MALIGNANT GASTROINTESTINAL STROMAL
TUMOR
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ALTERNATE NAMES
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Gastrointestinal Stromal Neoplasm; Gastrointestinal Stromal Sarcoma
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DESCRIPTION
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Malignant Gastrointestinal Stromal Tumor (GIST) is a rare type of soft tissue tumor that usually begins in cells in the wall of the
stomach, intestines, or rectum. GIST tumors are caused by mutations in the PDGFRA
gene. Clinical features of the GIST depend on the size and site of the tumor and may
include acute or chronic bleeding, intestinal obstruction, perforation, alteration
of bowel habits, difficult to distinguish abdominal discomfort, dysphagia (difficulty
swallowing) and externally palpable abdominal mass. The most common symptoms associated
with GISTs are vague, nonspecific abdominal pain or discomfort. GISTs may also produce
symptoms secondary to obstruction or hemorrhage (GI bleeding, malaise, fatigue, and
dyspnea). The obstructive symptoms can be site-specific (e.g., dysphagia with an esophageal
GIST, constipation with a colorectal GIST, obstructive jaundice with a duodenal tumor,
etc.)
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing:
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Imaging tests such as X-ray, MRI, CT or PET scans;
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Barium swallow tests (upper GI series);
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Barium enema (lower GI series);
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Double Balloon Enteroscopy;
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Physical findings:
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GI obstruction, appendicitis-like pain;
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Anemia due to GI bleed; and
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Feeling of stomach fullness (satiety).
ICD–9:
238.1
ICD-10: C49.A0, C49.A1, C49.A2, C49.A3, C49.A4, C49.A5, C49.A9
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PROGRESSION
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GIST tumors range from slow growing indolent tumors to aggressive malignant cancers
with the propensity to invade adjacent organs, metastasize to the liver, and recur
locally within the abdomen. Most GIST tumors are diagnosed at an advanced stage and
have a poor prognosis. These tumors are most commonly diagnosed between ages 55 –
65 years of age, and rarely are discovered in younger adults.
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TREATMENT
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Surgery with complete resection of GIST is the preferred method of treatment for localized
tumors. However, surgery has limited efficacy in the treatment of recurrent and metastatic
gastrointestinal stromal tumors, as compared with sarcomas in the extremities. The
development of drug therapy (i.e. imatinib) targeted at specific characteristics of
cancer cells in GIST has improved the treatment of this tumor compared with surgical
removal alone.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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Clinical history and examination that describes the diagnostic features of the impairment;
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Pathology report documenting type and stage of tumor;
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Abdominal endoscopy, enteroscopy, or ultrasound.
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Suggested Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets
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13.16
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When occurring in the stomach, which is the organ where most GISTs originate, any
metastases to or beyond the regional lymph nodes will meet the criteria in listing
13.16 B 2.
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13.17
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Since many of these tumors are benign, listing level cancerous tumors must be documented as inoperable, unresectable, extending to surrounding structures
(for example, the omentum), recurrent, or with metastases.
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13.18
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Equals
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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