Duodenal gastrointestinal stromal tumors: clinicopathological characteristics, surgery, and long-term outcome

Abstract

Background: Duodenal gastrointestinal stromal tumors (DGIST) are rare, and data on their management is limited. We here report the clinicopathological characteristics, different surgical treatments, and long-term prognosis of DGIST.

Methods: Data of 74 consecutive patients with DGIST in a single institution from June 2000 to June 2014 were retrospectively analyzed. The overall survival (OS) and recurrence/metastasis-free survival rates of 74 cases were calculated using Kaplan-Meier method.

Results: Out of 74 cases, 42 cases were female (56.76%) and 32 cases (43.24%) were male. Approximately 22.97, 47.30, 16.22, and 13.51% of the tumors originated in the first to fourth portion of the duodenum, respectively, with a tumor size of 5.08 ± 2.90 cm. Patients presented with gastrointestinal bleeding (n = 37, 50.00%), abdominal pain (n = 25, 33.78 %), mass (n = 5, 6.76%), and others (n = 7, 9.76%). A total of 18 patients (24.3%) underwent wedge resection (WR); 39 patients (52.7%) underwent segmental resection (SR); and 17 cases (23%) underwent pancreaticoduodenectomy (PD). The median follow-up was 56 months (1-159 months); 19 patients (25.68%) experienced tumor recurrence or metastasis, and 14 cases (18.92 %) died. The 1-, 3-, and 5-year recurrence/metastasis-free survival rates were 93.9, 73.7, and 69%, respectively. The 1-, 3- and 5-year OS were 100, 92.5, and 86%, respectively. The recurrence/metastasis-free survival rate in the PD group within 5 years was lower than that in the WR group (P = 0.047), but was not different from that in the SR group (P = 0.060). No statistically significant difference was found among the three operation types (P = 0.294).

Conclusions: DGIST patients have favorable prognosis after complete tumor removal, and surgical procedures should be determined by the DGIST tumor location and size.

Fig. 1

a, b: The abdominal CT scan and endoscopic ultrasonography images reveal a soft tissue mass with size of 6.5 × 4.5 cm located in the second portion of the duodenum, and with an unclear boundary with head of pancreas, right liver, and kidney. c shows a metastasis in the liver at 44 months postoperatively. d: A lump with a size of 4.5 × 3.7 cm adjacent to pancreas. e, f: Liver (mainly in the right liver lobe) and bone (multiple bone destruction of ilium) metastasis occurred 12 years after operation

Fig. 2

Recurrence/metastasis-free survival of 74 duodenal GIST patients. a: stratified by surgical procedures (WR, SR vs. PD). b: stratified by tumor size (<5 cm vs. ≥5 cm). c: stratified by mitotic count (≤5, 6–10 vs. > 10). d: stratified by NIH risk classification (low, intermediate vs. high)