Prognostic scores in primary biliary cholangitis patients with advanced disease

Abstract

Tumour rupture of gastrointestinal stromal tumours (GISTs) has been considered to be a remarkable risk factor because of its unfavourable impact on the oncological outcome. Although tumour rupture has not yet been included in the current tumor-node-metastasis classification of GISTs as a prognostic factor, it may change the natural history of a low-risk GIST to a high-risk GIST. Originally, tumour rupture was defined as the spillage or fracture of a tumour into a body cavity, but recently, new definitions have been proposed. These definitions distinguished from the prognostic point of view between the major defects of tumour integrity, which are considered tumour rupture, and the minor defects of tumour integrity, which are not considered tumour rupture. Moreover, it has been demonstrated that the risk of disease recurrence in R1 patients is largely modulated by the presence of tumour rupture. Therefore, after excluding tumour rupture, R1 may not be an unfavourable prognostic factor for GISTs. Additionally, after the standard adjuvant treatment of imatinib for GIST with rupture, a high recurrence rate persists. This review highlights the prognostic value of tumour rupture in GISTs and emphasizes the need to carefully take into account and minimize the risk of tumour rupture when choosing surgical strategies for GISTs.