Case report: First case of undifferentiated embryonal sarcoma of the liver in a child with neurofibromatosis type 1, treated by hepatic chemoperfusion with transcatheter arterial chemoembolization.

Introduction: In this report we firstly describe undifferentiated embryonal sarcoma of the liver (UESL) in a patient with neurofibromatosis type 1 (NF1), fatally complicated by synchronous malignant peripheral nerve sheath tumor (MPNST) with a highly aggressive metastatic course. The case also represents our first experience of chemoperfusion involving the transcatheter arterial chemoembolization (TACE) in a pediatric patient, applied as a treatment for UESL. Case presentation: A 13-year-old girl was diagnosed with NF1 and presented with a liver tumor identified as UESL by histological assessment. The tumor was refractive to the conventional first-line chemotherapy. The patient received hepatic chemoperfusion with TACE, which was efficacious; however, the overall curative outcome was unsatisfactory due to synchronous unresectable retroperitoneal MPNST with mesenteric metastases and ultimate progression of the UESL. Conclusion: This is the first reported case of UESL in a patient with NF1. The results demonstrate the efficacy of hepatic chemoperfusion with TACE in pediatric UESL.

Laparoscopic radical and conservative surgery for hydatid liver echinococcosis: PSM based comparative analysis of immediate and long-term outcomes.

BACKGROUND: The diffusion of laparoscopic radical surgery for hydatid liver echinococcosis remains limited. There are no published data on a comparative analysis of the immediate and long-term results of radical and conservative laparoscopic surgery for liver hydatid cysts. Comparison of the immediate and long-term outcomes after laparoscopic radical and conservative cystectomies was aimed. METHODS: HPB center (Center 1) and general surgery hospital in an endemic area (Center 2) participated in a retrospective study. Radical surgery included total, subtotal pericystectomy, and liver resection. Conservative surgery comprised cystectomy without/with partial pericystectomy. RESULTS: The total number of patients who underwent surgery for liver hydatid cysts was 213. Laparoscopic cystectomy was performed in 106 (50%) patients. This number included 47 radical laparoscopic cystectomy (Center 1). Conservative laparoscopic procedures were used in 59 patients (Center 2). Finally, twenty-seven pairs of patients were matched. Immediate outcomes were better for radical treatment in terms of severe morbidity, length of hospital stay, and time of abdominal drainage before and after PSM. The mean follow-up length was 23 (4-66) and 29 (6-66) months and the recurrence rate was 2% and 5% in groups of radical and conservative treatment respectively. No differences were found in 1-, 3-, and 5-year disease free survival. After second PSM for recurrence, 20 pairs were matched with no relapse of disease. CONCLUSION: Laparoscopic radical surgery leads to the better immediate outcomes and can be recommended as the preferred treatment option in a specialized HPB center. Conservative option is justified in general hospitals in endemic area for selected uncomplicated cysts.

Combining E-PASS model and disease specific risk factors to predict severe morbidity after liver and bile duct resection for perihilar cholangiocarcinoma.

BACKGROUND: Estimation of physiologic ability and surgical stress system (E-PASS) has been shown to be effective in predicting morbidity after surgery for perihilar cholangiocarcinoma (PHCC). Nevertheless, E-PASS does not include an assessment of the disease specific risk factors. The aim of the study was to estimate the combined impact of E-PASS and specific preoperative factors on major morbidity for PHCC patients. METHODS: A retrospective analysis of a prospectively collected data was performed. Severe morbidity according to complication comprehensive index was defined as ≥40 points. A value of comprehensive risk score (CRS) ≥1 was taken as critical. RESULTS: Multivariate analysis of perioperative data from 122 patients revealed significant impact of five factors (CRS ≥1, future liver remnant volume <50%, T4 stage, moderate and severe cholangitis, INR) on the risk of severe morbidity after resection. The AUC for the combination of these factors was classified as good predictive value (0.810, 95% CI 0.729-0.891) and poor predictive value (0.673, 95% CI 0.573-0.773) for CRS alone (p = 0.040). CONCLUSION: A combination of E-PASS with disease specific risk factors is a reliable predictive model for major morbidity for patients undergoing radical surgery for PHCC.

Comparative analysis of learning curve in complex robot-assisted and laparoscopic liver resection.

BACKGROUND: There is no comparative analysis of the learning curves for robot-assisted and laparoscopic liver resection. We aimed to compare learning curves in complex robotic and conventional laparoscopic liver resections with regards to estimation of the difficulty index score. METHODS: The results of 131 consecutive liver resections were analyzed retrospectively (40 robot-assisted and 91 laparoscopic). The learning curve evaluation was based on calculation of procedures number before significant change of the difficulty index for minimally invasive liver resection or the rate of posterosuperior segments resection. Groups of early and late experience were compared in every type of approach (robot-assisted and laparoscopic). RESULTS: Significant increase of difficulty index (from 5.0 [3.0-7.7] to 7.3 [4.3-10.2]) of robotic procedures required 16 procedures. It was necessary to perform 29 laparoscopic resections in order to significantly increase the rate of laparoscopic posterosuperior segments resection but without significant increase of difficulty index. The implementation of minimally invasive liver resection started with the robotic approach. CONCLUSION: The learning curve for robot-assisted liver resections is shorter in comparison with laparoscopic resections. The inclusion of robot-assisted resections in a minimally invasive liver surgery program may be useful to rapidly increase the complexity of laparoscopic liver resections.