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Open Access Research article

Primary localized rectal/pararectal gastrointestinal stromal tumors: results of surgical and multimodal therapy from the French Sarcoma group

Thanh-Khoa Huynh115*, Pierre Meeus2, Philippe Cassier3, Olivier Bouché4, Sophie Lardière-Deguelte5, Antoine Adenis6, Thierry André7, Julien Mancini8, Olivier Collard9, Michael Montemurro10, Emmanuelle Bompas11, Maria Rios12, Nicolas Isambert13, Didier Cupissol14, Jean-Yves Blay3 and Florence Duffaud1

Author Affiliations

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

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BMC Cancer 2014, 14:156  doi:10.1186/1471-2407-14-156

Published: 5 March 2014

Abstract

Background

Rectal and pararectal gastrointestinal stromal tumors (GISTs) are rare. The optimal management strategy for primary localized GISTs remains poorly defined.

Methods

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.

Results

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.

Conclusions

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.