History

Many gastrointestinal stromal tumors (GISTs) are discovered incidentally during endoscopic or surgical procedures. In Japan, mass screening for gastric adenocarcinoma with upper endoscopy has led to an increase in incidental findings of asymptomatic GISTs.

Other GISTs are detected on radiologic studies performed to investigate protean manifestations of GI tract disease or procedures performed to treat an emergent condition such as hemorrhage or obstruction. In a population-based study, the median tumor size of GISTs that were detected as incidental findings was 2.7 cm, versus 8.9 cm for those found on the basis of symptoms.^[31]

GISTs may produce symptoms secondary to hemorrhage or obstruction. Upper GI bleeding is the most common clinical manifestation of GISTs, manifesting as hematemesis or melena in 40-65% of patients. Bleeding occurs because of pressure necrosis and ulceration of the overlying mucosa with resultant hemorrhage from disrupted vessels. Patients who have experienced significant blood loss may report malaise, fatigue, or exertional dyspnea.

Obstruction can result from intraluminal growth of an endophytic tumor or from luminal compression from an exophytic lesion. The obstructive symptoms can be site-specific (eg, dysphagia with an esophageal GIST, constipation with a colorectal GIST, obstructive jaundice with a duodenal tumor). Other symptoms are generally associated with an enlarging abdominal mass and may include the following:

Abdominal pain
Anorexia
Nausea
Vomiting
Weight loss
Epigastric fullness
Early satiety

Physical Examination

Physical examination rarely demonstrates any significant findings. In some cases, examination may identify a palpable mass in the abdomen. Palpable masses are typically detected in patients with extraluminal tumor growth.

Other patients may present with nonspecific physical findings associated with GI blood loss, bowel obstruction, or bowel perforation and abscess formation.

Patients with significant GI bleeding may present with abnormal vital signs or overt shock. In others, fecal occult blood testing may be positive.

Physical findings associated with bowel obstruction can include a distended, tender abdomen. Duodenal obstruction involving the ampulla may be associated with jaundice and, rarely, even a distended palpable gallbladder.

If perforation has occurred, focal or widespread signs of peritonitis are present.

Differential Diagnoses

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Media Gallery

Shown here is a gastric gastrointestinal stromal tumor (GIST). This is a gross specimen following partial gastrectomy. Note the submucosal tumor mass with the classic features of central umbilication and ulceration.
CT scan of the abdomen with oral contrast in a 60-year-old woman with a gastric gastrointestinal stromal tumor (GIST). A huge mass with central necrosis is observed originating from the gastric wall and narrowing its lumen. An ulcer crater can be identified within the mass (arrow).
Photomicrograph of gastrointestinal stromal tumor (GIST) stained with hematoxylin and eosin (H&E) and magnified 40X. Note the solid sheet of spindle cells.
Photomicrograph of gastric gastrointestinal stromal tumor (GIST) stained with hematoxylin and eosin (H&E) and magnified 400X. This stromal tumor demonstrates spindle cells with epithelioid features.
Photomicrograph of gastrointestinal stromal tumor (GIST) with immunohistochemical staining for CD117. Note the strong positive staining of tumor cells with negative staining of the adjacent vessel. Positive stain for CD117 is diagnostic of GIST.

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Table 1. TNM Classification for Gastrointestinal Stromal Tumors

Primary tumor (T)
TX	Primary tumor cannot be assessed
T0	No evidence of primary tumor
T1	Tumor 2 cm or smaller
T2	Tumor more than 2 cm but not more than 5 cm
T3	Tumor more than 5 but not more than 10 cm
T4	Tumor more than 10 cm in greatest dimension
Regional lymph node (N)
N0	No regional lymph node metastasis
N1	Regional lymph node metastasis
Metastasis (M)
M0	No distant metastasis
M1	Distant metastases

Table 2. Anatomic Stage/Prognostic Groups for Gastric GISTs

Group	Tumor	Node	Metastasis	Mitotic Rate*
Stage IA	T1 or T2	N0	M0	Low
Stage IB	T3	N0	M0	Low
Stage II	T1	N0	M0	High
	T2	N0	M0	High
	T4	N0	M0	Low
Stage IIIA	T3	N0	M0	High
Stage IIIB	T4	N0	M0	High
Stage IV	Any T	N1	M0	Any rate
Stage IV	Any T	Any N	M1	Any rate
*Mitotic rate: Low (≤5 per 5 mm² or per 50 HPF); High (>5 per 5 mm² or per 50 HPF)

Table 3. Anatomic Stage/Prognostic Groups for Small-Intestinal GISTs

Group	Tumor	Node	Metastasis	Mitotic Rate*
Stage I	T1 or T2	N0	M0	Low
Stage II	T3	N0	M0	Low
Stage IIIA	T1	N0	M0	High
Stage IIIA	T4	N0	M0	Low
Stage IIIB	T2	N0	M0	High
	T3	N0	M0	High
	T4	N0	M0	High
Stage IV	Any T	N1	M0	Any Rate
Stage IV	Any T	Any N	M1	Any Rate
*Mitotic rate: Low (≤5 per 5 mm² or per 50 HPF); High (>5 per 5 mm² or per 50 HPF)

Table 4. Malignant Potential of Gastric GISTs (NCCN)

Tumor Size	Mitotic Rate*	Predicted Metastasis Rate
≤2 cm	Any mitotic rate	0%
>2 to 5 cm	≤5	1.9%
>2 to 5 cm	>5	16%
>5 to 10 cm	≤5	3.6%
>5 to 10 cm	>5	55%
>10 cm	≤5	12%
>10 cm	>5	86%
*Number of metastases per 5 mm² or per 50 high-power field

Table 5. Malignant Potential of Non-Gastric GISTs (NCCN)

Tumor Size	Mitotic Rate*	Predicted Metastasis Rate
≤2 cm	≤5	0%
≤2 cm	>5	50-54%
>2 to 5 cm	≤5	1.9-8.5%
>2 to 5 cm	>5	50-73%
>5 to 10 cm	≤5	24%
>5 to 10 cm	>5	85%
>10 cm	≤5	34-52%
>10 cm	>5	71-90%
*Number of metastases per 5 mm² or per 50 high-power field

Table 4. Staging of Gastric Gastrointestinal Stromal Tumors

Stage	T	N	M	Grade
IA	T1 or T2	N0	M0	Low
IB	T3	N0	M0	Low
II	T1	N0	M0	High
	T2	N0	M0	High
	T4	N0	M0	Low
IIIA	T3	N0	M0	High
IIIB	T4	N0	M0	High
IV	Any T	N1	M0	Any grade
IV	Any T	Any N	M1	Any grade