Primary localized rectal/pararectal gastrointestinal stromal tumors: results of surgical and multimodal therapy from the French Sarcoma group
1 Service d’Oncologie Médicale, CHU Timone, AP-HM, Marseille, and Aix-Marseille Université, Marseille, France
2 Service de Chirurgie, Centre Léon Bérard, Lyon, France
3 Service d’Oncologie Médicale, Centre Léon Bérard, Lyon, France
4 Service d’Oncologie Digestive, CHU Robert Debré, Reims, France
5 Service de Chirurgie, CHU Robert Debré, Reims, France
6 Service d’Oncologie Médicale, Centre Oscar Lambret, Lille, France
7 Service d’Oncologie Médicale, Hôpital St Antoine, Assistance Publique–Hôpitaux de Paris and Université Pierre & Marie Curie (UPMC), Paris, France
8 Service de Santé Publique et d’Information Médicale, Unité de Biostatistique, CHU Timone, APHM, Marseille, and Aix-Marseille Université, Marseille, France
9 Service d’Oncologie Médicale, CLCC, Institut de Cancérologie Lucien Neuwirth, Saint-Etienne, France
10 Centre Pluridisciplinaire d’Oncologie, CHUV Lausanne, Lausanne, Suisse
11 Service d’Oncologie médicale, Centre René Gauduchau, Nantes, France
12 Service d’Oncologie Médicale, Centre Alexis Vautrin, Vandoeuvre les, Nancy, France
13 Service d’Oncologie Médicale, Centre George François Leclerc, Dijon, France
14 Service d’Oncologie Médicale, Centre Valdorelle, Montpellier, France
15 Service d’Oncologie Médicale Adulte, CHU Timone, AP-HM, 264 rue Saint Pierre, 13385 Marseille, France
BMC Cancer 2014, 14:156 doi:10.1186/1471-2407-14-156Published: 5 March 2014
Rectal and pararectal gastrointestinal stromal tumors (GISTs) are rare. The optimal management strategy for primary localized GISTs remains poorly defined.
We conducted a retrospective analysis of 41 patients with localized rectal or pararectal GISTs treated between 1991 and 2011 in 13 French Sarcoma Group centers.
Of 12 patients who received preoperative imatinib therapy for a median duration of 7 (2-12) months, 8 experienced a partial response, 3 had stable disease, and 1 had a complete response. Thirty and 11 patients underwent function-sparing conservative surgery and abdominoperineal resection, respectively. Tumor resections were mostly R0 and R1 in 35 patients. Tumor rupture occurred in 12 patients. Eleven patients received postoperative imatinib with a median follow-up of 59 (2.4-186) months. The median time to disease relapse was 36 (9.8-62) months. The 5-year overall survival rate was 86.5%. Twenty patients developed local recurrence after surgery alone, two developed recurrence after resection combined with preoperative and/or postoperative imatinib, and eight developed metastases. In univariate analysis, the mitotic index (≤5) and tumor size (≤5 cm) were associated with a significantly decreased risk of local relapse. Perioperative imatinib was associated with a significantly reduced risk of overall relapse and local relapse.
Perioperative imatinib therapy was associated with improved disease-free survival. Preoperative imatinib was effective. Tumor shrinkage has a clear benefit for local excision in terms of feasibility and function preservation. Given the complexity of rectal GISTs, referral of patients with this rare disease to expert centers to undergo a multidisciplinary approach is recommended.