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Co-Localization of Gastrointestinal Stromal Tumors (GIST) and Peritoneal Mesothelioma: A Case Series

  • Peritoneal Surface Malignancy
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript



Gastrointestinal stromal tumor (GIST) is associated with increased risk of additional cancers. In this study, synchronous GIST, and peritoneal mesothelioma (PM) were characterized to evaluate the relationship between these two cancers.


A retrospective chart review was conducted for patients diagnosed with both GIST and PM between July 2010 and June 2021. Patient demographics, past tumor history, intraoperative reports, cross-sectional imaging, peritoneal cancer index (PCI) scoring, somatic next-generation sequencing (NGS) analysis, and histology were reviewed.


Of 137 patients who underwent primary GIST resection from July 2010 to June 2021, 8 (5.8%) were found to have synchronous PM, and 4 patients (50%) had additional cancers and/or benign tumors. Five (62.5%) were male, and the median age at GIST diagnosis was 57 years (range: 45–76). Seventy-five percent of GISTs originated from the stomach. Of the eight patients, one patient had synchronous malignant mesothelioma (MM), and the remaining had well-differentiated papillary mesothelioma (WDPM), which were primarily located in the region of the primary GIST (89%). The median PCI score was 2 in the WDPM patients. NGS of GIST revealed oncogenic KIT exon 11 (62.5%), PDGFRA D842V (25%), or SDH (12.5%) mutations, while NGS of the MM revealed BAP1 and PBRM1 alterations.


One in 17 GIST patients undergoing resection in this series have PM, which is significantly higher than expected if these two diseases were considered as independent events. Our results indicate that synchronous co-occurrence of GIST and PM is an underrecognized finding, suggesting a possible relationship that deserves further investigation.

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  1. Ma GL, Murphy JD, Martinez ME, et al. Epidemiology of gastrointestinal stromal tumors in the era of histology codes: results of a population-based study. Cancer Epidemiol Biomark Prev. 2015;24:298–302.

    Article  Google Scholar 

  2. Patil DT, Rubin BP. Gastrointestinal stromal tumor: advances in diagnosis and management. Arch Pathol Lab Med. 2011;135:1298–310.

    Article  CAS  Google Scholar 

  3. Murphy JD, Ma GL, Baumgartner JM, et al. Increased risk of additional cancers among patients with gastrointestinal stromal tumors: a population-based study. Cancer. 2015;121:2960–7.

    Article  Google Scholar 

  4. Ferreira SS, Werutsky G, Toneto MG, et al. Synchronous gastrointestinal stromal tumors (GIST) and other primary cancers: case series of a single institution experience. Int J Surg. 2010;8:314–7.

    Article  Google Scholar 

  5. Ponti G, Luppi G, Martorana D, et al. Gastrointestinal stromal tumor and other primary metachronous or synchronous neoplasms as a suspicion criterion for syndromic setting. Oncol Rep. 2010;23:437–44.

    CAS  PubMed  Google Scholar 

  6. Chen CI, Castellanos MB, Ruch MA, et al. Gastrointestinal stromal tumor with synchronous colorectal adenocarcinoma. QJM. 2017;110:591–2.

    Article  Google Scholar 

  7. Comandini D, Damiani A, Pastorino A. Synchronous GISTs associated with multiple sporadic tumors: a case report. Drugs Context. 2017;6:212307.

    Article  Google Scholar 

  8. Pandurengan RK, Dumont AG, Araujo DM, et al. Survival of patients with multiple primary malignancies: a study of 783 patients with gastrointestinal stromal tumor. Ann Oncol. 2010;21:2107–11.

    Article  CAS  Google Scholar 

  9. Greenbaum A, Alexander HR. Peritoneal mesothelioma. Transl Lung Cancer Res. 2020;9:S120–32.

    Article  CAS  Google Scholar 

  10. Sugarbaker PH, Welch LS, Mohamed F, et al. A review of peritoneal mesothelioma at the Washington cancer institute. Surg Oncol Clin N Am. 2003;12(605–21):xi.

    Google Scholar 

  11. Cheung M, Testa JR. BAP1, a tumor suppressor gene driving malignant mesothelioma. Transl Lung Cancer Res. 2017;6:270–8.

    Article  CAS  Google Scholar 

  12. Jacquet P, Sugarbaker PH. Clinical research methodologies in diagnosis and staging of patients with peritoneal carcinomatosis. Cancer Treat Res. 1996;82:359–74.

    Article  CAS  Google Scholar 

  13. Sun M, Zhao L, Yu L, et al. Well differentiated papillary mesothelioma: a 17 year single institution experience with a series of 75 cases. Ann Diagn Pathol. 2019;38:43–50.

    Article  Google Scholar 

  14. Smith MJ, Smith HG, Mahar A, et al. The impact of additional malignancies in patients diagnosed with gastrointestinal stromal tumors. Int J Cancer. 2016;139(8):1744–51.

    Article  CAS  Google Scholar 

  15. Stevers M, Rabban JT, Garg K, et al. Well-differentiated papillary mesothelioma of the peritoneum is genetically defined by mutually exclusive mutations in TRAF7 and CDC42. Mod Pathol. 2019;32:88–99.

    Article  CAS  Google Scholar 

  16. Shrestha R, Nabavi N, Volik S, et al. Well-differentiated papillary mesothelioma of the peritoneum is genetically distinct from malignant mesothelioma. Cancers (Basel). 2020;12:1568.

    Article  CAS  Google Scholar 

  17. Yu W, Chan-On W, Teo M, et al. First somatic mutation of E2F1 in a critical DNA binding residue discovered in well-differentiated papillary mesothelioma of the peritoneum. Genome Biol. 2011;12:R96.

    Article  CAS  Google Scholar 

  18. de la Torre J, Banerjee S, Baumgartner J, et al. Tumor symbiosis: gastrointestinal stromal tumor as a host for primary peritoneal mesothelioma. J Gastrointest Surg. 2019;23:879–81.

    Article  Google Scholar 

  19. Kim M, Kim HS. Clinicopathological characteristics of well-differentiated papillary mesothelioma of the peritoneum: a single-institutional experience of 12 cases. In vivo. 2019;33:633–42.

    Article  Google Scholar 

  20. Malpica A, Sant’Ambrogio S, Deavers MT, et al. Well-differentiated papillary mesothelioma of the female peritoneum: a clinicopathologic study of 26 cases. Am J Surg Pathol. 2012;36:117–27.

    Article  Google Scholar 

  21. Rathi V, Hyde S, Newman M. Well-differentiated papillary mesothelioma in association with endometrial carcinoma: a case report. Acta Cytol. 2010;54(5 Suppl):793–7.

    PubMed  Google Scholar 

  22. McGinnis JM, Bloomfield V, Kazerouni H, Helpman L. Well-differentiated papillary mesothelioma with two synchronous serous gynaecologic carcinomas in a 62-year-old woman: lessons learned for the gynaecologic surgeon. J Obstet Gynaecol Can. 2020;42(10):1262–6.

    Article  PubMed  Google Scholar 

  23. Atay S, Banskota S, Crow J, et al. Oncogenic KIT-containing exosomes increase gastrointestinal stromal tumor cell invasion. Proc Natl Acad Sci. 2014;111:711–6.

    Article  CAS  Google Scholar 

  24. Atay S, Godwin AK. Tumor-derived exosomes: a message delivery system for tumor progression. Commun Integr Biol. 2014;7:e28231.

    Article  Google Scholar 

  25. Atay S, Wilkey DW, Milhem M, et al. Insights into the proteome of gastrointestinal stromal tumors-derived exosomes reveals new potential diagnostic biomarkers. Mol Cell Proteomics. 2018;17:495–515.

    Article  CAS  Google Scholar 

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Correspondence to Jason K. Sicklick MD.

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J.K.S. receives research funds from Foundation Medicine Inc. and Amgen; consultant fees from Deciphera and Grand Rounds; speakers fees from Deciphera, La-Hoffman Roche, Foundation Medicine, Merck, and QED; owns stock in Personalis. A.S. received financial support through the NIH T32 CA121938 Cancer Therapeutics (CT2) Training Fellowship. All other authors have no competing interests.

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Courelli, A.S., Sharma, A.K., Madlensky, L. et al. Co-Localization of Gastrointestinal Stromal Tumors (GIST) and Peritoneal Mesothelioma: A Case Series. Ann Surg Oncol 29, 7542–7548 (2022).

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