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Alpert L , Al-Sabti R , Graham RP , Pai RK , Gonzalez RS , Zhang X , Smith V , Wang HL , Westbrook L , Goldblum JR , Bakhshwin A , Shetty S , Klimstra DS , Shia J , Askan G , Robert ME , Thomas C , Frankel WL , Alsomali M , Hagen C , Mostafa ME , Feely MM , Assarzadegan N , Misdraji J , Shih AR , Agostini-Vulaj D , Meis JM , Tang S , Chatterjee D , Kang LI , Hart J , Lee SM , Smith T , Yantiss RK , Hissong EM , Gao ZH , Wu J , Resnick MB , Wu EY , Pai RK , Zhao L , Doyle LA , Chopra S , Panarelli NC , Hu S , Longacre TA , Raghavan SS , Lauwers GY , Ghayouri M , Cooper HS , Nagarathinam R , Bellizzi AM , Kakar S , Hosseini M , Rong J , Greenson JK , Lamps LW , Dong Z , Bronner MP
Smooth muscle tumors of the gastrointestinal tract: an analysis of prognostic features in 407 cases
Mod Pathol. 2020 Jul;33(7) :1410-1419
PMID: 32051556    PMCID: PMC8405135    URL: https://www.ncbi.nlm.nih.gov/pubmed/32051556
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Smooth muscle tumors represent the second most common mural mesenchymal neoplasm in the gastrointestinal tract, but established criteria for prognostic assessment of these tumors are lacking. A large cohort of surgically resected intramural gastrointestinal smooth muscle tumors from 31 institutions was analyzed to identify potential prognostic features. Pathologic features were assessed by expert gastrointestinal and/or soft tissue pathologists at each center. Immunohistochemical confirmation was required. A total of 407 cases from the esophagus (n = 97, 24%), stomach (n = 180, 44%), small bowel (n = 74, 18%), and colorectum (n = 56, 14%) were identified. Patients ranged in age from 19 to 92 years (mean 55 years), with a slight female predominance (57%). Mean tumor size was 5.4 cm, with the largest tumor measuring 29 cm. Disease progression following surgery, defined as local recurrence, metastasis, or disease-related death, occurred in 56 patients (14%). Colorectal tumors were most likely to progress, followed by small bowel and gastric tumors. None of the esophageal tumors in this series progressed. Receiver operator characteristic analysis identified optimal cutoffs of 9.8 cm and 3 mitoses/5 mm(2) for discriminating between progressive and non-progressive tumors. Histologic features strongly associated with progression by univariate analysis included moderate-to-severe atypia, high cellularity, abnormal differentiation (defined as differentiation not closely resembling that of normal smooth muscle), tumor necrosis, mucosal ulceration, lamina propria involvement, and serosal involvement (P < 0.0001 for all features). Age, sex, and margin status were not significantly associated with progression (P = 0.23, 0.82, and 0.07, respectively). A risk assessment table was created based on tumor site, size, and mitotic count, and Kaplan-Meier plots of progression-free survival for each subgroup revealed progression-based tiers. Based on our findings, it appears that nonesophageal gastrointestinal smooth muscle tumors measuring >10 cm and/or showing ≥3 mitoses/5 mm(2) may behave aggressively, and therefore close clinical follow-up is recommended in these cases.
1530-0285 Alpert, Lindsay Al-Sabti, Ram Graham, Rondell P Orcid: 0000-0002-8686-4867 Pai, Rish K Orcid: 0000-0002-2692-221x Gonzalez, Raul S Orcid: 0000-0001-5526-4220 Zhang, Xuefeng Smith, Vanessa Orcid: 0000-0002-0431-503x Wang, Hanlin L Westbrook, Lindsey Orcid: 0000-0002-7357-0421 Goldblum, John R Bakhshwin, Ahmed Shetty, Sindhu Klimstra, David S Shia, Jinru Orcid: 0000-0002-4351-2511 Askan, Gokce Robert, Marie E Thomas, Courtney Frankel, Wendy L Alsomali, Mohammed Hagen, Catherine Mostafa, Mohamed E Feely, Michael M Assarzadegan, Naziheh Misdraji, Joseph Shih, Angela R Agostini-Vulaj, Diana Orcid: 0000-0002-3847-0302 Meis, Jeanne M Tang, Sherry Chatterjee, Deyali Kang, Liang-I Hart, John Lee, Sang Mee Smith, Theresa Yantiss, Rhonda K Hissong, Erika M Gao, Zu-Hua Wu, JingBo Resnick, Murray B Wu, Elizabeth Yiru Pai, Reet K Zhao, Lei Doyle, Leona A Chopra, Shefali Panarelli, Nicole C Hu, Shaomin Longacre, Teri A Raghavan, Shyam Sampath Orcid: 0000-0002-3816-1542 Lauwers, Gregory Y Ghayouri, Masoumeh Cooper, Harry S Nagarathinam, Rajeswari Bellizzi, Andrew M Kakar, Sanjay Hosseini, Mojgan Rong, Juan Greenson, Joel K Lamps, Laura W Dong, Zachary Bronner, Mary P P30 CA008748/CA/NCI NIH HHS/United States P30 CA013330/CA/NCI NIH HHS/United States P50 CA174521/CA/NCI NIH HHS/United States Journal Article Research Support, N.I.H., Extramural United States Mod Pathol. 2020 Jul;33(7):1410-1419. doi: 10.1038/s41379-020-0492-5. Epub 2020 Feb 12.