Quality of treatment and surgical approach for rectal gastrointestinal stromal tumour (GIST) in a large European cohort

Abstract

Background: Rectal gastrointestinal stromal tumours (GISTs) are rare tumours. Variability in the management may influence outcome, but there is a lack of understanding regarding contemporary variance in care. A multicenter, international, retrospective cohort study was performed to elucidate characteristics and outcomes of rectal GIST in European practice, with particular reference to surgical approach. Methods: All rectal GIST patients diagnosed between 2009 and 2018 were identified from five European databases. Recurrence free survival (RFS) and overall survival (OS) were estimated using Kaplan-Meier method. Possible confounders were identified using Cox regression analyses. Results: From 210 patients, 155 patients had surgery. The three main types of surgery were local tumour resection (LTR, n ¼ 46), low anterior resection (LAR, n ¼ 31) and abdomino-perineal resection (APR, n ¼ 32). Most patients received neoadjuvant (65%) and/or adjuvant imatinib therapy (66%). Local recurrence rate after surgery was 15% and overall recurrence rate 28%. No significant differences were found in terms of RFS nor OS between LTR, LAR and APR. However, locally resected tumours were smaller, while LAR and APR patients more often received perioperative imatinib. General hospitals treated smaller GISTs, offered imatinib less frequently, and had a higher tumour rupture rate. In the

The Outcome of Neoadjuvant Imatinib Therapy Combined With Surgery for Rectal Gastrointestinal Stromal Tumors: A Report of Three Cases and a Review of the Literature

“…GISTs are generally sporadic except in rare cases associated with familial syndromes such as neurofibromatosis type 1 [6]. The clinical presentation of rectal GIST is varied and nonspecific; the most frequent clinical symptoms include rectal bleeding, rectal mass, change in bowel habits, and abdominal/anal pain [4]. Anal pain was the revealing clinical sign in all our cases.…”

“…GISTs are mesenchymal tumors developing in the majority of cases in the stomach and small intestine; the rectal location remains exceptional with only 5% of cases. The average age at diagnosis is around 60 years according to large European and American cohorts [4,5]. GISTs are generally sporadic except in rare cases associated with familial syndromes such as neurofibromatosis type 1 [6].…”

“…The type of mutation influences the prognosis and the effectiveness of adjuvant therapy [3,9]. According to a large European cohort, most of the GISTs were KIT exon 11 mutated (74%); the other mutations found were KIT exon 9 (14%), KIT exon 13 (3%), and only one patient among 156 presented with a PDGFRA mutation; however, 12% of the cases were wildtype with no KIT or PDGFRA gene mutation [4].…”

Gastrointestinal stromal tumour of the rectum and intestinal obstruction: case report

“…Surgical resection is the treatment for all resectable rectal GIST patients [17,25]. The anatomical relationship of the rectum in the pelvis, the sphincter complexity, risk of tumour rupture and positive surgical margins makes a challenging management condition.…”

“…The decision on the optimal surgical treatment mainly depends on the tumour size, mutational status and the surgeon's capacity [35]. The lack of evidence in large-scale prospective studies makes neoadjuvant treatment, surgical approach and prognosis for rectal GIST still controversial for some authors [25,28]. In the present case, a radical approach was performed; the size of the tumour in the lower rectum and the high risk of recurrence due to previous incisional biopsy through the mesorectum, with the intraluminal ulcerated component (Figure 3a and b), were the most important considerations for the management decision.…”