A large gastrointestinal stromal tumor of the duodenum: a case report
© Morcos and Al-Ahmad; licensee BioMed Central Ltd. 2011
Received: 14 May 2011
Accepted: 14 September 2011
Published: 14 September 2011
Gastrointestinal stromal tumors of the duodenum are uncommon. They can reach a large size. Diagnosis can be elusive and managing them can be difficult. Our case report aims to increase awareness and highlight some issues related to the diagnosis and management of duodenal gastrointestinal stromal tumors.
We present the case of a 38-year-old Middle Eastern woman with a large, slowly-growing gastrointestinal stromal tumor of the duodenum. Her complaints were minor epigastric discomfort and swelling. A pancreaticoduodenectomy with complete tumor excision was performed. She was doing very well with no evidence of disease recurrence when she was last seen 34 months after her operation.
Gastrointestinal stromal tumors of the duodenum should be suspected in any patient with a duodenal wall mass. Extramural growth and central ulceration with or without bleeding should alert the endoscopist to the possibility of a duodenal gastrointestinal stromal tumor diagnosis. There is more than one surgical approach available; however, complete surgical excision, with negative margins, is the absolute requirement. Preoperative imatinib mesylate can be considered in unresectable or borderline resectable cases.
The most common sites for gastrointestinal stromal tumors (GIST) are the stomach and, to a lesser extent, the small intestine . Small intestinal GIST can occur anywhere along the length of the bowel and can be multiple. The duodenum is involved in about 10% to 20% of small intestinal GIST . Although duodenal GIST is similar pathologically to that involving other organs, they do have some peculiar features. GISTs in the duodenum pose particular challenges for diagnosis and management.
We describe the case of a large duodenal GIST including its presentation, diagnosis, and the type of surgery performed, as well as a review of issues related to GIST in the duodenum.
A 38-year-old Middle Eastern woman presented with a slowly enlarging abdominal mass of 12 years duration. According to the patient, a surgeon had attempted to resect the mass 12 years earlier, but could not do so due to excessive bleeding from the tumor. She was offered no further treatment.
At presentation, her main complaint was epigastric discomfort. She also gave a history of some mild back pain and occasional abdominal pain. Her appetite was good and she had not lost weight. There was no history of vomiting, change in bowel habits or melena. She had been diagnosed with a peptic ulcer many years ago.
GISTs are the most common mesenchymal tumors of the gastrointestinal tract . They are most commonly found in the stomach and small bowel. Uncommon sites include the colon, rectum, esophagus and even the liver and mesentery. They mainly affect adults and are uncommon in children . The duodenum is an uncommon site for GIST. It comprises 10%-20% of small-intestinal GISTs, or only three to five percent of all GIST cases . Most data on duodenal GIST are from single case reports or from a few small series [4, 5]. Duodenal GIST is usually asymptomatic when small in size and can reach a large size before causing any symptom. As the tumor enlarges it causes variable symptomatology. The most common presentation is gastrointestinal bleeding which may be chronic and mild or sudden and massive . Although our patient had a large tumor, she had mild anemia. The next most common presentations are abdominal discomfort, pain and swelling .
Diagnosis can be made with upper gastrointestinal endoscopy . The tumor is usually exophytic, and appears as a submucosal swelling. Sometimes it presents only as an ulcer, as in our case. The biopsy should be deep, but may not always be diagnostic. Endoscopic ultrasound can help in delineating the submucosal tumor. A CT scan of the abdomen usually shows a retroperitoneal tumor at the site of the duodenum and head of the pancreas . However, CT scans are not always helpful in specifying the origin of the mass. In a number of cases reported in the literature, the mass was misdiagnosed as arising from the head of the pancreas .
The treatment of choice for duodenal GIST is complete surgical excision. This can be performed by local or segmental duodenal resection with preservation of the pancreas for small tumors . As for larger tumors, a pancreaticoduodenectomy is required. The surgical choice depends not only on the size of the tumor but also on the location in the duodenal wall and the relation to the ampulla of Vater. It is not clear what the optimal surgical margin should be, but a negative one is essential to prevent local recurrence of the tumor. No lymph node dissection is required since they are very unlikely to be involved .
The outcome depends on the pathological features of the tumor and the completeness of surgical resection. Large tumors with high mitotic counts behave much worse than small tumors with low mitotic counts, which are considered benign . Local recurrence is higher in tumors not completely removed or with a positive microscopic margin. Most GISTs respond to IM, so patients with tumors with a high malignant potential should be offered IM as an adjuvant therapy. Preoperative IM can be given in cases of unresectable or borderline resectable cases. This might improve resectability.
Duodenal GIST should be suspected in any patient with a duodenal wall mass. Extramural growth and central ulceration with or without bleeding should alert the endoscopist to the possibility of this diagnosis. There is more than one surgical approach available, but the absolute requirement is complete surgical excision. Preoperative IM can be considered in unresectable or borderline resectable cases.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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