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Special Feature
November 2002

Image of the Month

Arch Surg. 2002;137(11):1311-1312. doi:
Answer: Small-Bowel Stromal Tumor

Figure 1. A computed tomographic scan demonstrates a 7 × 5-cm heterogeneous mass involving the third to fourth portions of the duodenum.

Figure 2. An esophagogastroduodenoscopy shows a submucosal mass in the distal duodenum with normal overlying mucosa and an unrelated diverticulum.

The patient underwent exploratory laparotomy at the location of a 7 × 5-cm tumor in the fourth portion of the duodenum, and another 1-cm tumor was identified in the jejunum. Both tumors were resected along with the involved small-bowel segments. The pathologic findings included 7.5-cm and 1.1-cm gastrointestinal stromal tumors (GISTs) with uninvolved surgical margins. The patient had an uneventful postoperative course and was free of disease 6 months after the operation.

Gastrointestinal stromal tumors are rare mesenchymal tumors accounting for 0.1% to 3% of all gastrointestinal neoplasms. Approximately 150 new cases are diagnosed in the United States each year.1,2The cellular origin of GISTs has been traced to the interstitial cell of Cajal, originating from the gastrointestinal stem cell.3These tumors are distributed evenly between the sexes, with a peak occurrence in the sixth decade of life.14The stomach and small bowel are the anatomic sites of origin for 90% of the tumors, and the distribution is equal between these locations. The occurrence of multiple primary GISTs is unusual, and the finding of multiple lesions generally indicates metastatic disease. Patients with small-bowel GISTs larger than 4 cm are frequently symptomatic, with gastrointestinal bleeding, pain, and obstruction being the most common associated symptoms. Bleeding associated with GIST is usually significant and necessitates blood transfusion in the majority of patients.4Enteroclysis, computed tomography, and ultrasonography have been found useful for the diagnosis of small-bowel GISTs.5When identified, GISTs should be treated by complete resection. The prognosis of patients with these tumors is influenced by the tumor involvement at the resected margins, tumor size, tumor cellularity, degree of histologic differentiation, and the number of mitoses per 50 high-power fields.14Local and metastatic recurrences are common following resection of malignant small-bowel GISTs, and current chemotherapeutic regimens have not been shown to improve patient survival.13The 5-year actuarial survival of patients with malignant small bowel GISTs has been reported at 41%.2Small-bowel GISTs should be considered as a potential cause of gastrointestinal bleeding whenever patients exhibit significant bleeding that is not identified with an upper gastrointestinal tract or colorectal source. Patients with continued active bleeding should be further evaluated with mesentery angiography, whereas those whose bleeding spontaneously resolves should undergo evaluation of the small bowel with enteroclysis or computed tomography.

Pidhorecky  ICheney  RTKraybill  WGGibbs  JF Gastrointestinal stromal tumors: current diagnosis, biologic behavior, and management.  Ann Surg Oncol. 2000;7705- 712Crossref
Crosby  JACatton  CNDavis  A  et al.  Malignant gastrointestinal stromal tumor of the small intestine: a review of 50 cases from a perspective database.  Ann Surg Oncol. 2001;850- 59Crossref
Dematteo  RPLewis  JJLeung  D  et al.  Two hundred gastrointestinal stromal tumors: recurrent patterns and prognostic factors for survival.  Ann Surg. 2000;23151- 58Crossref
Ludwig  DJTraverso  LW Gut stromal tumors and their clinical behavior.  Am J Surg. 1997;173390- 394Crossref
Rioux  MMailloux  C Crescent-shaped necrosis: a new imaging sign suggestive of stromal tumor of the small bowel.  Abdom Imaging. 1997;22376- 380Crossref