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DOI: 10.1148/rg.232025146
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(Radiographics. 2003;23:283-304.)
© RSNA, 2003


AFIP ARCHIVES

From the Archives of the AFIP

Gastrointestinal Stromal Tumors: Radiologic Features with Pathologic Correlation1

Angela D. Levy, LTC, MC, USA, Helen E. Remotti, MAJ, MC, USAR, William M. Thompson, MD, Leslie H. Sobin, MD and Markku Miettinen, MD

1 From the Departments of Radiologic Pathology (A.D.L., W.M.T.), Hepatic and Gastrointestinal Pathology (H.E.R., L.H.S.), and Soft Tissue Pathology (M.M.), Armed Forces Institute of Pathology, 6825 16th St NW, Washington, DC 20306-6000; Department of Radiology and Nuclear Medicine, Uniformed Services University of the Health Sciences, Bethesda, Md (A.D.L.); and Department of Radiology, Duke University, Durham, NC (W.M.T.). Received August 30, 2002; revision requested October 3 and received October 11; accepted October 16. Address correspondence to A.D.L. (levya@afip.osd.mil).


    Abstract
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 Abstract
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 Introduction
 Clinical Features
 Pathologic Features
 Radiologic Features
 Prognosis and Therapy
 Conclusions
 References
 
Gastrointestinal stromal tumors (GISTs) are unique neoplasms that occur throughout the gastrointestinal tract, mesentery, omentum, and retroperitoneum. They are the most common mesenchymal neoplasm of the gastrointestinal tract and are defined by their expression of KIT (CD117), a tyrosine kinase growth factor receptor. The expression of KIT is important to distinguish GISTs from other mesenchymal neoplasms such as leiomyomas, leiomyosarcomas, schwannomas, and neurofibromas and to determine the appropriateness of KIT-inhibitor therapy. The series described herein was accumulated over 2 years and includes 64 pathologically proved GISTs (28 gastric, 27 small intestinal, six anorectal, one colonic, one esophageal, and one from the small bowel mesentery). Radiologic features of GISTs vary depending on tumor size and organ of origin. Since most GISTs arise within the muscularis propria of the stomach or intestinal wall, they most commonly have an exophytic growth pattern and manifest as dominant masses outside the organ of origin. Dominant intramural and intraluminal masses are less common radiologic manifestations. GISTs occurring in the gastrointestinal tract and mesentery characteristically have hemorrhage, necrosis, or cyst formation that appears as focal areas of low attenuation on computed tomographic images. Although the radiologic features of GISTs are often distinct from those of epithelial tumors, criteria to separate GISTs radiologically from other nonepithelial tumors have not yet been fully developed.

© RSNA, 2003

Index Terms: Gastrointestinal stromal tumor (GIST), 70.30 • Gastrointestinal tract, neoplasms, 70.30 • Intestinal neoplasms, 74.30 • Stomach, neoplasms, 72.30


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    Introduction
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 Abstract
 LEARNING OBJECTIVES FOR TEST...
 Introduction
 Clinical Features
 Pathologic Features
 Radiologic Features
 Prognosis and Therapy
 Conclusions
 References
 
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. The term gastrointestinal stromal tumor defines a unique group of mesenchymal neoplasms that are distinct from true smooth muscle and neural tumors. The histogenesis of GISTs has been debated for over 50 years (1). Older medical literature referred to these tumors as smooth muscle tumors, leiomyomas, leiomyosarcomas, epithelioid leiomyosarcomas, and leiomyoblastomas because these tumors were believed to originate from the smooth muscle layers of the wall of the gastrointestinal tract. This variable nomenclature has led to considerable diagnostic confusion and is indicative of the diverse radiologic and histologic manifestations of mesenchymal neoplasms.

The best defining feature of GISTs is the expression of KIT (CD117), a tyrosine kinase growth factor receptor. Immunoreactivity for KIT distinguishes GISTs from true leiomyomas, leiomyosarcomas, schwannomas, and neurofibromas (2,3). The expression of KIT by GISTs has led several authors to postulate that GISTs arise from primitive stem cells that phenotypically resemble the native KIT-positive gut pacemaker cell or interstitial cell of Cajal (4,5). Not only is KIT immunoreactivity of useful diagnostic utility in diagnosing GISTs, but also more important, ligand-independent constitutive activation of KIT is central to the pathogenesis of GISTs. Pharmacologically targeting this receptor with a KIT-tyrosine kinase inhibitor (STI-571, Imatinib [Gleevec]; Novartis, Basel, Switzerland) has been shown to be of clinical utility in treating patients with GISTs.

GISTs, leiomyomas, and leiomyosarcomas are distinctly different neoplasms that arise with variable frequency throughout the gastrointestinal tract. GISTs are the most common and may occur from the esophagus to the anus. They may also occur primarily in the omentum, mesentery, and retroperitoneum. The esophagus is the only site where leiomyomas predominate (75% of esophageal mesenchymal tumors are leiomyomas; 25% are GISTs) and leiomyosarcomas are rare (6). In the stomach, small intestine, colon, and anorectum, GISTs account for almost all mesenchymal tumors, as leiomyomas and leiomyosarcomas in these sites are very rare (79).

The clinical manifestation of GISTs is highly variable. In some patients, small benign GISTs are discovered incidentally during radiologic evaluation or surgery for another condition. In contrast, other patients present with profound symptoms that reflect large or highly aggressive GISTs that invade adjacent organs and metastasize. As a result, GISTs have a wide spectrum of radiologic appearances. This article summarizes the current literature and our recent experience with 64 cases of GIST (28 gastric, 27 small intestinal, six anorectal, one colonic, one esophageal, and one from the small bowel mesentery) accessioned into the Radiologic Pathology Archives at the Armed Forces Institute of Pathology from April 1998 to May 2002. The clinical, pathologic, and radiologic spectrum of GISTs throughout the gastrointestinal tract, omentum, and mesentery is presented.


    Clinical Features
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 Prognosis and Therapy
 Conclusions
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The exact prevalence of GISTs is difficult to determine. Miettinen and Lasota (8) estimate the frequency of GISTs as 10–20 cases per million persons. No association between geographic location, ethnicity, race, or occupation has been established (10). Most individuals are over 50 years of age at the time of presentation, and GISTs are rarely seen in patients younger than 40 years of age (11). Although some studies in the literature show a slight male predominance, others show no gender predilection (1214). Patients with neurofibromatosis type 1 (NF1) have an increased prevalence of GISTs. Classically, patients with NF1 have multiple small intestinal GISTs (15, 16). GISTs are likely a feature of the Carney triad, which is a rare condition referring to the association of an epithelioid leiomyosarcoma with paraganglioma and pulmonary chondroma (17). Patients with KIT germ line mutations have an increased prevalence of GISTs (18).

Presenting signs and symptoms depend on the size and anatomic location of the tumor. GISTs most frequently occur in the stomach (70% of cases), followed by the small intestine (20%– 30%), anorectum (7%), colon, and esophagus (8). The most common clinical manifestation for symptomatic GISTs in the stomach, small intestine, colon, and anorectum is gastrointestinal bleeding from mucosal ulceration (19). Patients may present with hematemesis, melena, hematochezia, or signs and symptoms of anemia caused by occult bleeding. Other signs and symptoms include nausea, vomiting, abdominal pain, weight loss, abdominal distention, and intestinal obstruction. Occasionally, small asymptomatic GISTs are discovered incidentally during a radiologic evaluation or surgical procedure performed for other reasons. Asymptomatic anorectal tumors may be discovered as a palpable mass during routine digital rectal examination. The most common clinical manifestation for patients with esophageal GISTs is dysphagia (6). Less common manifestations for esophageal lesions include cough, gastrointestinal bleeding, and the incidental discovery of a posterior or middle mediastinal mass on chest radiographs.


    Pathologic Features
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 Pathologic Features
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Gross Pathologic Features
GISTs of the hollow gastrointestinal tract most commonly involve the muscularis propria of the intestinal wall. Mesenchymal tumors that involve the muscularis mucosae most frequently arise in the colon and occur as polyps. Such tumors are thought to uniformly represent true leiomyomas (20). Because GISTs usually involve the outer muscular layer, they have a propensity for exophytic growth (Fig 1a). Therefore, the most common appearance is that of a mass arising from the intestinal wall and projecting into the abdominal cavity (19). Often, a component of the tumor distends to the mucosal surface of the involved segment of intestine. Mucosal ulceration is seen on the luminal surface of the tumor in up to 50% of cases (Fig 1c) (19).



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Figure 1a.  Gross pathologic features of GISTs. (a) Photomicrograph (actual size [1:]; hematoxylin-eosin [H-E] stain) of resected jejunum from an 82-year-old man with gastrointestinal bleeding shows an intramural GIST with its origin in the muscularis propria (solid arrows). There is intact overlying normal small intestinal mucosa (open arrow). (b) Intraoperative photograph of the serosal surface of the ileum in a 70-year-old man who presented with melena shows a 6.0-cm pedunculated GIST. (c) Photograph of resected and opened jejunum from a 67-year-old man who presented with melena shows a 4.0-cm hemorrhagic GIST protruding into the intestinal lumen. The mucosa overlying the tumor is ulcerated (arrow). (d) Photograph of a bivalved resected 18-cm GIST from the ileum of a 66-year-old man who presented with weight loss shows a central cavity containing hemorrhage.

 


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Figure 1b.  Gross pathologic features of GISTs. (a) Photomicrograph (actual size [1:]; hematoxylin-eosin [H-E] stain) of resected jejunum from an 82-year-old man with gastrointestinal bleeding shows an intramural GIST with its origin in the muscularis propria (solid arrows). There is intact overlying normal small intestinal mucosa (open arrow). (b) Intraoperative photograph of the serosal surface of the ileum in a 70-year-old man who presented with melena shows a 6.0-cm pedunculated GIST. (c) Photograph of resected and opened jejunum from a 67-year-old man who presented with melena shows a 4.0-cm hemorrhagic GIST protruding into the intestinal lumen. The mucosa overlying the tumor is ulcerated (arrow). (d) Photograph of a bivalved resected 18-cm GIST from the ileum of a 66-year-old man who presented with weight loss shows a central cavity containing hemorrhage.

 


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Figure 1c.  Gross pathologic features of GISTs. (a) Photomicrograph (actual size [1:]; hematoxylin-eosin [H-E] stain) of resected jejunum from an 82-year-old man with gastrointestinal bleeding shows an intramural GIST with its origin in the muscularis propria (solid arrows). There is intact overlying normal small intestinal mucosa (open arrow). (b) Intraoperative photograph of the serosal surface of the ileum in a 70-year-old man who presented with melena shows a 6.0-cm pedunculated GIST. (c) Photograph of resected and opened jejunum from a 67-year-old man who presented with melena shows a 4.0-cm hemorrhagic GIST protruding into the intestinal lumen. The mucosa overlying the tumor is ulcerated (arrow). (d) Photograph of a bivalved resected 18-cm GIST from the ileum of a 66-year-old man who presented with weight loss shows a central cavity containing hemorrhage.

 


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Figure 1d.  Gross pathologic features of GISTs. (a) Photomicrograph (actual size [1:]; hematoxylin-eosin [H-E] stain) of resected jejunum from an 82-year-old man with gastrointestinal bleeding shows an intramural GIST with its origin in the muscularis propria (solid arrows). There is intact overlying normal small intestinal mucosa (open arrow). (b) Intraoperative photograph of the serosal surface of the ileum in a 70-year-old man who presented with melena shows a 6.0-cm pedunculated GIST. (c) Photograph of resected and opened jejunum from a 67-year-old man who presented with melena shows a 4.0-cm hemorrhagic GIST protruding into the intestinal lumen. The mucosa overlying the tumor is ulcerated (arrow). (d) Photograph of a bivalved resected 18-cm GIST from the ileum of a 66-year-old man who presented with weight loss shows a central cavity containing hemorrhage.

 
GISTs range in size from several millimeters to greater than 30 cm (19). They are typically well-circumscribed masses that compress adjacent tissue and lack a true capsule. Cut sections of specimens have a pink, tan, or gray surface (Fig 1c). Focal areas of hemorrhage, cystic degeneration, and necrosis may occur, particularly in large lesions. Cavities form from extensive hemorrhage or necrosis and may communicate with the intestinal lumen (Fig 1d). Aneurysmal dilatation of the involved segment of the colon is an uncommon feature of colonic GISTs (9).

Histologic Features
GISTs can be histologically classified by their predominant cell morphology, either spindle cell or epithelioid. The spindle cell morphology is present in 70%–80% of gastric GISTs, with the remaining 20%–30% having the epithelioid morphology (Fig 2) (11). In the older literature, the tumors with an epithelioid morphology were referred to as leiomyoblastomas or epithelioid leiomyosarcomas.



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Figure 2a.  Cytologic features of GISTs. (a) Photomicrograph (original magnification, x40; H-E stain) of a spindle cell GIST shows uniform cigar-shaped cells with elongated nuclei. (b) Photomicrograph (original magnification, x40; H-E stain) of an epithelioid GIST shows round cells with centrally placed nuclei. (c) Photomicrograph (original magnification, x40; H-E stain) shows the signet ring appearance from prominent cytoplasmic vacuolization in a GIST.

 


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Figure 2b.  Cytologic features of GISTs. (a) Photomicrograph (original magnification, x40; H-E stain) of a spindle cell GIST shows uniform cigar-shaped cells with elongated nuclei. (b) Photomicrograph (original magnification, x40; H-E stain) of an epithelioid GIST shows round cells with centrally placed nuclei. (c) Photomicrograph (original magnification, x40; H-E stain) shows the signet ring appearance from prominent cytoplasmic vacuolization in a GIST.

 


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Figure 2c.  Cytologic features of GISTs. (a) Photomicrograph (original magnification, x40; H-E stain) of a spindle cell GIST shows uniform cigar-shaped cells with elongated nuclei. (b) Photomicrograph (original magnification, x40; H-E stain) of an epithelioid GIST shows round cells with centrally placed nuclei. (c) Photomicrograph (original magnification, x40; H-E stain) shows the signet ring appearance from prominent cytoplasmic vacuolization in a GIST.

 
Spindle cell GISTs are composed of cigar-shaped cells with elongated nuclei and eosinophilic to basophilic cytoplasm (Fig 2a). Epithelioid GISTs are composed of round or polygonal cells with centrally placed nuclei (Fig 2b) (19). Fixation artifact may cause clear perinuclear halos or cytoplasmic vacuolization. Prominent cytoplasmic vacuolization may create a signet ring cell appearance caused by displacement of the nuclei to the periphery of the cell (Fig 2c). GISTs may display a variety of architectural patterns. Spindle cell GISTs may be arranged in bundles of interlacing fascicles resembling smooth muscle tumors or a nuclear palisading pattern resembling nerve sheath tumors (Fig 3a, 3b). GISTs may also display a pattern with prominent vascularity (Fig 3c). Occasionally, GISTs are composed of uniform small round cells that display a nesting organoid pattern that resembles neuroendocrine tumors. The stromal portions of the tumor may show extensive perivascular or stromal hyalinization, myxoid change, or hemorrhage (Fig 4).



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Figure 3a.  Architectural patterns of GISTs. (a) Photomicrograph (original magnification, x10; H-E stain) of a spindle cell GIST shows a pattern of interlacing fascicles. (b) Photomicrograph (original magnification, x10; H-E stain) of a spindle cell GIST shows a nuclear palisading pattern. (c) Photomicrograph (original magnification, x10; H-E stain) of a spindle cell GIST shows an angiomatoid pattern with large blood-filled vascular spaces within the tumor.

 


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Figure 3b.  Architectural patterns of GISTs. (a) Photomicrograph (original magnification, x10; H-E stain) of a spindle cell GIST shows a pattern of interlacing fascicles. (b) Photomicrograph (original magnification, x10; H-E stain) of a spindle cell GIST shows a nuclear palisading pattern. (c) Photomicrograph (original magnification, x10; H-E stain) of a spindle cell GIST shows an angiomatoid pattern with large blood-filled vascular spaces within the tumor.

 


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Figure 3c.  Architectural patterns of GISTs. (a) Photomicrograph (original magnification, x10; H-E stain) of a spindle cell GIST shows a pattern of interlacing fascicles. (b) Photomicrograph (original magnification, x10; H-E stain) of a spindle cell GIST shows a nuclear palisading pattern. (c) Photomicrograph (original magnification, x10; H-E stain) of a spindle cell GIST shows an angiomatoid pattern with large blood-filled vascular spaces within the tumor.

 


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Figure 4a.  Stromal features of GISTs. (a) Photomicrograph (original magnification, x10; H-E stain) shows a GIST that has extensive myxoid stroma with interspersed tumor cells. (b) Photomicrograph (original magnification, x10; H-E stain) of a spindle cell GIST shows perivascular hyalinization.

 


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Figure 4b.  Stromal features of GISTs. (a) Photomicrograph (original magnification, x10; H-E stain) shows a GIST that has extensive myxoid stroma with interspersed tumor cells. (b) Photomicrograph (original magnification, x10; H-E stain) of a spindle cell GIST shows perivascular hyalinization.

 
Small intestinal GISTs are most often spindle cell tumors. They often contain extracellular collagenous skeinoid fibers (Fig 5) (21). Although the majority of anorectal, colonic, and esophageal GISTs are of the spindle cell type, epithelioid GISTs may occasionally be seen in these sites (6,9,22). Tumors may show an admixture of spindle and epithelioid elements.



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Figure 5.  Photomicrograph of a small intestinal GIST (original magnification, x80; H-E stain) shows eosinophilic skeinoid fibers (arrow).

 
In general, malignant GISTs are larger, more highly cellular, and more mitotically active than their benign counterparts (Fig 6). In the stomach, benign GISTs are three times more common than malignant ones (8). It is generally accepted that gastric GISTs less than 5 cm in largest dimension and with five or fewer mitoses per 50 consecutive high power fields (HPF) have low risk for metastasis and are probably benign. Gastric GISTs larger than 10 cm and with more than five mitoses per 50 HPF are considered malignant, whereas those that fall between these categories have uncertain malignant potential or intermediate risk for metastasis or recurrence. GISTs with marked mitotic activity (more than 50 mitoses per 50 HPF) are considered high-grade malignancies with an extremely aggressive clinical behavior (23). Small intestinal GISTs may have a more aggressive course compared with that of gastric GISTs of the same size. Therefore, the size threshold for estimating recurrent or metastatic risk in small intestinal GISTs may be smaller than that for gastric GISTs. The majority of esophageal, colonic, and anorectal GISTs are malignant.



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Figure 6a.  Cytologic features as a reflection of biologic potential. (a) Photomicrograph (original magnification, x40; H-E stain) of a low-risk or probably benign GIST shows bland-appearing tumor cells and absent mitotic activity. (b) Photomicrograph (original magnification, x40; H-E stain) of an intermediate-risk or low-grade malignant GIST shows a more cellular tumor with higher nuclear-cytoplasmic ratio. (c) Photomicrograph (original magnification, x40; H-E stain) of a high-risk or highly malignant GIST shows increased cellularity, high nuclear-cytoplasmic ratio, and numerous mitoses (arrows).

 


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Figure 6b.  Cytologic features as a reflection of biologic potential. (a) Photomicrograph (original magnification, x40; H-E stain) of a low-risk or probably benign GIST shows bland-appearing tumor cells and absent mitotic activity. (b) Photomicrograph (original magnification, x40; H-E stain) of an intermediate-risk or low-grade malignant GIST shows a more cellular tumor with higher nuclear-cytoplasmic ratio. (c) Photomicrograph (original magnification, x40; H-E stain) of a high-risk or highly malignant GIST shows increased cellularity, high nuclear-cytoplasmic ratio, and numerous mitoses (arrows).

 


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Figure 6c.  Cytologic features as a reflection of biologic potential. (a) Photomicrograph (original magnification, x40; H-E stain) of a low-risk or probably benign GIST shows bland-appearing tumor cells and absent mitotic activity. (b) Photomicrograph (original magnification, x40; H-E stain) of an intermediate-risk or low-grade malignant GIST shows a more cellular tumor with higher nuclear-cytoplasmic ratio. (c) Photomicrograph (original magnification, x40; H-E stain) of a high-risk or highly malignant GIST shows increased cellularity, high nuclear-cytoplasmic ratio, and numerous mitoses (arrows).

 
Immunohistochemical Features
By definition, GISTs are positive for KIT (CD117) (Fig 7). KIT (CD117) is a transmembrane receptor for a growth factor called stem cell factor that is normally expressed on hematopoietic stem cells, germ cells, mast cells, melanocytes, and the myenteric plexus of the normal adult gastrointestinal tract (3,24). Approximately 70% of GISTs coexpress CD34 (2,3). CD34 is   a hematopoietic progenitor cell antigen that is typically expressed in normal and neoplastic endothelial cells and in some fibroblasts and their neoplasms (25,26). GISTs may also be positive for smooth muscle actin and rarely for desmin and S-100 protein. The current consensus opinion of pathologists working in the field is that the term GIST applies only to gastrointestinal mesenchymal neoplasms with KIT immunoreactivity, with only rare exceptions (27).



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Figure 7.  KIT immunoreactivity. Photomicrograph (original magnification, x20; KIT [CD117] stain) of a GIST shows that the cytoplasm of the tumor cells stains brown, indicating immunoreactivity.

 

    Radiologic Features
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 Abstract
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 Introduction
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 Pathologic Features
 Radiologic Features
 Prognosis and Therapy
 Conclusions
 References
 
Stomach
The stomach is the most common location for GISTs, which make up 2%–3% of all gastric tumors. In our series of 64 GISTs, 28 (44%) were located in the stomach. Four (14%) of our cases were confined to the cardia and fundus of the stomach, 21 (75%) were located in the body, and three (11%) were in the antrum. The tumors ranged from 4 to 25 cm in maximal dimension. The mean tumor dimension was 10.8 cm. On the basis of recently published criteria on tumor size and mitotic rate (28), three cases were classified as probably benign, nine uncertain malignant potential, and 16 malignant. No correlation between radiologic appearance and malignant potential could be established with regard to the degree of necrosis, hemorrhage, cyst formation, or contrast material enhancement on computed tomographic (CT) images.

Abdominal radiography may show a nonspecific soft-tissue mass indenting or displacing the gastric air shadow (Fig 8). Rarely, calcification may occur and be visible on abdominal radiographs. In barium studies of the stomach, GISTs have the classic features of submucosal masses, similar to those of leiomyomas and leiomyosarcomas (29). The margin of the lesion forms obtuse or right angles with the gastric wall when viewed in profile, and the masses are smoothly circumscribed when viewed en face (Fig 9). They have a smooth mucosal surface when coated with barium, and the overlying mucosal surface is generally intact with the exception of focal areas of ulceration, which can be seen in 60% of cases (29). A focal intraluminal polypoid mass resembling a mucosal polyp is the least common appearance of GISTs on barium images of the stomach. A polypoid intraluminal component was present in only four (14%) of our cases.



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Figure 8a.  GIST arising from the stomach in a 66-year-old woman with left-sided upper abdominal pain. (a) Chest radiograph shows elevation of the left hemidiaphragm and a mass of soft-tissue opacity that displaces an irregular gas collection away from the diaphragm (arrow). (b, c) Contrast material-enhanced CT scans (c obtained at a lower level than b) show the subdiaphragmatic cavitary mass of heterogeneous attenuation. The cavity (*) is air-filled. The mass originates from the gastric wall (arrow). There is a metastatic lesion within the liver.

 


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Figure 8b.  GIST arising from the stomach in a 66-year-old woman with left-sided upper abdominal pain. (a) Chest radiograph shows elevation of the left hemidiaphragm and a mass of soft-tissue opacity that displaces an irregular gas collection away from the diaphragm (arrow). (b, c) Contrast material-enhanced CT scans (c obtained at a lower level than b) show the subdiaphragmatic cavitary mass of heterogeneous attenuation. The cavity (*) is air-filled. The mass originates from the gastric wall (arrow). There is a metastatic lesion within the liver.

 


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Figure 8c.  GIST arising from the stomach in a 66-year-old woman with left-sided upper abdominal pain. (a) Chest radiograph shows elevation of the left hemidiaphragm and a mass of soft-tissue opacity that displaces an irregular gas collection away from the diaphragm (arrow). (b, c) Contrast material-enhanced CT scans (c obtained at a lower level than b) show the subdiaphragmatic cavitary mass of heterogeneous attenuation. The cavity (*) is air-filled. The mass originates from the gastric wall (arrow). There is a metastatic lesion within the liver.

 


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Figure 9a.  Features of GISTs seen during upper gastrointestinal tract series. (a) Anteroposterior view of the stomach from a barium study in a 67-year-old man shows a smoothly circumscribed mass in the body of the stomach viewed en face (arrows). (b) Oblique view of the stomach from a barium study in a 67-year-old woman shows a smoothly marginated, mural-based mass that forms obtuse angles with the gastric wall.

 


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Figure 9b.  Features of GISTs seen during upper gastrointestinal tract series. (a) Anteroposterior view of the stomach from a barium study in a 67-year-old man shows a smoothly circumscribed mass in the body of the stomach viewed en face (arrows). (b) Oblique view of the stomach from a barium study in a 67-year-old woman shows a smoothly marginated, mural-based mass that forms obtuse angles with the gastric wall.

 
CT showed an intramural component in all 28 cases of gastric GIST that we reviewed. Extragastric extension was present in 86% of cases. Extension may occur into the gastrohepatic ligament, into the gastrosplenic ligament, or posteriorly into the lesser sac. In many cases, the bulk of the tumor will be in an extragastric location, which makes it difficult to appreciate the origin of the tumor from the gastric wall on CT images. The tumor may be attached to the gastric wall by a thin pedicle. Careful evaluation of the gastric wall in these cases may reveal subtle wall thickening that will help establish the stomach as the origin of the mass (Fig 10).



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Figure 10a.  GIST arising from the stomach in a 65-year-old man with left-sided upper abdominal pain. (a) Contrast-enhanced CT scan shows a large mass arising from the posterior gastric wall (arrow) that extends into the gastrosplenic ligament. There are areas of low attenuation within the mass. (b) Photograph of the cut surface of the resected specimen shows areas of hemorrhage and necrosis within the tumor.

 


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Figure 10b.  GIST arising from the stomach in a 65-year-old man with left-sided upper abdominal pain. (a) Contrast-enhanced CT scan shows a large mass arising from the posterior gastric wall (arrow) that extends into the gastrosplenic ligament. There are areas of low attenuation within the mass. (b) Photograph of the cut surface of the resected specimen shows areas of hemorrhage and necrosis within the tumor.

 
A peripheral enhancement pattern was present in the majority (92%) of our cases on intravenous contrast-enhanced CT images. Correlation of this appearance with gross pathologic findings demonstrates that this pattern represents enhancement of peripheral areas of viable tumor. Central areas of low attenuation correspond to hemorrhage, necrosis, or cyst formation (Fig 10) (30). Homogeneous enhancement was present in a minority (8%) of cases. Lesions with extensive hemorrhage or necrosis may form large cystic spaces or cavities. The cavities may communicate with the gastric lumen and contain air, air-fluid levels, or oral contrast media (Fig 11) (31). Calcification is an unusual feature of GISTs, seen in only one (3%) of our gastric cases. It may occur in a mottled pattern or be present extensively throughout the tumor (Fig 12). CT may also demonstrate evidence of adjacent organ invasion, ascites, omental and peritoneal spread of tumor, or liver metastasis. Metastatic lymphadenopathy is not a feature in patients with GISTs.



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Figure 11a.  GIST arising from the lesser curvature of the stomach in a 55-year-old man with melena and hematemesis. (a, b) Contrast-enhanced CT scans (b obtained at a lower level than a) show a cavitary mass that extends into the gastrohepatic and gastrosplenic ligaments. The cavity contains air (solid straight arrow) and oral contrast material (curved arrow). Low-attenuation areas in the tumor (open arrow) represent hemorrhage. (c) Photograph of the bivalved resected specimen shows a cavity (*) and areas of hemorrhage (arrow) in the solid portions of the tumor.

 


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Figure 11b.  GIST arising from the lesser curvature of the stomach in a 55-year-old man with melena and hematemesis. (a, b) Contrast-enhanced CT scans (b obtained at a lower level than a) show a cavitary mass that extends into the gastrohepatic and gastrosplenic ligaments. The cavity contains air (solid straight arrow) and oral contrast material (curved arrow). Low-attenuation areas in the tumor (open arrow) represent hemorrhage. (c) Photograph of the bivalved resected specimen shows a cavity (*) and areas of hemorrhage (arrow) in the solid portions of the tumor.

 


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Figure 11c.  GIST arising from the lesser curvature of the stomach in a 55-year-old man with melena and hematemesis. (a, b) Contrast-enhanced CT scans (b obtained at a lower level than a) show a cavitary mass that extends into the gastrohepatic and gastrosplenic ligaments. The cavity contains air (solid straight arrow) and oral contrast material (curved arrow). Low-attenuation areas in the tumor (open arrow) represent hemorrhage. (c) Photograph of the bivalved resected specimen shows a cavity (*) and areas of hemorrhage (arrow) in the solid portions of the tumor.

 


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Figure 12a.  GIST arising from the posterior stomach in a 72-year-old woman with back pain. Contrast-enhanced CT scans (b obtained at a lower level than a) show a soft-tissue attenuation mass arising from the posterior wall of the stomach (straight arrow). The mass extends into the gastrosplenic ligament and contains extensive dense calcification (curved arrow).

 


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Figure 12b.  GIST arising from the posterior stomach in a 72-year-old woman with back pain. Contrast-enhanced CT scans (b obtained at a lower level than a) show a soft-tissue attenuation mass arising from the posterior wall of the stomach (straight arrow). The mass extends into the gastrosplenic ligament and contains extensive dense calcification (curved arrow).

 
Magnetic resonance (MR) imaging features of gastric GISTs are variable. The degree of necrosis and hemorrhage greatly affects the signal-intensity pattern. The solid portions of tumor are typically low signal intensity on T1-weighted images, are high signal intensity on T2-weighted images, and enhance after administration of gadolinium. Areas of hemorrhage within the tumor will vary from high to low signal intensity on both T1- and T2-weighted images, depending on the age of the hemorrhage (32). MR imaging is a useful adjunct to CT, particularly in the evaluation of large tumors. The multiplanar capability of MR imaging may be helpful in determining the organ of origin in large tumors and the relationship of the tumor to other organs and major blood vessels (Fig 13).



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Figure 13a.  Gastric GIST in a 66-year-old woman with left-sided upper abdominal pain. (a) Axial T1-weighted MR image shows a subdiaphragmatic hypointense mass containing a cavity (*) in the left side of the upper abdomen. (b) On the T2-weighted image, the mass increases in signal intensity. Areas of focal high-signal-intensity hemorrhage are present within the mass (arrow). (c) Coronal T2-weighted image shows that the mass originates from the gastric fundus (arrow).

 


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Figure 13b.  Gastric GIST in a 66-year-old woman with left-sided upper abdominal pain. (a) Axial T1-weighted MR image shows a subdiaphragmatic hypointense mass containing a cavity (*) in the left side of the upper abdomen. (b) On the T2-weighted image, the mass increases in signal intensity. Areas of focal high-signal-intensity hemorrhage are present within the mass (arrow). (c) Coronal T2-weighted image shows that the mass originates from the gastric fundus (arrow).

 


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Figure 13c.  Gastric GIST in a 66-year-old woman with left-sided upper abdominal pain. (a) Axial T1-weighted MR image shows a subdiaphragmatic hypointense mass containing a cavity (*) in the left side of the upper abdomen. (b) On the T2-weighted image, the mass increases in signal intensity. Areas of focal high-signal-intensity hemorrhage are present within the mass (arrow). (c) Coronal T2-weighted image shows that the mass originates from the gastric fundus (arrow).

 
The differential diagnosis for gastric GISTs includes other mesenchymal neoplasms such as true leiomyomas, leiomyosarcomas, schwannomas, neurofibromas, and neuroendocrine neoplasms (eg, solitary gastric carcinoids). Because all of these neoplasms arise in the gastric wall, their imaging features may be similar to those of GISTs. True leiomyomas and leiomyosarcomas occur infrequently in the stomach. Likewise, schwannomas occur in the stomach with much less frequency than GISTs but have similar radiologic features (Fig 14). Schwannomas are histologically characterized by bundled spindle-shaped cells and often have a distinctive lymphoid cuff that may contain germinal centers (Fig 14d). They stain positive for S-100 protein. Solitary gastric carcinoids are most commonly seen in the antrum and characteristically have a central ulceration.



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Figure 14a.  Gastric schwannoma in a 69-year-old woman with postprandial epigastric pain. (a) Image from an upper gastrointestinal tract series shows a mural-based mass along the lesser curvature of the stomach (arrows). (b) Contrast-enhanced CT scan shows the homogeneous low-attenuation gastric mass (S) extending into the gastrohepatic ligament. (c) Photograph of the cut surface of the resected specimen shows a yellow tumor in the gastric wall with no evidence of hemorrhage or necrosis. Scale is in centimeters. (d) Photomicrograph (original magnification, x2; H-E stain) shows the tumor, which is composed of spindle-shaped cells arising from the muscularis propria. There is a peripheral lymphoid cuff (arrows) within the tumor.

 


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Figure 14b.  Gastric schwannoma in a 69-year-old woman with postprandial epigastric pain. (a) Image from an upper gastrointestinal tract series shows a mural-based mass along the lesser curvature of the stomach (arrows). (b) Contrast-enhanced CT scan shows the homogeneous low-attenuation gastric mass (S) extending into the gastrohepatic ligament. (c) Photograph of the cut surface of the resected specimen shows a yellow tumor in the gastric wall with no evidence of hemorrhage or necrosis. Scale is in centimeters. (d) Photomicrograph (original magnification, x2; H-E stain) shows the tumor, which is composed of spindle-shaped cells arising from the muscularis propria. There is a peripheral lymphoid cuff (arrows) within the tumor.

 


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Figure 14c.  Gastric schwannoma in a 69-year-old woman with postprandial epigastric pain. (a) Image from an upper gastrointestinal tract series shows a mural-based mass along the lesser curvature of the stomach (arrows). (b) Contrast-enhanced CT scan shows the homogeneous low-attenuation gastric mass (S) extending into the gastrohepatic ligament. (c) Photograph of the cut surface of the resected specimen shows a yellow tumor in the gastric wall with no evidence of hemorrhage or necrosis. Scale is in centimeters. (d) Photomicrograph (original magnification, x2; H-E stain) shows the tumor, which is composed of spindle-shaped cells arising from the muscularis propria. There is a peripheral lymphoid cuff (arrows) within the tumor.

 


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Figure 14d.  Gastric schwannoma in a 69-year-old woman with postprandial epigastric pain. (a) Image from an upper gastrointestinal tract series shows a mural-based mass along the lesser curvature of the stomach (arrows). (b) Contrast-enhanced CT scan shows the homogeneous low-attenuation gastric mass (S) extending into the gastrohepatic ligament. (c) Photograph of the cut surface of the resected specimen shows a yellow tumor in the gastric wall with no evidence of hemorrhage or necrosis. Scale is in centimeters. (d) Photomicrograph (original magnification, x2; H-E stain) shows the tumor, which is composed of spindle-shaped cells arising from the muscularis propria. There is a peripheral lymphoid cuff (arrows) within the tumor.

 
Gastric adenocarcinoma and lymphoma rarely demonstrate marked exophytic growth. However, they may occasionally have a radiologic appearance similar to that of GISTs—that is, a predominantly mural location or an intraluminal component. Advanced gastric carcinomas and lymphomas commonly have associated perigastric, hepatoduodenal ligament, and celiac lymphadenopathy, which are not seen in malignant GISTs. Lymphoma may be associated with bulky adenopathy or adenopathy that extends into the lower abdomen and pelvis. Adenopathy is not usually observed in cases of gastric GISTs.

Small Intestine
GISTs may occur throughout the small intestine. Of the 27 small intestinal GISTs in our series, eight were located in the duodenum, 12 in the jejunum, six in the ileum, and one at the jejunoileal junction. The tumors ranged from 2.2 to 21 cm in maximal dimension, with a mean size of 8.6 cm.

In those patients who presented with signs and symptoms of small intestinal obstruction, abdominal radiography showed evidence of small intestinal dilatation or a soft-tissue mass (Fig 15). Irregular gas collections were evident on abdominal radiographs in those patients who had cavitary masses containing air (Fig 16).



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Figure 15a.  Small intestinal GIST in a 93-year-old woman with acute abdominal pain. (a) Abdominal radiograph shows a small bowel obstruction and a mass of soft-tissue opacity in the right lower quadrant. Intestinal gas is absent. (b) Unenhanced CT scan shows dilated segments of small intestine and the well-circumscribed, low-attenuation mass in the right side of the pelvis. (c) Intraoperative photograph shows the 20-cm mass arising from the jejunum that had undergone torsion, which resulted in small bowel obstruction.

 


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Figure 15b.  Small intestinal GIST in a 93-year-old woman with acute abdominal pain. (a) Abdominal radiograph shows a small bowel obstruction and a mass of soft-tissue opacity in the right lower quadrant. Intestinal gas is absent. (b) Unenhanced CT scan shows dilated segments of small intestine and the well-circumscribed, low-attenuation mass in the right side of the pelvis. (c) Intraoperative photograph shows the 20-cm mass arising from the jejunum that had undergone torsion, which resulted in small bowel obstruction.

 


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Figure 15c.  Small intestinal GIST in a 93-year-old woman with acute abdominal pain. (a) Abdominal radiograph shows a small bowel obstruction and a mass of soft-tissue opacity in the right lower quadrant. Intestinal gas is absent. (b) Unenhanced CT scan shows dilated segments of small intestine and the well-circumscribed, low-attenuation mass in the right side of the pelvis. (c) Intraoperative photograph shows the 20-cm mass arising from the jejunum that had undergone torsion, which resulted in small bowel obstruction.

 


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Figure 16.  Small intestinal GIST in a 54-year-old woman with a palpable abdominal mass. Abdominal radiograph obtained with the patient supine shows a soft-tissue mass in the left side of the midabdomen. The mass contains an irregular collection of air (arrows).

 
Barium studies of the small intestine may reveal an intraluminal or submucosal mass. The margins are typically sharply defined. However, the mucosal surface may show luminal irregularity or focal ulceration (Fig 17). As with gastric GISTs, many intestinal tumors often have an extraserosal component. These tumors may exhibit significant mass effect on the affected segment of intestine or adjacent segments. Cavity and fistula formation may occur, resulting in luminal enlargement and communication of the cavity or fistula with the intestinal lumen (Fig 18).



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Figure 17a.  Small intestinal GIST in a 28-year-old man who presented with melena. (a) Image from an enteroclysis study shows a smoothly circumscribed, 3-cm mural mass with central ulceration in the proximal jejunum (arrow). (b) Photograph from enteroscopy shows the ulcerated mass in the proximal jejunum (arrows).

 


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Figure 17b.  Small intestinal GIST in a 28-year-old man who presented with melena. (a) Image from an enteroclysis study shows a smoothly circumscribed, 3-cm mural mass with central ulceration in the proximal jejunum (arrow). (b) Photograph from enteroscopy shows the ulcerated mass in the proximal jejunum (arrows).

 


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Figure 18a.  Small intestinal GIST in a 56-year-old man who presented with fatigue, weight loss, and melena. (a) Image from a barium study of the small intestine shows barium extending from the intestinal lumen into a cavity (arrows). There is mass effect on adjacent segments of small intestine. (b) Contrast-enhanced CT scan shows a cavitary mass in the pelvis that contains air and oral contrast material (arrows).

 


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Figure 18b.  Small intestinal GIST in a 56-year-old man who presented with fatigue, weight loss, and melena. (a) Image from a barium study of the small intestine shows barium extending from the intestinal lumen into a cavity (arrows). There is mass effect on adjacent segments of small intestine. (b) Contrast-enhanced CT scan shows a cavitary mass in the pelvis that contains air and oral contrast material (arrows).

 
The intraluminal, mural, and extraserosal components of small intestinal GISTs are well depicted on CT images. GISTs may appear as an intramural mass or intraluminal polyp (Fig 19) (33). Six (22%) of the small intestinal GISTs in our series were primarily in an extraserosal location such that a small bowel origin was not readily evident at CT. Following intravenous administration of contrast media, GISTs are typically enhancing masses with areas of low attenuation from hemorrhage, necrosis, or cyst formation (34). A homogeneous pattern of attenuation is less common and was present in four of our cases. Extension into the adjacent small bowel mesentery and encasement of noncontiguous segments of small intestine, colon, bladder, ureter, and abdominal wall may occur (Fig 19c) (14). Patients with malignant GISTs may present with metastases to the liver, omentum, and peritoneum (14). The MR imaging appearance of small intestinal GISTs is very similar to that of gastric GISTs. The pattern of signal intensity is variable depending on the degree of hemorrhage and necrosis. Gadolinium enhancement in areas of viable tumor helps delineate areas of necrosis.



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Figure 19a.  CT features of small intestinal GISTs. (a) Unenhanced CT scan of a 77-year-old man shows an intraluminal polypoid small intestinal mass (arrow) that was incidentally discovered when he was being evaluated for an abdominal aortic aneurysm. (b) Contrast-enhanced CT scan of a 62-year-old woman with melena shows a heterogeneously enhancing mass in the posterior pelvis (arrow) that is arising from the distal ileum. (c) Contrast-enhanced CT scan of a 67-year-old man who presented with melena and a history of gastrointestinal bleeding shows a soft-tissue attenuation mass in the distal jejunum. The mass has an intraluminal component (arrow) and an extraluminal component, which invades an adjacent segment of small intestine. (d) Contrast-enhanced CT scan of a 66-year-old man with abdominal pain shows a cavitary mass in the right side of the midabdomen that contains air, debris, and oral contrast material.

 


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Figure 19b.  CT features of small intestinal GISTs. (a) Unenhanced CT scan of a 77-year-old man shows an intraluminal polypoid small intestinal mass (arrow) that was incidentally discovered when he was being evaluated for an abdominal aortic aneurysm. (b) Contrast-enhanced CT scan of a 62-year-old woman with melena shows a heterogeneously enhancing mass in the posterior pelvis (arrow) that is arising from the distal ileum. (c) Contrast-enhanced CT scan of a 67-year-old man who presented with melena and a history of gastrointestinal bleeding shows a soft-tissue attenuation mass in the distal jejunum. The mass has an intraluminal component (arrow) and an extraluminal component, which invades an adjacent segment of small intestine. (d) Contrast-enhanced CT scan of a 66-year-old man with abdominal pain shows a cavitary mass in the right side of the midabdomen that contains air, debris, and oral contrast material.

 


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Figure 19c.  CT features of small intestinal GISTs. (a) Unenhanced CT scan of a 77-year-old man shows an intraluminal polypoid small intestinal mass (arrow) that was incidentally discovered when he was being evaluated for an abdominal aortic aneurysm. (b) Contrast-enhanced CT scan of a 62-year-old woman with melena shows a heterogeneously enhancing mass in the posterior pelvis (arrow) that is arising from the distal ileum. (c) Contrast-enhanced CT scan of a 67-year-old man who presented with melena and a history of gastrointestinal bleeding shows a soft-tissue attenuation mass in the distal jejunum. The mass has an intraluminal component (arrow) and an extraluminal component, which invades an adjacent segment of small intestine. (d) Contrast-enhanced CT scan of a 66-year-old man with abdominal pain shows a cavitary mass in the right side of the midabdomen that contains air, debris, and oral contrast material.

 


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Figure 19d.  CT features of small intestinal GISTs. (a) Unenhanced CT scan of a 77-year-old man shows an intraluminal polypoid small intestinal mass (arrow) that was incidentally discovered when he was being evaluated for an abdominal aortic aneurysm. (b) Contrast-enhanced CT scan of a 62-year-old woman with melena shows a heterogeneously enhancing mass in the posterior pelvis (arrow) that is arising from the distal ileum. (c) Contrast-enhanced CT scan of a 67-year-old man who presented with melena and a history of gastrointestinal bleeding shows a soft-tissue attenuation mass in the distal jejunum. The mass has an intraluminal component (arrow) and an extraluminal component, which invades an adjacent segment of small intestine. (d) Contrast-enhanced CT scan of a 66-year-old man with abdominal pain shows a cavitary mass in the right side of the midabdomen that contains air, debris, and oral contrast material.

 
The differential diagnosis for small intestinal GISTs includes primary and metastatic small intestinal neoplasms. Adenocarcinoma is the most common primary malignancy of the small bowel. It typically manifests as an annular lesion in the proximal small intestine; thus, its appearance usually does not overlap with that of GISTs. Lymphoma, however, has many features similar to those of GISTs. Lymphoma produces large masses within the small intestine that may ulcerate, cavitate, and extend into the adjacent mesentery. In these cases, lymphoma may be indistinguishable from a GIST on radiologic images. The presence of associated lymphadenopathy, however, would favor the diagnosis of lymphoma. Neoplasms that originate from the mesentery and secondarily involve the small intestine should also be included in the differential diagnosis. These tumors include mesenteric fibromatosis (desmoid tumor), inflammatory pseudotumor, lymphoma, sclerosing mesenteritis, and metastatic disease.

Anorectum
Six (9%) of the 64 GISTs in our series were located in the anorectal region. Anorectal GISTs are most commonly mural masses that expand the rectal wall. As a result, a focal well-circumscribed mural mass is the most common finding on CT images (Fig 20). Mucosal ulceration may be present. External spread frequently occurs with extension of the mass into the ischiorectal fossa, prostate, or vagina (Fig 21) (35). The least common appearance is a focal intraluminal polypoid mass (22).



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Figure 20a.  Rectal GIST in a 69-year-old woman with pelvic pain and hematochezia. (a) CT scan shows a mural mass along the left anterolateral rectal wall (arrow). (b) Photograph of the opened abdominoperineal resected specimen and bivalved GIST shows the tumor in the distal rectum. Multiple foci of hemorrhage are present within the tumor.

 


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Figure 20b.  Rectal GIST in a 69-year-old woman with pelvic pain and hematochezia. (a) CT scan shows a mural mass along the left anterolateral rectal wall (arrow). (b) Photograph of the opened abdominoperineal resected specimen and bivalved GIST shows the tumor in the distal rectum. Multiple foci of hemorrhage are present within the tumor.

 


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Figure 21.  Rectal GIST in a 55-year-old man with rectal pain. CT scan shows a 5-cm mass (arrows) arising from the posterior rectal wall and extending into the ischiorectal fossa.

 
The CT attenuation of anorectal GISTs is similar to that of GISTs in other locations of the gastrointestinal tract. Low-attenuation areas of hemorrhage are commonly present. On T1-weighted MR images, anorectal GISTs have uniform, intermediate signal intensity; on T2-weighted images they have heterogeneous high signal intensity, with heterogeneous enhancement following gadolinium administration (36).

The differential diagnosis for anorectal GISTs includes both epithelial and nonepithelial neoplasms of the anorectal region. Rectal adenocarcinoma, anal squamous cell carcinoma, lymphoma, malignant melanoma, carcinoid, leiomyoma, and leiomyosarcoma may have imaging appearances similar to that of GISTs, although leiomyosarcoma may have a dominant polypoid intraluminal component (22). Carcinomas tend to have irregular margins and may be associated with perirectal lymphadenopathy, whereas GISTs tend to have well-defined margins and lack perirectal adenopathy. GISTs that have significant perirectal extension may be mistaken as tumor arising from adjacent structures such as prostatic adenocarcinoma or sarcomas of the prostate and perineum. Anorectal lymphoma is seen in patients with AIDS (acquired immunodeficiency syndrome) and manifests radiographically as an eccentric or annular mural mass that may be associated with mucosal ulceration or perianal fistulization.

Colon
Primary colonic GISTs are much less common than gastric, small intestinal, and anorectal GISTs, although GISTs metastatic from other sites commonly involve the external aspect of the colon. We had only one case of a colonic GIST in our series. In the literature, they are described as transmural tumors that involve the intraluminal and extraserosal surfaces of the colon (9). They may be smooth or multinodular in contour and may contain central areas of hemorrhage, cystic change, necrosis, or calcification. A circumferential growth pattern with aneurysmal dilatation of the affected colonic segment has been observed in a colonic GIST (9).

The radiographic and cross-sectional imaging appearances of colonic GISTs are similar to those of leiomyosarcomas (37). Small lesions are typically confined to the wall of the colon and appear as mural or submucosal masses at barium examination. Mucosal ulceration may be present. The lesion in our series was a 10-cm homogeneously enhancing mass that extended beyond the serosal surface of the colon on CT images (Fig 22).



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Figure 22a.  Colonic GIST in a 47-year-old woman with a small bowel obstruction. (a) CT scan shows a lobulated 10-cm mass obstructing the ascending colon (arrow). The mass shows heterogeneous enhancement with intravenous contrast material. There are dilated, fluid-filled segments of small intestine, and a small amount of fluid is in the adjacent right paracolic gutter. (b) Photograph of the right hemicolectomy specimen shows the GIST at the level of the hepatic flexure (arrows). C = cecum, TI = terminal ileum.

 


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Figure 22b.  Colonic GIST in a 47-year-old woman with a small bowel obstruction. (a) CT scan shows a lobulated 10-cm mass obstructing the ascending colon (arrow). The mass shows heterogeneous enhancement with intravenous contrast material. There are dilated, fluid-filled segments of small intestine, and a small amount of fluid is in the adjacent right paracolic gutter. (b) Photograph of the right hemicolectomy specimen shows the GIST at the level of the hepatic flexure (arrows). C = cecum, TI = terminal ileum.

 
The radiologic differential diagnosis for colonic GISTs includes adenocarcinoma, lymphoma, metastatic melanoma, and leiomyosarcoma. Retroperitoneal sarcomas such as malignant fibrous histiocytoma, fibrosarcoma, and liposarcoma arising adjacent to the colon may appear to have a colonic origin and may also be confused with a GIST.

Esophagus
Esophageal GISTs are relatively uncommon, and we had only one case of an esophageal GIST in our series. GISTs accounted for approximately 25% of esophageal mesenchymal neoplasms studied by Miettinen et al (6). In contrast, leiomyomas are the most common mesenchymal neoplasm of the esophagus (75% of cases) and occur in a younger population (median age, 35 years) compared with GISTs (for whom the median patient age is 63 years) (6).

To our knowledge, there have been no reports of the radiologic appearance of esophageal GISTs. The one esophageal GIST in our series had radiologic features similar to tumors reported as esophageal leiomyosarcomas (38,39).

Esophageal GISTs are reported to range up to 25 cm in size and are most commonly located in the distal third of the esophagus (6). The single esophageal GIST in our series measured 16 cm in size and manifested as a middle mediastinal mass on chest radiographs (Fig 23). Barium studies of the esophagus may show a smooth intramural mass or a large, ulcerative mass that extends into the esophageal lumen. Distal lesions may extend into the proximal stomach. On CT images, these lesions may be homogeneous or heterogeneous in attenuation. They may contain central areas of low attenuation from hemorrhage, necrosis, or cystic degeneration.



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Figure 23a.  Esophageal GIST in a 71-year-old man who presented with upper gastrointestinal tract bleeding. (a) Lateral chest radiograph shows a retrocardiac mass. (b) Barium esophagram shows the distal esophageal mass that distorts and widens the esophageal lumen. (c) Unenhanced CT scan shows a 16-cm soft-tissue attenuation mass involving the esophagus.

 


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Figure 23b.  Esophageal GIST in a 71-year-old man who presented with upper gastrointestinal tract bleeding. (a) Lateral chest radiograph shows a retrocardiac mass. (b) Barium esophagram shows the distal esophageal mass that distorts and widens the esophageal lumen. (c) Unenhanced CT scan shows a 16-cm soft-tissue attenuation mass involving the esophagus.

 


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Figure 23c.  Esophageal GIST in a 71-year-old man who presented with upper gastrointestinal tract bleeding. (a) Lateral chest radiograph shows a retrocardiac mass. (b) Barium esophagram shows the distal esophageal mass that distorts and widens the esophageal lumen. (c) Unenhanced CT scan shows a 16-cm soft-tissue attenuation mass involving the esophagus.

 
The radiologic differential diagnosis for esophageal GISTs is based on size and growth pattern. Small lesions confined to the esophageal wall are more likely to represent true leiomyomas histologically. However, duplication cyst, lipoma, granular cell tumor, and hemangioma should be considered in the differential diagnosis, as they are lesions of submucosal origin. The use of endoluminal ultrasonography with fine-needle aspiration biopsy has been reported as a useful technique to aid in characterization, diagnosis, and management of submucosal lesions of the esophagus (40,41).

GISTs that extend into the esophageal lumen may simulate a polypoid mass so that lesions of mucosal origin may occasionally be considered in the differential diagnosis. Papilloma, adenoma, inflammatory polyp, fibrovascular polyp, and carcinoma manifest as intraluminal polypoid masses. Large, highly aggressive GISTs that extend into the mediastinum have radiologic appearances similar to those of advanced stage carcinoma, malignant melanoma, lymphoma, and leiomyosarcoma.

Mesentery and Omentum
Primary GISTs may occur in any of the mesenteric or omental structures within the peritoneum. We had a single case in our series that originated in the small bowel mesentery. In the series reported by Miettinen et al (4), the median size of primary omental and mesenteric GISTs was 16.5 cm. The presence of hemorrhage, necrosis, and cystic change in these tumors results in the appearance of a complex mass on cross-sectional images. Our case was characterized as a well-circumscribed, multilobulated mass containing areas of low attenuation (Fig 24). The cystic component of the tumor may be the dominant feature. The peripheral solid portions of the tumor enhance during intravenous contrast material administration.



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Figure 24a.  GIST arising from the small bowel mesentery in a 65-year-old man with abdominal pain. (a) Intravenous contrast-enhanced CT scan shows a 15-cm mass in the small bowel mesentery that contains areas of contrast enhancement and low attenuation (white arrows). There are adjacent metastatic lesions in the mesentery (black arrows). (b) CT scan through the upper abdomen shows liver metastases and intraperitoneal spread of tumor (arrow).

 


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Figure 24b.  GIST arising from the small bowel mesentery in a 65-year-old man with abdominal pain. (a) Intravenous contrast-enhanced CT scan shows a 15-cm mass in the small bowel mesentery that contains areas of contrast enhancement and low attenuation (white arrows). There are adjacent metastatic lesions in the mesentery (black arrows). (b) CT scan through the upper abdomen shows liver metastases and intraperitoneal spread of tumor (arrow).

 
The imaging appearance of mesenteric and omental GISTs is indistinguishable from those of other sarcomas that may arise in these locations, such as leiomyosarcoma, malignant fibrous histiocytoma, fibrosarcoma, and liposarcoma. Mesenteric fibromatosis (desmoid tumor) and inflammatory pseudotumor are typically well-circumscribed mesenteric masses that are homogeneous in attenuation on CT images and should be considered in the differential diagnosis when there is no evidence of hemorrhage, necrosis, or cystic change within the mass on CT or MR images.

GISTs from the gastrointestinal tract may metastasize to the omentum and mesentery; however, they typically result in multiple masses throughout the peritoneal cavity. In these instances, the differential diagnosis includes peritoneal carcinomatosis, lymphomatosis, and the benign condition leiomyomatosis peritonealis disseminata; the latter typically manifests as innumerable small nodules measuring only a few millimeters each (42).


    Prognosis and Therapy
 Top
 Abstract
 LEARNING OBJECTIVES FOR TEST...
 Introduction
 Clinical Features
 Pathologic Features
 Radiologic Features
 Prognosis and Therapy
 Conclusions
 References
 
Tumor size and mitotic rate (mitoses per consecutive 50 high-power fields) are the most common criteria used by pathologists to assess benign versus malignant biologic behavior. Anatomic site alone has been demonstrated to be an independent prognostic factor (43), and the significance of size is also site dependent (28).

A small number of GISTs recur or metastasize despite a histologically benign appearance (ie, small size and absence of mitoses or low mitotic rate). Therefore, some authors support stratifying GISTs into very low-, low-, intermediate-, and high-risk categories rather than classifying them as benign or malignant (23,27). Currently, it is recommended that all patients with GISTs be carefully monitored for an indefinite period because patients with recurrence have a poor prognosis (27).

The conventional therapy for patients with GISTs has been surgical resection of the primary disease. Malignant GISTs typically recur and metastasize to the liver and peritoneal surface. Lymph node metastasis is very uncommon. Conventional systemic and intraperitoneal chemotherapeutic agents, arterial chemoembolization, surgery, and irradiation have been ineffective in treating patients with metastatic and recurrent disease.

In 2001, Hirota et al (44) described a small series of patients with GISTs containing activating mutations in exon 11 of the KIT gene. Such mutations cause increased enzymatic activity of the KIT tyrosine kinase. Tuveson et al (45) showed that inhibition of the mutant KIT by STI-571 leads to growth arrest and death of the tumor cells. KIT mutation is not present in all GISTs; the reported frequency of the mutation ranges from 52% to 85% (46,47). Current studies show that 59%–69% of patients respond to therapy with STI-571, 19%–26% stabilize, and in 11%–13% disease progresses (48,49). The exact role of STI-571 has not yet been established; it may prove useful in neoadjuvant and adjuvant treatment or in combination with conventional therapy (49).


    Conclusions
 Top
 Abstract
 LEARNING OBJECTIVES FOR TEST...
 Introduction
 Clinical Features
 Pathologic Features
 Radiologic Features
 Prognosis and Therapy
 Conclusions
 References
 
GISTs most commonly involve the muscularis propria of the stomach or intestinal wall and extend to involve extramural, mural, and intraluminal surfaces of the stomach and small intestine. The extramural component of GISTs may be extensive such that the bulk of the tumor is outside the organ of origin. GISTs occurring in the gastrointestinal tract and mesentery characteristically have hemorrhage, necrosis, or cyst formation that appears as focal areas of low attenuation on CT images. Although the radiologic features of GISTs are often distinct from those of epithelial tumors, criteria to separate GISTs radiologically from other nonepithelial tumors have not yet been fully developed.


    Footnotes
 
See also the drawing by Cooper (p 456) in this issue .

Abbreviations: GIST = gastrointestinal stromal tumor, H-E = hematoxylin-eosin

The opinions and assertions contained herein are the private views of the authors and are not to be construed as official nor as reflecting the view of the Departments of the Army or Defense.


    References
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 Abstract
 LEARNING OBJECTIVES FOR TEST...
 Introduction
 Clinical Features
 Pathologic Features
 Radiologic Features
 Prognosis and Therapy
 Conclusions
 References
 

  1. Mazur MT, Clark HB. Gastric stromal tumors: reappraisal of histogenesis. Am J Surg Pathol 1983; 7:507-519.[Medline]
  2. Miettinen M, Virolainen M, Maarit Sarlomo R. Gastrointestinal stromal tumors: value of CD34 antigen in their identification and separation from true leiomyomas and schwannomas. Am J Surg Pathol 1995; 19:207-216.[Medline]
  3. Sarlomo-Rikala M, Kovatich AJ, Barusevicius A, Miettinen M. CD117: a sensitive marker for gastrointestinal stromal tumors that is more specific than CD34. Mod Pathol 1998; 11:728-734.[Medline]
  4. Miettinen M, Monihan JM, Sarlomo-Rikala M, et al. Gastrointestinal stromal tumors/smooth muscle tumors (GISTs) primary in the omentum and mesentery: clinicopathologic and immunohistochemical study of 26 cases. Am J Surg Pathol 1999; 23:1109-1118.[CrossRef][Medline]
  5. Kindblom LG, Remotti HE, Aldenborg F, Meis-Kindblom JM. Gastrointestinal pacemaker cell tumor (GIPACT): gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cells of Cajal. Am J Pathol 1998; 152:1259-1269.[Abstract]
  6. Miettinen M, Sarlomo-Rikala M, Sobin LH, Lasota J. Esophageal stromal tumors: a clinicopathologic, immunohistochemical, and molecular genetic study of 17 cases and comparison with esophageal leiomyomas and leiomyosarcomas. Am J Surg Pathol 2000; 24:211-222.[CrossRef][Medline]
  7. Miettinen M, Sobin LH. Gastrointestinal stromal tumors in the appendix: a clinicopathologic and immunohistochemical study of four cases. Am J Surg Pathol 2001; 25:1433-1437.[CrossRef][Medline]
  8. Miettinen M, Lasota J. Gastrointestinal stromal tumors: definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch 2001; 438:1-12.[CrossRef][Medline]
  9. Miettinen M, Sarlomo-Rikala M, Sobin LH, Lasota J. Gastrointestinal stromal tumors and leiomyosarcomas in the colon: a clinicopathologic, immunohistochemical, and molecular genetic study of 44 cases. Am J Surg Pathol 2000; 24:1339-1352.[Medline]
  10. Licht JD, Weissmann LB, Antman K. Gastrointestinal sarcomas. Semin Oncol 1988; 15:181-188.[Medline]
  11. Miettinen M, Sarlomo-Rikala M, Lasota J. Gastrointestinal stromal tumors: recent advances in understanding of their biology. Hum Pathol 1999; 30:1213-1220.[CrossRef][Medline]
  12. Ueyama T, Guo KJ, Hashimoto H, Daimaru Y, Enjoji M. A clinicopathologic and immunohistochemical study of gastrointestinal stromal tumors. Cancer 1992; 69:947-955.[CrossRef][Medline]
  13. DeMatteo RP, Lewis JJ, Leung D, Mudan SS, Woodruff JM, Brennan MF. Two hundred gastrointestinal stromal tumors: recurrence patterns and prognostic factors for survival. Ann Surg 2000; 231:51-58.[CrossRef][Medline]
  14. Crosby JA, Catton CN, Davis A, et al. Malignant gastrointestinal stromal tumors of the small intestine: a review of 50 cases from a prospective database. Ann Surg Oncol 2001; 8:50-59.[Abstract/Free Full Text]
  15. Fuller CE, Williams GT. Gastrointestinal manifestations of type 1 neurofibromatosis (von Recklinghausen’s disease). Histopathology 1991; 19:1-11.[Medline]
  16. Boldorini R, Tosoni A, Leutner M, et al. Multiple small intestinal stromal tumours in a patient with previously unrecognised neurofibromatosis type 1: immunohistochemical and ultrastructural evaluation. Pathology 2001; 33:390-395.[Medline]
  17. Carney JA. The triad of gastric epithelioid leiomyosarcoma, pulmonary chondroma, and functioning extra-adrenal paraganglioma: a five-year review. Medicine (Baltimore) 1983; 62:159-169.[Medline]
  18. Heinrich MC, Rubin BP, Longley BJ, Fletcher JA. Biology and genetic aspects of gastrointestinal stromal tumors: KIT activation and cytogenetic alterations. Hum Pathol 2002; 33:484-495.[CrossRef][Medline]
  19. Suster S. Gastrointestinal stromal tumors. Semin Diagn Pathol 1996; 13:297-313.[Medline]
  20. Miettinen M, Sarlomo-Rikala M, Sobin LH. Mesenchymal tumors of muscularis mucosae of colon and rectum are benign leiomyomas that should be separated from gastrointestinal stromal tumors: a clinicopathologic and immunohistochemical study of eighty-eight cases. Mod Pathol 2001; 14:950-956.[CrossRef][Medline]
  21. Min KW. Small intestinal stromal tumors with skeinoid fibers. Clinicopathological, immunohistochemical, and ultrastructural investigations. Am J Surg Pathol 1992; 16:145-155.
  22. Miettinen M, Furlong M, Sarlomo-Rikala M, Burke A, Sobin LH, Lasota J. Gastrointestinal stromal tumors, intramural leiomyomas, and leiomyosarcomas in the rectum and anus: a clinicopathologic, immunohistochemical, and molecular genetic study of 144 cases. Am J Surg Pathol 2001; 25:1121-1133.[CrossRef][Medline]
  23. Franquemont DW. Differentiation and risk assessment of gastrointestinal stromal tumors. Am J Clin Pathol 1995; 103:41-47.[Medline]
  24. Tsuura Y, Hiraki H, Watanabe K, et al. Preferential localization of c-KIT product in tissue mast cells, basal cells of skin, epithelial cells of breast, small cell lung carcinoma and seminoma/dysgerminoma in humans: immunohistochemical study on formalin-fixed, paraffin-embedded tissues. Virchows Arch 1994; 424:135-141.[Medline]
  25. Miettinen M, Lindenmayer AE, Chaubal A. Endothelial cell markers CD31, CD34, and BNH9 antibody to H- and Y-antigens: evaluation of their specificity and sensitivity in the diagnosis of vascular tumors and comparison with von Willebrand factor. Mod Pathol 1994; 7:82-90.[Medline]
  26. Traweek ST, Kandalaft PL, Mehta P, Battifora H. The human hematopoietic progenitor cell antigen (CD34) in vascular neoplasia. Am J Clin Pathol 1991; 96:25-31.[Medline]
  27. Fletcher CD, Berman JJ, Corless C, et al. Diagnosis of gastrointestinal stromal tumors: a consensus approach. Hum Pathol 2002; 33:459-465.[CrossRef][Medline]
  28. Miettinen M, El-Rifai W, Sobin LH, Lasota J. Evaluation of malignancy and prognosis of gastrointestinal stromal tumors: a review. Hum Pathol 2002; 33:478-483.[CrossRef][Medline]
  29. Nauert TC, Zornoza J, Ordonez N. Gastric leiomyosarcoma. AJR Am J Roentgenol 1982; 139:291-297.[Abstract/Free Full Text]
  30. Sharp RM, Ansel HJ, Keel SB. Best cases from the AFIP: gastrointestinal stromal tumor. RadioGraphics 2001; 21:1557-1560.[Free Full Text]
  31. Scatarige JC, Fishman EK, Jones B, Cameron JL, Sanders RC, Siegelman SS. Gastric leiomyosarcoma: CT observations. J Comput Assist Tomogr 1985; 9:320-327.[Medline]
  32. Hasegawa S, Semelka RC, Noone TC, et al. Gastric stromal sarcomas: correlation of MR imaging and histopathologic findings in nine patients. Radiology 1998; 208:591-595.[Abstract/Free Full Text]
  33. Buckley JA, Fishman EK. CT evaluation of small bowel neoplasms: spectrum of disease. RadioGraphics 1998; 18:379-392.[Abstract]
  34. Shojaku H, Futatsuya R, Seto H, Tajika S, Matsunou H. Malignant gastrointestinal stromal tumor of the small intestine: radiologic-pathologic correlation. Radiat Med 1997; 15:189-192.[Medline]
  35. Hama Y, Okizuka H, Odajima K, Hayakawa M, Kusano S. Gastrointestinal stromal tumor of the rectum. Eur Radiol 2001; 11:216-219.[CrossRef][Medline]
  36. van den Berg JC, van Heesewijk JP, van Es HW. Malignant stromal tumour of the rectum: findings at endorectal ultrasound and MRI. Br J Radiol 2000; 73:1010-1012.[Abstract]
  37. Lee SH, Ha HK, Byun JY, et al. Radiological features of leiomyomatous tumors of the colon and rectum. J Comput Assist Tomogr 2000; 24:407-412.[CrossRef][Medline]
  38. Levine MS, Buck JL, Pantongrag-Brown L, Buetow PC, Hallman JR, Sobin LH. Leiomyosarcoma of the esophagus: radiographic findings in 10 patients. AJR Am J Roentgenol 1996; 167:27-32.[Abstract/Free Full Text]
  39. Visioli A, Daniel FJ. Leiomyosarcoma of the oesophagus: a case report and literature review of leiomyosarcoma. Australas Radiol 1997; 41:160-165.[Medline]
  40. Shen EF, Arnott ID, Plevris J, Penman ID. Endoscopic ultrasonography in the diagnosis and management of suspected upper gastrointestinal submucosal tumours. Br J Surg 2002; 89:231-235.[Medline]
  41. Gu M, Ghafari S, Nguyen PT, Lin F. Cytologic diagnosis of gastrointestinal stromal tumors of the stomach by endoscopic ultrasound-guided fine-needle aspiration biopsy: cytomorphologic and immunohistochemical study of 12 cases. Diagn Cytopathol 2001; 25:343-350.[CrossRef][Medline]
  42. Minassian SS, Frangipane W, Polin JI, Ellis M. Leiomyomatosis peritonealis disseminata: a case report and literature review. J Reprod Med 1986; 31:997-1000.[Medline]
  43. Emory TS, Sobin LH, Lukes L, Lee DH, O’Leary TJ. Prognosis of gastrointestinal smooth-muscle (stromal) tumors: dependence on anatomic site. Am J Surg Pathol 1999; 23:82-87.[CrossRef][Medline]
  44. Hirota S, Nishida T, Isozaki K, et al. Gain-of-function mutation at the extracellular domain of KIT in gastrointestinal stromal tumours. J Pathol 2001; 193:505-510.[CrossRef][Medline]
  45. Tuveson DA, Willis NA, Jacks T, et al. STI571 inactivation of the gastrointestinal stromal tumor c-KIT oncoprotein: biological and clinical implications. Oncogene 2001; 20:5054-5058.[CrossRef][Medline]
  46. Lasota J, Wozniak A, Sarlomo-Rikala M, et al. Mutations in exons 9 and 13 of KIT gene are rare events in gastrointestinal stromal tumors: a study of 200 cases. Am J Pathol 2000; 157:1091-1095.[Abstract/Free Full Text]
  47. Lux ML, Rubin BP, Biase TL, et al. KIT extracellular and kinase domain mutations in gastrointestinal stromal tumors. Am J Pathol 2000; 156:791-795.[Abstract/Free Full Text]
  48. van Oosterom AT, Judson I, Verweij J, et al. Safety and efficacy of imatinib (STI571) in metastatic gastrointestinal stromal tumours: a phase I study. Lancet 2001; 358:1421-1423.[CrossRef][Medline]
  49. Dematteo RP, Heinrich MC, El-Rifai WM, Demetri G. Clinical management of gastrointestinal stromal tumors: before and after STI-571. Hum Pathol 2002; 33:466-477.[CrossRef][Medline]

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