Smooth muscle tumors of the gastrointestinal tract: An analysis of prognostic features in 407 cases Journal Article


Authors: Alpert, L.; Al-Sabti, R.; Graham, R. P.; Pai, R. K.; Gonzalez, R. S.; Zhang, X.; Smith, V.; Wang, H. L.; Westbrook, L.; Goldblum, J. R.; Bakhshwin, A.; Shetty, S.; Klimstra, D. S.; Shia, J.; Askan, G.; Robert, M. E.; Thomas, C.; Frankel, W. L.; Alsomali, M.; Hagen, C.; Mostafa, M. E.; Feely, M. M.; Assarzadegan, N.; Misdraji, J.; Shih, A. R.; Agostini-Vulaj, D.; Meis, J. M.; Tang, S.; Chatterjee, D.; Kang, L. I.; Hart, J.; Lee, S. M.; Smith, T.; Yantiss, R. K.; Hissong, E. M.; Gao, Z. H.; Wu, J. B.; Resnick, M. B.; Wu, E. Y.; Pai, R. K.; Zhao, L.; Doyle, L. A.; Chopra, S.; Panarelli, N. C.; Hu, S.; Longacre, T. A.; Raghavan, S. S.; Lauwers, G. Y.; Ghayouri, M.; Cooper, H. S.; Nagarathinam, R.; Bellizzi, A. M.; Kakar, S.; Hosseini, M.; Rong, J.; Greenson, J. K.; Lamps, L. W.; Dong, Z.; Bronner, M. P.
Article Title: Smooth muscle tumors of the gastrointestinal tract: An analysis of prognostic features in 407 cases
Abstract: 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 mm2 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 mm2 may behave aggressively, and therefore close clinical follow-up is recommended in these cases. © 2020, The Author(s), under exclusive licence to United States & Canadian Academy of Pathology.
Keywords: immunohistochemistry; adult; controlled study; aged; cancer surgery; young adult; major clinical study; clinical feature; cancer recurrence; cancer growth; follow up; progression free survival; tumor volume; tumor differentiation; cohort analysis; cell differentiation; cancer mortality; risk assessment; disease severity; colorectal tumor; sex difference; disease exacerbation; muscle tumor; surgical margin; tumor necrosis; gastrointestinal tumor; lamina propria; small intestine tumor; cancer prognosis; human; male; female; priority journal; article
Journal Title: Modern Pathology
Volume: 33
Issue: 7
ISSN: 0893-3952
Publisher: Nature Research  
Date Published: 2020-07-01
Start Page: 1410
End Page: 1419
Language: English
DOI: 10.1038/s41379-020-0492-5
PUBMED: 32051556
PROVIDER: scopus
PMCID: PMC8405135
DOI/URL:
Notes: Article -- Export Date: 3 August 2020 -- Source: Scopus
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  1. David S Klimstra
    978 Klimstra
  2. Jinru Shia
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  3. Gokce Askan
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