HBP Surgery Week 2020

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[Liver Symposium 2]

[LV SY 2-4] Proper management of benign hepatic tumors
Quirino LAI*
Hepatobiliary and Organ Transplantation Unit, Sapienza University of Rome, Italy

Lecture : Different vascular tumors exist, ranging from the benign hepatic hemangioma (HH), to the intermediately aggressive hepatic epithelioid hemangioendothelioma (HEHE), to the very aggressive hepatic angio-sarcoma (HAS). Therefore, the surgical approach of these tumors present different peculiarities in light of their different aggressiveness and post-surgical results. HH represents an extremely common liver disease, however not requiring in the great majority of cases any kind of management. In peculiar cases, observation should be considered. Surgery is anecdotal, mainly in light of the evidence that spontaneous or post-traumatic rupture represent rare conditions. Surgery is indicated only in peculiar conditions, like the presence of abdominal pain otherwise not explicable, and the complications of HH. In this uncommon condition, the mini-invasive approach should play a relevant role, mainly in consideration of the fact that HH represents a benign condition. Consequently, a less invasive approach appears to be coherent in this field. Approximately 500 cases of laparoscopic surgery/locoregional therapy have been reported so far, showing promising results. Even more anecdotal are the reported cases of robotic resection. Only 10 cases of robotic surgery for HH have been reported. Therefore, it is impossible to give definitive conclusions on the role of robotic surgery in this field. When the mini-invasive approach is chosen as the first-step technique to use for the cure of a patient, surgery should be done exclusively in high-volume centers specialized in mini-invasive surgery. HEHE is a rare vascular tumor that has an intermediate aggressive behavior. Although the value of liver transplantation (LT) is well established, its place in the management of HEHE is still unclear. A recent study from Europe was published. The study was based on the outcomes of 149 transplant recipients with HEHE recorded in the European Liver Transplant Registry during the period November 1984 to May 2014. Median post-LT follow-up was 7.6 years (interquartile range, 2.8-14.4). Cox regression analysis showed that macrovascular invasion (hazard ratio [HR], 4.8; P < 0.001), pre-LT waiting time of 120 days or less (HR, 2.6; P = 0.01) and hilar lymph node invasion (HR = 2.2; P = 0.03), but not pre-LT extrahepatic disease, were significant risk factors for recurrence. These findings, which were also confirmed in a propensity score analysis, allowed the development of a HEHE-LT score enabling stratification of patients in relation to their risk of tumor recurrence. Patients with a score of two or less had a much better 5-year disease-free survival compared to those having a score of six or higher (93.9% vs 38.5%; P < 0.001). The analysis of this (largest in the world) HEHE adult liver recipient cohort clearly confirms the value of LT in the treatment of this rare disorder and permits identification of patients at risk of posttransplant recurrence. Posttransplant follow-up should take the HEHE-LT score into account. Extrahepatic disease localization is reconfirmed not to be a contraindication for LT. As for HAS, LT was performed despite disappointing results. A retrospective study based on 14 males and eight females coming from the European Liver Transplant Registry was reported. A difficult differential diagnosis between HAS and HEHE was reported. However, when HAS ad HEHE were compared, clinical signs, symptoms, and biochemical parameters differed significantly. Pre-LT diagnosis of HAS was made in only five of 16 (31%) biopsied patients. HEHE (seven patients) and hepatocellular cancer (two patients) were confounding diagnoses leading to LT. Extrahepatic disease was present at time of LT in four (19%) patients. Giant invalidating tumor (five HAS, one with Budd-Chiari syndrome [BCS], and 10 supposed HEHE, 1 with BCS), acute BCS of unknown origin (two patients), chronic liver failure (four patients), and solitary hepatocellular cancer (one patient) were the main indications for LT. Overall survival was 7.2±2.6 months; no patient survived after 23 months. Recurrence was diagnosed after 5.0±2.6 months. Seventeen (77.2%) patients died of tumor recurrence, and the remaining five patients died of early infections. HAS is an absolute contraindication to LT due to the poor outcome. When dealing with the difficult differential diagnosis between HAS and HEHE, futile LT can be avoided by taking into consideration their distinct clinical and biochemical behaviors as well as a 6-month wait-list observation period. This time enables the evaluation of HAS disease progression without compromising prognosis of HE patients, thereby allowing to avoid organ wastage.


HBP SURGERY WEEK 2020_LV_SY_2_4.pdf
SESSION
Liver Symposium 2
Room A 7/27/2020 4:10 PM - 4:30 PM