eMedicine Specialties > Oncology > Carcinomas of the Gastrointestinal Tract

Gastric Gastrointestinal Stromal Tumors: Differential Diagnoses & Workup

Author: Michael A Choti, MD, MBA, Jacob C Handelsman Professor of Surgery, Professor of Oncology and Engineering, Johns Hopkins University School of Medicine
Coauthor(s): Matthew Hueman, MD, Fellow in Surgical Oncology, The Johns Hopkins Hospital; Instructor, Department of Surgery, The Johns Hopkins School of Medicine
Contributor Information and Disclosures

Updated: Jun 4, 2009

Differential Diagnoses

Gastric Cancer
Gastrointestinal Stromal Tumors

Other Problems to Be Considered

Gastric schwannoma
True smooth muscle tumor of the stomach (leiomyoma)
Gastric sarcoma
Gastric adenocarcinoma

The differential diagnosis for gastric stromal tumors includes benign lesions such as true leiomyoma, schwannoma, lipoma, ectopic pancreas, and sarcomas.13

Other possible lesions include the much more common gastric adenocarcinoma and other rare submucosal malignant tumors such as lymphoma and carcinoid.

Not infrequently, patients with GISTs of the stomach present with a large mass in the epigastrium or left upper quadrant. In such cases, the differential diagnosis may include masses originating from other organs such as the liver, spleen, pancreas, left adrenal gland, or retroperitoneum.

Workup

Laboratory Studies

Laboratory studies are not diagnostic, and no identifiable tumor markers exist.

Imaging Studies

  • Computed tomography scanning of the abdomen: Abdominal CT scanning with intravenous and oral contrast material is a necessary step in the evaluation of these patients. The gastric mass can be detected originating from the gastric wall (see Image 2), but, at times, the organ site of origin is not clear on CT scan. CT scanning can also be used to evaluate tumor invasion to adjacent structures and the presence of intra-abdominal metastasis. The identification of distant disease is important as many as half of patients who initially present with a GIST have distant metastases (two-thirds of whom have hepatic involvement).

    CT scan of the abdomen with oral contrast in a 60...

    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).

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    CT scan of the abdomen with oral contrast in a 60...

    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).

  • Endoscopic ultrasonography: Endoscopic ultrasonography (EUS) can be a valuable tool in the diagnosis and preoperative assessment of gastric GISTs when the diagnosis or location is in doubt but is not generally required for preoperative workup.14,15
    • If the location of the lesion is in doubt, the EUS can help plan the operative approach (eg, demonstrating that a proximal gastric lesion on CT scan is far enough away from the GE junction to allow local wedge resection as opposed to total gastrectomy). EUS can also demonstrate the submucosal location of the tumor and can define its size, borders, and echoic pattern.
    • In general, ultrasonic features of a mass suspicious for malignancy are large tumors, tumors with irregular extraluminal borders, and the presence of cystic spaces and echogenic foci.
    • All gastric GISTs should be considered to have malignant potential. Although, generally, gastric GISTs less than 2 cm tend to behave as "benign" masses, there have been rare reports of distant spread. EUS is generally the preferred modality to facilitate biopsy of the lesion in cases in which biopsy aids in the workup and management of the patient (see Preoperative biopsy).

Procedures

  • Upper endoscopy: Upper endoscopy is often the first examination performed in the evaluation of patients with upper gastrointestinal symptoms, but endoscopy is not generally required in the workup of patients with lesions on CT suspicious for a gastric GIST. Gastroscopy may demonstrate a firm, smooth, yellowish submucosal mass, which can be ulcerated (see Image 1). These tumors can be missed endoscopically because of their frequent submucosal and extraluminal growth. If the diagnosis is suspected prior to endoscopy, an endoscopic ultrasound can be performed to further characterize and help confirm the lesion's growth from the stomach (when the organ site of the tumor is not clearly evident on CT scan), even if not visible endoscopically.

    Shown here is a gastric gastrointestinal stromal ...

    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.

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    Shown here is a gastric gastrointestinal stromal ...

    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.

  • Preoperative biopsy: While the diagnosis can often be made using ultrasonographic-guided biopsy, the use of biopsy is controversial in an otherwise primary, resectable lesion suspicious for GIST.
    • Generally, unless a concern for an alternative diagnosis or use of neoadjuvant therapy is being entertained, the use of biopsy is not recommended in this setting (www.nccn.org).16 The biopsy of a GIST, which tends to be soft and fragile, may cause intratumoral hemorrhage or even rupture and may increase the risk for tumor dissemination. Generally, irrespective of the biopsy results, surgical resection is required for treatment and for definitive diagnosis. 
    • Reasons to perform a biopsy: A biopsy is important and required in the setting of suspected metastatic disease or when neoadjuvant treatment of borderline resectable GIST is being entertained.
      • The initial treatment of metastatic GIST should generally be tyrosine-kinase inhibitor therapy with imatinib. Patients with GISTs that appear to involve critical structures or are in challenging locations (eg, duodenal requiring Whipple) may benefit from neoadjuvant therapy. Prior to initiation of imatinib for either metastatic disease or in the neoadjuvant setting, a pretreatment biopsy is generally required to confirm the diagnosis prior to initiation of such treatment. 
      • Biopsy may also be important when the diagnosis of GIST in is question, such as when the submucosal nature of this tumor is in doubt or when tumor characteristics as demonstrated by upper endoscopy and endoscopic ultrasonography are not typical. In specific patients, such as high-risk operative patients with small benign-appearing lesions and minimal or no symptoms, tissue diagnosis may help in further decision-making.
    • The 2 ways to obtain a preoperative histologic diagnosis are as follows:
      • Endoscopic biopsy: Preoperative endoscopic biopsy may be taken with or without EUS guidance. When taken without the help of EUS, endoscopic biopsy is not accurate and leads to a correct diagnosis in less than 50% of patients. Biopsies may miss the tumor and show only mucosal tissue. In addition, samples from the tumor itself are often too small to establish malignant nature. EUS-guided biopsy is more accurate. This technique can achieve a correct histologic diagnosis in more than 80% of cases and should be performed whenever preoperative histology seems necessary.
      • Percutaneous biopsy: Tumor biopsy can be obtained percutaneously under CT scanning or ultrasonographic guidance.17 Consider this procedure in selected patients when endoscopic biopsy is impossible to perform or the results are negative.

Histologic Findings

Cellular morphology as visualized by light microscopy can be variable. Most often, the tumors are highly cellular and composed of spindle-shaped cells that resemble smooth-muscle tissue (see Image 3). However, this histologic appearance is not uniform. A similar tumor with a predominant epithelioid component was historically diagnosed as leiomyoblastoma (see Image 4). This variant is occasionally associated with a well-defined condition called Carney syndrome.

Photomicrograph of gastrointestinal stromal tumor...

Photomicrograph of gastrointestinal stromal tumor (GIST) stained with hematoxylin and eosin (H&E) and magnified 40X. Note the solid sheet of spindle cells.

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Photomicrograph of gastrointestinal stromal tumor...

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 strom...

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.

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Photomicrograph of gastric gastrointestinal strom...

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.


Important histologic factors to consider in evaluating these tumors are mitotic index, cellularity, necrosis, nuclear atypia and nuclear-cytoplasmic ratio, cell shape, amount of stroma, and vascularity.

Investigations of GISTs by immunohistochemistry and electron microscopy (ultrastructural parameters) reveal phenotype variability that includes myoid, neural, and indeterminate characteristics.18 Study of GISTs by immunohistochemistry methods reveals expression of CD117 and other various antigens, such as nestin (90-100% positivity), CD34 (70% positivity), CD44,19 vimentin, desmin, muscle-specific actin, smooth-muscle actin, S-100 protein, neurofilament, neuron-specific enolase, and PGP9.5. CD117 plays an important role in the latest specific diagnostic criteria for GISTs. CD117 (c-kit protein) is a growth factor receptor with tyrosine-kinase activity and is a product of the proto-oncogene c-kit. CD117, although not tumor-specific, is expressed in all GISTs but not in true smooth muscle tumors and neural tumors.

CD117 has become a very important tool in the differentiation of GIST from other GI mesenchymal tumors.20,21 Positive CD117 staining in a spindle-shaped cell GI tumor is diagnostic for GIST (see Image 5). CD34 is another important diagnostic marker. It is detected in approximately 70% of GISTs, and its presence may indicate a higher probability for a malignant phenotype. CD44 is variably expressed by GISTs, but its expression has been demonstrated to correlate with a better prognosis.

Photomicrograph of gastrointestinal stromal tumor...

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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Photomicrograph of gastrointestinal stromal tumor...

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.


Recent studies suggest that GISTs may originate from the interstitial cells of Cajal. These cells are distributed along the GI tract and play a role in the control of gut motility. The interstitial cells of Cajal exhibit both myeloid and neural features and express the c-kit proto-oncogene receptor. However, the fact that GISTs are detected (although very rarely) outside of the GI tract (ie, omentum, mesentry, retroperitoneum) argues against this hypothesis.

Staging

No consensus has been reached regarding a uniform staging system, and none of the currently used classifications is fully satisfactory. Most staging systems employ the 3 most important survival predictors—tumor size, histologic grade, and presence or absence of distant metastatic disease.

The NCCN criteria for risk stratification of primary GIST have not been incorporated into the AJCC staging seen below but may be more helpful in determining individual risk for progressive disease.16 The stratification is by mitotic index (5 or less or more than 5 per 50 HPF) and then further divided by tumor size (2 cm or less or more than 2 cm; 5 cm or less or more than 5; 10 cm or less or more than 10 cm) and tumor location (gastric, duodenum, jejunum-ileum, and rectum). Gastric GISTs larger than 10 cm but 5 or less per 50 HPF mitotic index have only a 10% risk of progressive disease despite 34-57% risk of progressive disease in the other tumor locations. Gastric GISTs greater than 10 cm and a high mitotic index (>5 per 50 HPF), however, have an equally high risk of progressive disease (86%) as the other tumor locations.

Many studies have shown that tumor diameter greater than 5 cm is associated with increased risk for malignancy. However, relation of size to malignant potential may be gradual, with no clear cut-off point.

The number of mitotic figures is the most accepted index for grade classification, although other histologic parameters, such as cellularity, atypia, and necrosis, are also taken into consideration. A high mitotic index of more than 5 mitoses per 10 HPF usually signifies highly malignant disease. However, a low mitotic index is not always associated with benign course. As many as 25% of tumors with mitotic index of less than 5 mitoses per 10 HPF may manifest an aggressive biological behavior. Some authors have defined an intermediate-risk category applied for tumors with a mitotic index of 2-4 mitoses per 10 HPF.

  • Tumor size
    • T1- Tumor smaller than 5 cm, localized
    • T2 - Tumor 5 cm or larger, localized
    • T3 - Contiguous organ invasion or peritoneal implants
    • T4 - Tumor rupture
  • Tumor grade
    • G1- Low grade
    • G2 - High grade
  • Metastasis
  • M0 - No metastasis
  • M1 - Distant metastases Table 1. Proposed Staging System for Malignant Gastrointestinal Stromal Tumors

    Open table in new window

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    Table
    StageTumor SizeTumor GradeMetastasis
    Stage IT1G1M0
    Stage IIT2G1M0
    Stage IIIT1-2
    T3    		G2
    Any G    		M0
    M0
    Stage IVa……M1 or residual disease after surgery
    Stage IVbT4……
    StageTumor SizeTumor GradeMetastasis
    Stage IT1G1M0
    Stage IIT2G1M0
    Stage IIIT1-2
    T3    		G2
    Any G    		M0
    M0
    Stage IVa……M1 or residual disease after surgery
    Stage IVbT4……

More on Gastric Gastrointestinal Stromal Tumors

Overview: Gastric Gastrointestinal Stromal Tumors
Differential Diagnoses & Workup: Gastric Gastrointestinal Stromal Tumors
Treatment & Medication: Gastric Gastrointestinal Stromal Tumors
Follow-up: Gastric Gastrointestinal Stromal Tumors
Multimedia: Gastric Gastrointestinal Stromal Tumors
References
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Further Reading

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Keywords

gastric gastrointestinal stromal tumors, gastric GISTs, malignant gastric stromal tumors, gastric leiomyosarcomas, gastrointestinal stromal tumors, GISTs, gastric GIST, fibrosarcoma, angiosarcoma, hemangiopericytoma, gastric smooth muscle tumors, intestinal smooth muscle tumors

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Contributor Information and Disclosures

Author

Michael A Choti, MD, MBA, Jacob C Handelsman Professor of Surgery, Professor of Oncology and Engineering, Johns Hopkins University School of Medicine
Michael A Choti, MD, MBA is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Surgeons, American Hepato-Pancreato-Biliary Association, American Society of Clinical Oncology, American Surgical Association, Association for Academic Surgery, International Hepato-Pancreato-Biliary Association, Society for Surgery of the Alimentary Tract, Society of Surgical Oncology, and Society of University Surgeons
Disclosure: Nothing to disclose.

Coauthor(s)

Matthew Hueman, MD, Fellow in Surgical Oncology, The Johns Hopkins Hospital; Instructor, Department of Surgery, The Johns Hopkins School of Medicine
Matthew Hueman, MD is a member of the following medical societies: American Association for Cancer Research, American College of Surgeons, American Hepato-Pancreato-Biliary Association, American Society of Clinical Oncology, Association for Academic Surgery, and Society of Surgical Oncology
Disclosure: Nothing to disclose.

Medical Editor

Robert C Shepard, MD, FACP, Associate Professor of Medicine in Hematology and Oncology at University of North Carolina at Chapel Hill; Vice President of Scientific Affairs, Therapeutic Expertise, Oncology, at PRA International
Robert C Shepard, MD, FACP is a member of the following medical societies: American Association for Cancer Research, American College of Physician Executives, American College of Physicians, American Federation for Clinical Research, American Federation for Medical Research, American Medical Association, American Medical Informatics Association, American Society of Hematology, Association of Clinical Research Professionals, Eastern Cooperative Oncology Group, European Society for Medical Oncology, Massachusetts Medical Society, and Society for Biological Therapy
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

CME Editor

Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Jules E Harris, MD, Clinical Professor of Medicine, Division of Hematology/Medical Oncology, Department of Internal Medicine, University of Arizona College of Medicine at Tucson; Consulting Staff, Arizona Cancer Center
Jules E Harris, MD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Association of Immunologists, American Society of Hematology, and Central Society for Clinical Research
Disclosure: GlobeImmune Salary Consulting; Amplimed Consulting fee Consulting; FibroGen Consulting fee Consulting

 
 
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