Gastrointestinal stromal tumors (GISTs) arising in uncommon locations: Clinicopathologic features and risk assessment of esophageal, colonic, and appendiceal GISTs Journal Article

  

Authors:	Hu, S.; Alpert, L.; Cates, J. M. M.; Gonzalez, R. S.; Rare GIST Risk Stratification Group
Contributors:	Klimstra, D.; Shia, J.; Vyas, M.
Article Title:	Gastrointestinal stromal tumors (GISTs) arising in uncommon locations: Clinicopathologic features and risk assessment of esophageal, colonic, and appendiceal GISTs
Abstract:	Risk stratification of gastrointestinal stromal tumors (GISTs) is based on experience with tumors of the stomach, small bowel, and rectum, which are far more common than GISTs of other sites. In this study from 47 institutions, we analyzed GISTs of the esophagus (n = 102), colon (n = 136), and appendix (n = 27) for their size, mitotic rate, morphology, and outcome to determine which criteria predict their behavior. Esophageal GISTs were small (median: 2.5 cm) with spindle cell morphology and a low mitotic rate (mean: 3.6/5 mm2). Twelve (12%) tumors progressed, including 11 with a mitotic rate >5/5 mm2 and one large (6.8 cm) GIST with a mitotic rate of 2/5 mm2. Colonic GISTs were smaller (median: 1.4 cm) and presented with abdominal pain or bleeding in 29% of cases. Most (92%) were composed of spindle cells with a mean mitotic rate of 4.6/5 mm2. Sixteen (12%) tumors progressed: 14 had mitotic rates >5/5 mm2, and two were >5.0 cm with a mitotic rate <5/5 mm2. All but one appendiceal GIST measured <2.0 cm. These tumors were composed of spindle cells with low mitotic rates (<5/5 mm2), and none progressed. Our results suggest that progression risk among esophageal and colonic GISTs is associated with increased mitotic activity (>5/5 mm2) and size >5.0 cm. These findings support the use of size and mitotic rate for prognostication of GISTs in these locations, similar to tumors of the stomach, small bowel, and rectum. © 2021, The Author(s), under exclusive licence to United States & Canadian Academy of Pathology.
Keywords:	adult; controlled study; human tissue; aged; gene mutation; major clinical study; gene deletion; clinical feature; histopathology; gene; gastrointestinal stromal tumor; imatinib; stem cell factor receptor; gastrointestinal stromal tumors; proto-oncogene proteins c-kit; cohort analysis; pathology; risk assessment; carcinogenesis; colon tumor; appendix; mitosis rate; checkpoint kinase 2; appendectomy; hemicolectomy; stomach neoplasms; disease exacerbation; colon; esophagus; esophagus tumor; esophagectomy; stomach tumor; appendix tumor; colectomy; kit gene; pdgfra gene; nf1 gene; humans; human; male; female; article; cecum resection
Journal Title:	Modern Pathology
Volume:	35
Issue:	4
ISSN:	0893-3952
Publisher:	Nature Research
Date Published:	2022-04-01
Start Page:	554
End Page:	563
Language:	English
DOI:	10.1038/s41379-021-00949-w
PUBMED:	34702994
PROVIDER:	scopus
DOI/URL:	https://www.scopus.com/inward/record.uri?eid=2-s2.0-85118236112&doi=10.1038%2fs41379-021-00949-w&partnerID=40&md5=7413ac53dfdc995b900cf1c9dcff7e8e
Notes:	Article -- Export Date: 1 August 2022 -- Source: Scopus

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