Our experience in surgical resection of hepatoblastoma

Poster Session Conclusion: Preoperative Y 90 radioembolization may be an optional procedure to provide an adequate FLR in patients with extensive liver malignancies. P13.02 EXTENDED RIGHT HEPATECTOMY AND VCI RESECTION TO ACHIEVE R0 RESECTION OF A VCI LEIOMYOSARCOMA C. Bilgic1, C. Indelen2, S. Ates2, A. Armutlu3 and M. Erkan1 1 Koç University School of Medicine, Surgery, 2Koç University School of Medicine, Cardiovascular Surgery, and 3 Koç University School of Medicine, Pathology, Istanbul, Turkey Background: Leiomyosarcoma (LMS) of the vena cava inferior (VCI) is an extremely rare disease. The only curative treatment is achieved by R0 resection of the tumor. Methods: Here we present a case of a 10x8x8cm LMS of the VCI that was successfully R0 resected with extended right hepatectomy and enbloc VCI resection. Preoperative right portal vein embolization was performed to enlarge the future liver remnant. Complete VCI resection was performed transdiaphragmatically with clamping of the right atrium. The reconstruction was made with reimplantation of the left hepatic vein, two renal veins and right iliac vein on a 20mm goretex graft. Biliary reconstruction was made with Roux-Y cholangiojejunostomy with individual anastomosis of the Segment 2 and 3 bile ducts. Results: Postoperative course was eventless and the patient is tumor free 6 months postoperatively. Conclusion: R0 resection of VCI LMS can be achieved in tertiary HPB centers with capacity of multidisciplinary approach. P13.03 SCHWANNOMA, IVC LEIOMYOSARCOMA, AND IPMN OF HETEROTOPIC PANCREATIC TISSUE. A. Zanellato, R. Thompson, I. Reccia, M. Pai and N. Habib Imperial College of London, Surgery and Cancer, London, United Kingdom Background: We report a unique first case in which one patient presented with of all these rare conditions, discussing presentation, diagnosis and management. Methods: A 73 year old lady presented complaining of epigastric pain. Abdominal ultrasound revealed a cystic mass arising from within the mesentery. Subsequent CT / MRI confirmed the presence of a well circumscribed, uniformly enhancing, low density mesenteric mass measuring 5 x 5 x 6.7 cm and lying adjacent to the duodenum. Tumour makers were within the normal range. Appearances were thought to be consistent with a benign, cystic lesion, but annual follow-up with CT was recommended. During the second year of follow-up the mass grew to 7.5 x 5.5 x 5.5 cm. Additionally, a enlarged aorto-caval lymph node. Both lesions demonstrated increased uptake of F18-FDG on CT PET, malignancy was thought to be likely and the patient proceeded to surgery. At operation, the mesenteric mass HPB 2019, 21 (S3), S783eS923 S811 was resected uneventfully. The aorto-caval mass excision required resection of a sleeve of anterior wall of IVC. Incidentally, an indeterminate mass was noted in the jejunum, and was excised. Results: Histopatolgy: a mesenteric schwannoma; a leiomyosarcoma of the inferior vena cava; and intraductal papillary mucinous neoplasma, with high-grade dysplasia, arising in heterotopic jejunal pancreatic tissue. Conclusion: Although these conditions are usually low symptomatic and is often discovered incidentally, surgical management is the only way to confirm diagnosis, manage and curatively treat them. P13.04 SOLITARY FIBROUS TUMORS OF HEPATOPANCREATOBILIARY SYSTEM; 2 CASE REPORTS Y. Türk, A. U guz, O. V. Unalp and A. Coker Ege University School of Medicine, General Surgery, Izmir, Turkey Background: A solitary fibrous tumor (SFT) of the hepatopancreatobiliary(HPB) system is a rare neoplasm of mesenchymal origin. SFTs are an uncommon neoplasms of mesenchymal origin that primarily affects the pleura and mediastinum and may occur elsewhere in the body including respiratory tract, pancreas ,liver, peritoneum, kidney and salivary glands, breast. Methods: Case 1:A 56-year-old male was admitted to the Department of General Surgery with upper abdomen pain. On computed tomography, there was a 11.7x7.8x9.5cm mass that appeared to arise from the head of the pancreas. At endosonographic biopsy ; A mesenchymal tumor with atypical and indefinite proliferative activity was observed with Suspicious of SFT. A pancreaticoduodenectomy was performed. Results: Case 2:A 43-year-old female presented with right upper abdominal pain. There was no history of vomiting, jaundice or fever.Patient referred a HPB surgeon for surgery. Liver perfusion MRI showed that the presence of a giant hypervascular 9x8cm mass lesion filling the segment 4. A central hepatectomy was performed. Conclusion: Because of, SFT of HPB system is an uncommon neoplasm and has non-spesific clinical presentation , diagnosing SFT is challenging. Also It’s difficult to distinguish radiologically from other HPB lesions. Fine needle aspiration cytology is helpful but histologic examination and immunohistochemistry were necessary to make the diagnosis of SFT. The treatment of extra-pleural SFT is complete surgical resection with negative border as it recommended. P13.05 OUR EXPERIENCE IN SURGICAL RESECTION OF HEPATOBLASTOMA J. -R. Oliver1, M. Serradilla1, S. Genzor1, A. Palomares1, L. Serrablo2 and A. Serrablo1 1 Miguel Servet University Hospital, Division of HepatoPancreato-Biliary Surgery, and 2Zaragoza University, Zaragoza, Spain S812 Poster Session Background: Hepatoblastoma (HB) is the most common malignant liver tumour in children; it accounts for 50% of liver tumours and 1.3% of malignant tumours in childhood. Surgical resection remains the cornerstone of curative therapy. Methods: Retrospective review of patients diagnosed of HB in the last 15 years in our centre and review of literature. Results: 5 patients were identified. Male: 100%. Mean age: 3.15 years-old. Low birth weight: 0. Tumour biopsy: 100%. a -fetoprotein ( a FP) < 100 ng/mL: 20%; the same patient was initially diagnosed of metastases (lungs, bilateral) and finally died. Chemotherapy regimens: SIOPEL 3 (PLADO): 60%, SIOPEL 4: 20%, SIOPEL 6: 20%. Mean time diagnostic-surgery: 96.26 days. Staging CT post neoadjuvant chemotherapy: 100%. Types of surgery performed: right hepatectomy 60%, left hepatectomy 20%, transverse hepatectomy 20%. Postoperative complications: Clavien-Dindo grade II: 80%, grade III: 20%. Free surgical margins: 80%. Histology: epithelial 60%; mixed epithelialsarcomatous 20%; mixed epithelial-mesenchymal 20%. Portal, suprahepatic or extrahepatic intraabdominal disease were not observed. Mean disease free survival: 9.19 years. Mortality: 20%. Conclusion: HB is the most frequent malignant liver tumour in children. Low a FP (< 100 ng/mL) and the presence of metastases initially diagnosed worsen prognosis. Neoadjuvant chemotherapy may lead to downstage tumour size and ease surgery, even though surgery is the only curative therapy. P13.06 ROLE OF A.L.P.P.S. IN TREATMENT OF PERIHILAR CHOLANGIOCARCINOMA: EXPERIENCE OF SINGLE CENTRE F. Mocchegiani1, R. Montalti2 and M. Vivarelli1 1 Polytechnic University of Marche, Department of Experimental and Clinical Medicine, and 2United Hospitals of Ancona, Department of Gastroenterology and Transplants, Ancona, Italy Background: We reported results of a mono-centric experience in the treatment of perihilar cholangiocarcinoma (PHCC) by Associated liver partition and portal vein ligation for staged hepatectomy (ALPPS). Methods: Since January 2013, 16 patients with primary or secondary tumors of the liver and biliary tract, whose future liver remnant (FLR) was considered too small to allow for a liver resection, were evaluated prospectively for ALPPS procedure. Procedure was completed in 13 patients. The indication for surgical resection was PHCC in 4 cases (30.8%). Results: The increase of the FLR between the two procedures was 80  31% (p <0.001). By comparing the results of 4 patients underwent ALPPS for PHCC than the other 9 (No PHCC) emerge the following data: the average time between the first and second phase of the procedure was 8  2 ( PHCC) vs 11.7  2 days (No PHCC); The overall postoperative mortality was 7.69% with only 1 death from liver failure in a patient with PHCC and concomitant liver fibrosis, 25% (PHCC) vs 0% (No PHCC); The overall postoperative morbidity rate was 75% in PHCC patients vs 77.7% (No PHCC). Overall survival was 75% at 6, 12, 24, 30 months (PHCC) vs 100% in 6-12 months, 85% at 12, 24, 30 months (No PHCC). The recurrence-free survival was 100% in 6-12, 24, 30 months (PHCC) vs 100% at 6 months, 87.5% at 12 months, 75% at 24, 30 months (No PHCC). Conclusion: The ALPPS technique has effectively increased the resection of liver tumors otherwise inoperable as PHCC. P14.01 PROGNOSTIC IMPACT OF OPN AND DKK1 IN PATIENT OF HEPATOCELLULAR CARCINOMA AFTER HEPATECTOMY Y. Seo, H. R. Byeon and S. D. Lee National Cancer Center, Liver Cancer Center, Goyang-si, Gyeonggi-do, South Korea Background: The new biomarkers are essential for improving the survival and prognosis of hepatocellular carcinoma(HCC) patients. Alpha-fetoprotein(AFP) is the most widely used biomarker. But the low sensitivity and specificity limits its clinical application. Recent study assessed the combination of AFP, DKK1, and OPN as a panel for the diagnosis of HCC. Based on these previous studies, we hypothesized that combination of OPN and DKK1 can be used to as a marker for prognosis to patient of HCC after hepatectomy. Methods: From January 2006 to December 2008, patients undergoing hepatectomy for HCC were screened serum that has been stored in the Bank of tumor. To target the selected patient, it was confirmed that the remaining tissue specimen is stored after diagnosis. Serum of the patients was used to investigate the OPN and DKK1 by ELISA. In the paraffin block were prepared unstained slide and OPN and DKK1 level checked by IHC. It examined the correlation between prognosis and biomarkers through statistical analysis. Results: AFP, OPN(serum level) and DKK1(serum level) are an independent prognostic factor for overall survival(OS) in HCC after hepatectomy(n=60, P=0.0204, 0.0167 and 0.0455 respectively). New biomarkers combinations based on the AFP existing biomarker are showed a falling curve of the overall survival(OS) and disease-free survival(DFS) in Kaplan Meier curve. Conclusion: In conclusion, combination of OPN, DKK1 and AFP as a biomarker could support the correct diagnosis for HCC after hepatectomy. P14.02 DEVELOPED OPERATION METHOD FOR PATIENT-DRIVED ORTHOTOPIC XENOGRAFT MODEL OF HEPATOCELLULAR CARCINOMA. H. R. Byeon, Y. Seo and S. D. Lee National Cancer Center, Liver Cancer Center, Goyang-si, Gyeonggi-do, South Korea Background: Patient-derived xenograft(PDX) model is a biological organized platform and a patient substitute. Recent studies use the ectopic model for hepatocellular carcinoma(HCC) but this model don’t reflect likewise the tumor state of patient. For exact prognosis, it is need to HPB 2019, 21 (S3), S783eS923