Abstract
ObjectivesThis study aimed to report the clinicopathological features, management and long-term outcomes of patients with gastrointestinal stromal tumours (GISTs) in Oman.MethodsThis retrospective study was conducted on patients treated for GIST between January 2003 and December 2017 at three tertiary referral centres in Muscat, Oman. All patients with confirmed histopathological diagnoses of GIST and followed-up at the centres during this period were included. Relevant information was retrieved from hospital records until April 2019.ResultsA total of 44 patients were included in the study. The median age was 55.5 years and 56.8% were female. The most common primary site of disease was the stomach (63.6%) followed by the jejunum/ileum (18.2%). Two patients (4.5%) had c-Kit-negative, discovered on GIST-1-positive disease. A total of 24 patients (54.5%) presented with localised disease and eight (33.3%) were classified as being at high risk of relapse. Patients with metastatic disease received imatinib in a palliative setting, whereas those with completely resected disease in the intermediate and high-risk groups were treated with adjuvant imatinib. Of the six patients (13.6%) with progressive metastatic disease, of which four had mutations on exon 11 and one on exon 9, while one had wild-type disease. Overall, rates of progression-free survival and overall survival (OS) at 100 months were 77.4% and 80.4%, respectively. Rates of OS for patients with localised and metastatic disease were 89.9% and 80.2%, respectively.ConclusionThe presenting features and outcomes of patients with GISTs in Oman were comparable to those reported in the regional and international literature.
Highlights
Patients with gastrointestinal stromal tumours (GISTs) without CD117 expression may harbour mutations in the platelet‐derived growth factor receptor-α (PDGFR-α) gene or may have wildtype tumours with substantial reductions in the expression of succinate dehydrogenase.[6]
It has become possible to stratify patients based on risk of recurrence into various categories using either the National Institutes of Health (NIH), Armed Forces Institute of Pathology (AFIP) or modified Jonessu criteria.[4,7,8]
Clinicopathological Features and Outcomes of Gastrointestinal Tumours in Oman A 15-year multi-centre study has dramatically improved the prognosis of patients with unresectable, recurrent or metastatic GISTs, and reduced the risk of recurrence and improved progression-free survival (PFS) and overall survival (OS) rates in patients considered to have a high risk of relapse after complete resection.[12]
Summary
Patients with GISTs without CD117 expression may harbour mutations in the platelet‐derived growth factor receptor-α (PDGFR-α) gene or may have wildtype tumours with substantial reductions in the expression of succinate dehydrogenase.[6]. Complete surgical resection is the mainstay of treatment for GIST cases with localised disease.[4] More recently, it has become possible to stratify patients based on risk of recurrence into various categories using either the National Institutes of Health (NIH), Armed Forces Institute of Pathology (AFIP) or modified Jonessu criteria.[4,7,8] In general, the risk of relapse depends on the site and size of the tumour and its mitotic rate.[8,9,10] In particular, metastatic GISTs are resistant to standard chemotherapy.[11] The introduction of tyrosine kinase inhibitors (TKIs). While several studies have sought to report the presentation and outcomes of GIST cases across the world, there remains a scarcity of literature from Oman and the Gulf region.[13,14,15,16] As such, this study aimed to report the clinicopathological features and outcomes of GIST cases in Oman
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