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Pathology - Gastrointestinal stromal tumors
Definition: Mesenchymal tumors with varying degrees of malignancy that originate in the gastrointestinal tract wall and mimic the phenotype of interstitial cells of Cajal, the pacemaker cells of the Auerbach plexus.
Epidemiology • Incidence is approximately 15 cases per million people annually. • Predominantly occurs in adults with a median age of 50 to 60 years
. The aetiology of occasional occurrences remains unidentified. A minor percentage occurs in conjunction with neurofibromatosis type 1, Carney's triad, and families possessing germline KIT mutations.
Genetics • The majority exhibit activating mutations in the oncogene KIT. • The remainder display activating mutations in the related gene PDGFRA.
Presentation • Noticeable upper abdominal mass, discomfort, or hemorrhage. • Malignant neoplasms may manifest with symptoms associated with metastasis. Locations of engagement • Can manifest throughout the gastrointestinal tract, from the esophagus to the rectum. • The majority originate in the stomach (60–70%) or small intestine (20–30%). A little quantity seems to originate predominantly in the omentum.
Macroscopy • Clearly delineated tumor mass located inside the submucosal, muscular, or serosal layer of the bowel. • Size varies from 1 to over 20 cm.
Histopathology • Characterized by spindle cells, frequently exhibiting paranuclear vacuoles. • More robust epithelioid cells may also be observed, and certain tumors may be exclusively epithelioid. Small intestinal tumors may also exhibit so-called 'skeinoid' fibers. Nearly all exhibit the markers CD117 (c-kit) and DOG-1.
Prognosis: All cases should be regarded as potentially malignant. They are categorized into extremely low-risk, low-risk, intermediate-risk, and high-risk classifications for malignancy based on location, size, and mitotic activity.
Definition: Mesenchymal tumors with varying degrees of malignancy that originate in the gastrointestinal tract wall and mimic the phenotype of interstitial cells of Cajal, the pacemaker cells of the Auerbach plexus.
Epidemiology • Incidence is approximately 15 cases per million people annually. • Predominantly occurs in adults with a median age of 50 to 60 years
. The aetiology of occasional occurrences remains unidentified. A minor percentage occurs in conjunction with neurofibromatosis type 1, Carney's triad, and families possessing germline KIT mutations.
Genetics • The majority exhibit activating mutations in the oncogene KIT. • The remainder display activating mutations in the related gene PDGFRA.
Presentation • Noticeable upper abdominal mass, discomfort, or hemorrhage. • Malignant neoplasms may manifest with symptoms associated with metastasis. Locations of engagement • Can manifest throughout the gastrointestinal tract, from the esophagus to the rectum. • The majority originate in the stomach (60–70%) or small intestine (20–30%). A little quantity seems to originate predominantly in the omentum.
Macroscopy • Clearly delineated tumor mass located inside the submucosal, muscular, or serosal layer of the bowel. • Size varies from 1 to over 20 cm.
Histopathology • Characterized by spindle cells, frequently exhibiting paranuclear vacuoles. • More robust epithelioid cells may also be observed, and certain tumors may be exclusively epithelioid. Small intestinal tumors may also exhibit so-called 'skeinoid' fibers. Nearly all exhibit the markers CD117 (c-kit) and DOG-1.
Prognosis: All cases should be regarded as potentially malignant. They are categorized into extremely low-risk, low-risk, intermediate-risk, and high-risk classifications for malignancy based on location, size, and mitotic activity.
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