 43 patients who underwent hepatectomy and preoperative liver tumor biopsy for HCC. We performed clinicopathological and immunohistochemical (IHC) analyses. The expression of the following molecules was examined: regulator of G-protein signaling 5 (RGS5), glypican-3 (GPC3), keratin 19 (K19), epithelial cell adhesion molecule (EpCAM), protein induced by vitamin K absence or antagonist-II (PIVKA-II), ß-Catenin, and p53. RESULTS: There was an overall 83.7% agreement regarding tumor differentiation between the preoperative biopsy specimens and the resected specimens. The accuracy of IHC analysis was more than 70% for all molecules between the preoperative biopsy specimens and the resected specimens. The RGS5-positive biopsy cases had higher serum α-fetoprotein levels (p=0.04), a higher rate of moderately or poorly differentiated tumors (p=0.02) and portal vein invasion (p=0.0003) than the RGS5-negative biopsy cases. The GPC3-positive biopsy cases were younger (p=0.04), had higher serum PIVKA-II levels (p=0.01), and a higher rate of portal vein invasion (p=0.03) than the GPC3-negative biopsy cases. The PIVKA-II-positive biopsy cases had significantly higher serum PIVKA-II levels than the PIVKA-II-negative biopsy cases (p=0.02). The other molecular markers showed no significantly different clinical findings between the positive and negative cases. CONCLUSION: In HCC, there was a high agreement rate of both the histopathological and IHC findings between preoperative biopsy specimens and resected specimens. In the biopsy specimens of HCC, RGS5 and GPC3 expression were useful molecular makers for predicting portal vein invasion. Liver tumor biopsy is useful for predicting the biological behavior of HCC through histopathological and immunohistochemical findings.

Polypoid gallbladder neuroendocrine tumor diagnosed as benign polyp before surgery: A case report.

Gallbladder neuroendocrine tumors (GB-NETs) comprise only 0.5% of all NET cases, and their biology has been incompletely characterized. In the present study we report the case of a 50-year-old male patient with GB-NET who was admitted to Naito Hospital with diarrhea as the main complaint. At initial diagnosis, serum carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9 (CA19-9) levels were within the normal range. Abdominal ultrasonography and contrast-enhanced computed tomography (CT) revealed gallbladder adenomyomatosis and cholecystitis, and an 8-mm pedunculated polypoid lesion was found in the neck of the gallbladder using drip infusion cholecystocholangiography-CT. As it was considered a benign polyp, laparoscopic cholecystectomy was performed. Pathological examination revealed a polypoid lesion that comprised NET cells with a cord-like or ribbon-like arrangement, and the cells exhibited positive immunostaining for chromogranin A and synaptophysin. In addition, immunohistochemical staining showed a Ki-67 index (i.e., proliferation index) of <1%, and no necrosis or mitotic figures were observed in the background. Based on these observations, we diagnosed the following: GB-NET, G1, 10x12 mm in size and located in the gallbladder neck. According to the World Health Organization 2010 classification, NET G1 is a well-differentiated tumor, with the tumor cells having a low proliferative potential [Ki-67 index ≤2%; mitotic figure number <2 (/10 HPF)]. It is regarded as a low- to mild-grade malignancy. Low-grade GB-NET occurs relatively rarely, and no clear guidelines have been formulated regarding its surgical treatment, such as minimal surgical excision margins or lymph node dissection. Detailed treatment recommendations should be developed after systematic studies of additional cases of GB-NET.