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Eileen T. Consorti, MD; Terrence H. Liu, MD; Angela McGee, MD
From the Departments of Surgery (Dr Consorti) and Gastroenterology (Dr McGee), Kelsey-Seybold Clinic, Houston, and the Department of Surgery, University of Texas–Houston Medical School (Dr Liu). Dr Liu is now with the Department of Surgery, University of California, San Francisco–East Bay, Oakland.

Arch Surg. 2002;137:1311-1312.

INTRODUCTION
A 56-YEAR-OLD man sought treatment because of bloody stools and syncope. He underwent a transfusion and an esophagogastroduodenoscopy, which showed a hiatal hernia and mild gastritis but no source of bleeding. Results of a colonoscopy were normal. Although the bleeding spontaneously resolved, during the subsequent 2 years, he continued to have trace hemoccult-positive stools and was treated for reflux esophagitis. A routine chest radiograph demonstrated an abnormality in his mediastinum. A computed tomographic scan showed a large hiatal hernia and a 7 x 5-cm incidental mass in the abdomen (Figure 1). Subsequently, an esophagogastroduodenoscopy was performed, and it showed a submucosal mass between the third to fourth portions of the duodenum (Figure 2).

Figure 1.

Figure 2.

What Is the Diagnosis?

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A. Duplication cyst

B. Pancreatic pseudocyst

C. Duodenal diverticulum

D. Small-bowel stromal tumor

Answer: Small-Bowel Stromal Tumor

Figure 1. A computed tomographic scan demonstrates a 7 x 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 x 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.^1-2 The cellular origin of GISTs has been traced to the interstitial cell of Cajal, originating from the gastrointestinal stem cell.³ These tumors are distributed evenly between the sexes, with a peak occurrence in the sixth decade of life.^1-4 The 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.⁴ Enteroclysis, computed tomography, and ultrasonography have been found useful for the diagnosis of small-bowel GISTs.⁵ When 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.^1-4 Local and metastatic recurrences are common following resection of malignant small-bowel GISTs, and current chemotherapeutic regimens have not been shown to improve patient survival.^1-3 The 5-year actuarial survival of patients with malignant small bowel GISTs has been reported at 41%.² Small-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.

AUTHOR INFORMATION

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Accepted for publication March 8, 2002.

Corresponding author and reprints: Terrence H. Liu, MD, Department of Surgery, University of California, San Francisco–East Bay, 1411 E 31st St, Oakland, CA 94602.

REFERENCES

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1. Pidhorecky I, Cheney RT, Kraybill WG, Gibbs JF. Gastrointestinal stromal tumors: current diagnosis, biologic behavior, and management. Ann Surg Oncol. 2000;7:705-712. ABSTRACT 2. Crosby JA, Catton CN, Davis A, et al. Malignant gastrointestinal stromal tumor of the small intestine: a review of 50 cases from a perspective database. Ann Surg Oncol. 2001;8:50-59. FREE FULL TEXT 3. Dematteo RP, Lewis JJ, Leung D, et al. Two hundred gastrointestinal stromal tumors: recurrent patterns and prognostic factors for survival. Ann Surg. 2000;231:51-58. FULL TEXT | ISI | PUBMED 4. Ludwig DJ, Traverso LW. Gut stromal tumors and their clinical behavior. Am J Surg. 1997;173:390-394. FULL TEXT | PUBMED 5. Rioux M, Mailloux C. Crescent-shaped necrosis: a new imaging sign suggestive of stromal tumor of the small bowel. Abdom Imaging. 1997;22:376-380. FULL TEXT | PUBMED

SECTION EDITOR: GRACE S. ROZYCKI, MD

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