Repeated hepatic resections and radio-frequency ablations may improve the survival of adult undifferentiated embryonal sarcoma of the liver: report of two cases

A 52-year-old female was admitted to the hospital to treat a huge liver tumor with complaints of dyspnea, severe abdominal distension, and lower-extremity edema. Hepatitis B virus surface antigen (HBs-Ag) and hepatitis C virus antibody (HCV-Ab) were negative. The serum level of DUPAN-II was 810 U/ml and that of CA125 was 157 IU/ml, and alpha-fetoprotein (AFP), protein induced by vitamin K absence (PIVKA-II), carcinoembryonic antigen (CEA), and carbohydrate antigen 19–9 (CA19-9) were in the normal range. Computed tomography (CT) showed a huge low-density tumor with a clear margin, 23 cm in diameter, and showed little enhancement after administration of contrast medium (Fig. 1a). Magnetic resonance (MR) imaging demonstrated the multicystic tumor to be hypointense on T1-weighted images (Fig. 1b) and hyperintense on T2-weighted images (Fig. 1c). A right tri-sectionectomy as the first operation was performed safely and successfully, even though the tumor entirely compressed the inferior vena cava and the root of three hepatic veins. Operation time was 13 h and intraoperative bleeding was 2400 g. Resected specimen showed a heterogenic tumor sized 23 × 22 × 11 cm (Fig. 1d). Histologically, spindled, oval, or stellate tumor cells were distributed in myxoid or fibrous stroma. Multiple varying-sized cytoplasmic eosinophilic globules were seen (Fig. 2a, b). Immunohistologically, tumor cells were positive for vimentin (Fig. 2c), alpha 1-antitrypsin (Fig. 2d), and alpha 1-antichymotrypsin (Fig. 2e), and partially positive for alpha-smooth muscle actin (SMA) and CD68/kp-1. S-100, calponin, cytokeratin, factor VIII, CD34, and AFP were negative in tumor cells. The MIB-1 index was 20 % (Fig. 2f). Finally, the tumor was histologically diagnosed as a UESL, and the liver parenchyma was normal liver. Twelve and 19 months after the first operation, for the sequential recurrent diseases, a left caudate lobectomy and a partial hepatectomy of segment 3 were performed as the second and the third surgery, respectively. Twenty months after the first operation, twice transarterial chemoembolizations (TACEs) with CDDP 50 mg + lipiodol 2.5 ml, epirubicin 30 mg, and gelatin sponge were performed. Twenty-five months after the first operation, a radio-frequency ablation (RFA) was performed (pre, Fig. 3a, c; post, Fig. 3b, d). Thirty-five, 42, and 47 months after the first operation, two times of partial hepatic resections and a hepatectomy in combination with RFA (pre, Fig. 3e; post, Fig. 3f) were additionally performed. Because of bone metastases and tumor thrombosis in azygos vein, she unfortunately died 62 months after the initial hepatectomy.

A 53-year-old female was admitted to our hospital with a huge liver tumor with symptoms of epigastralgia and back pain. Her HBs-Ag and HCV-Ab were negative. Serum level of PIVKA-II was elevated to 10,369 mAU/ml, and AFP, CEA, and CA19-9 were in the normal range. CT showed a huge low-density tumor sized 18 × 14 cm that showed mild enhancement after administration of contrast medium (Fig. 4a). MR imaging demonstrated the heterogeneous tumor to be hypointense on T1-weighted images (Fig. 4b) and hyperintense on T2-weighted images (Fig. 4c). Some satellite lesions suspected as cavernous hemangiomas were identified in the bilateral lobe of the liver. The first extended left hepatectomy was performed. Operation time was 459 min and intraoperative bleeding was 877 g. Resected specimen revealed a tumor with multiple components, sized 22 × 19 × 14 cm (Fig. 4d). Histologically, spindled, oval, or stellate tumor cells were distributed in myxoid or fibrous stroma. Nuclear pleomorphism and hyperchromasia with frequent multinucleated or bizarre giant cells were apparent. Multiple varying-sized cytoplasmic eosinophilic globules were seen (Fig. 5a, b). The tumor cells were positive for vimentin (Fig. 5c), alpha 1-antitrypsin (Fig. 5d), alpha 1-antichymotrypsin (Fig. 5e), and desmin, and partially positive for CD34 and alpha-SMA. The MIB-1 index was 30 % (Fig. 5f). Hepatocyte specific antigen (HSA) and cytokeratin were negative in the tumor. The tumor was histologically diagnosed as a UESL in the normal liver. Three months after the first operation, a TACE (CDDP 80 mg + lipiodol 4 ml, 5-FU 1000 mg, and gelatin sponge) was performed. Four and 14 months after the first operation, a partial hepatic resection of segments 6 and 7 and a partial hepatic resection of segment 8 in combination with RFA (pre, Fig. 6a; post, Fig. 6b) were also performed, respectively. She is now alive without any recurrent diseases for more than 65 months from the initial hepatic resection.