TY - JOUR
T1 - Gastrointestinal stromal tumors (GISTs) arising in uncommon locations
T2 - clinicopathologic features and risk assessment of esophageal, colonic, and appendiceal GISTs
AU - Rare GIST Risk Stratification Group
AU - Hu, Shaomin
AU - Alpert, Lindsay
AU - Cates, Justin M.M.
AU - Gonzalez, Raul S.
AU - Graham, Rondell
AU - Goldblum, John R.
AU - Bakhshwin, Ahmed
AU - Shetty, Sindhu
AU - Wang, Hanlin L.
AU - Lollie, Trang
AU - Ma, Changqing
AU - Siddique, Ayesha
AU - Karamchandani, Dipti M.
AU - Chen, Fengming
AU - Yantiss, Rhonda K.
AU - Hissong, Erika
AU - Chatterjee, Deyali
AU - Chopra, Shefali
AU - Chen, Wei
AU - Vazzano, Jennifer
AU - Wang, Wei Lien
AU - Ai, Di
AU - Lin, Jingmei
AU - Zheng, Lan
AU - Davis, Jessica L.
AU - Brinkerhoff, Brian
AU - Breitbarth, Amanda
AU - Yang, Michelle
AU - Madahian, Sepideh
AU - Panarelli, Nicole
AU - Kuan, Kevin
AU - Pomper, Jonathan
AU - Longacre, Teri
AU - Raghavan, Shyam
AU - Misdraji, Joseph
AU - Cui, Min
AU - Yang, Zhaohai
AU - Savant, Deepika
AU - Harpaz, Noam
AU - Chen, Xiuxu
AU - Resnick, Murray
AU - Wu, Elizabeth Yiru
AU - Klimstra, David
AU - Shia, Jinru
AU - Vyas, Monika
AU - Kakar, Sanjay
AU - Choi, Won Tak
AU - Robert, Marie E.
AU - Li, Hongjie
AU - Lee, Michael
AU - Clark, Ian
AU - Li, Yongchao
AU - Cao, Wenqing
AU - Chang, Qing
AU - Bronner, Mary P.
AU - Dong, Zachary
AU - Zhang, Wei
AU - Buehler, Darya
AU - Swanson, Paul E.
AU - Mantilla, Jose G.
AU - Bellizzi, Andrew M.
AU - Feely, Michael
AU - Cooper, Harry S.
AU - Nagarathinam, Rajeswari
AU - Pai, Rish
AU - Hammer, Suntrea
AU - Hosseini, Mojgan
AU - Hu, Jing Jing
AU - Westerhoff, Maria
AU - Cheng, Jerome
AU - Agostini-Vulaj, Diana
AU - Lauwers, Gregory
AU - Ghayouri, Masoumeh
AU - Pezhouh, Maryam K.
AU - Zeng, Jianying
AU - Xia, Rong
AU - Yin, Feng
AU - Zhang, Tao
AU - Gao, Zu hua
AU - Demko, Nadine
AU - Chen, Hannah H.
AU - Yu, Sanhong
AU - Hart, John
N1 - Publisher Copyright:
© 2021, The Author(s), under exclusive licence to United States & Canadian Academy of Pathology.
PY - 2022/4
Y1 - 2022/4
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85118236112&partnerID=8YFLogxK
U2 - 10.1038/s41379-021-00949-w
DO - 10.1038/s41379-021-00949-w
M3 - Article
C2 - 34702994
AN - SCOPUS:85118236112
SN - 0893-3952
VL - 35
SP - 554
EP - 563
JO - Modern Pathology
JF - Modern Pathology
IS - 4
ER -