Annals of Surgical Oncology

, Volume 22, Issue 8, pp 2633–2639 | Cite as

Additional Primary Malignancies in Patients with Gastrointestinal Stromal Tumor (GIST): A Clinicopathologic Study of 260 Patients with Molecular Analysis and Review of the Literature

  • Jaclyn Frances Hechtman
  • Ronald DeMatteo
  • Khedoudja Nafa
  • Ping Chi
  • Maria E. Arcila
  • Snjezana Dogan
  • Alifya Oultache
  • Wen Chen
  • Meera Hameed
Bone and Soft Tissue Sarcomas



The incidence of other primary neoplasms in gastrointestinal stromal tumor (GIST) patients is relatively high. Our aim was to better characterize the clinicopathologic and molecular relationships in a cohort of GIST patients.


All GIST patients with tumor samples sent for molecular testing were identified via electronic medical records. Clinicopathologic characteristics of GIST and additional primary malignancies were analyzed.


Of 260 patients, 50 (19 %) had at least one additional primary malignancy. In 33 patients, separate primary neoplasms predated their GIST diagnosis and most commonly included: prostate (n = 9), breast (n = 8), and hematologic (n = 5). Renal (n = 4) and hematologic (n = 3) malignancies were the most frequent cancers identified after GIST diagnosis. The majority (8 of 12, 66 %) of malignancies diagnosed after GIST were found incidentally. Patients who developed other malignancies after GIST more often had KIT exon 11 mutations (100 vs. 66 %, P = 0.01). In comparison to patients with only GIST, patients with a second primary neoplasm of any chronology had GISTs with increased mitotic rate (≥5 per 50 high-power fields) (P = 0.0006). Literature review revealed colorectal cancer, gastric, prostate, renal, leukemia, and desmoid-type fibromatosis as the most common secondary neoplasms.


Nineteen percent of GIST patients develop other malignancies. This is the first report to describe a relationship between additional primary malignancy and both mutation and mitotic rate of GIST. Although the basis of these relationships remains to be investigated, caution in the clinical management of GIST patients with additional lesions is warranted.


Renal Cell Carcinoma Imatinib Chronic Lymphocytic Leukemia Papillary Thyroid Carcinoma Seminoma 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.



The authors declare no conflict of interest.

Supplementary material

10434_2014_4332_MOESM1_ESM.docx (18 kb)
Supplementary material 1 (DOCX 18 kb)


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Copyright information

© Society of Surgical Oncology 2015

Authors and Affiliations

  • Jaclyn Frances Hechtman
    • 1
  • Ronald DeMatteo
    • 2
  • Khedoudja Nafa
    • 1
  • Ping Chi
    • 3
  • Maria E. Arcila
    • 1
  • Snjezana Dogan
    • 1
  • Alifya Oultache
    • 1
  • Wen Chen
    • 4
  • Meera Hameed
    • 1
  1. 1.Department of PathologyMemorial Sloan Kettering Cancer CenterNew YorkUSA
  2. 2.Department of SurgeryMemorial Sloan Kettering Cancer CenterNew YorkUSA
  3. 3.Department of MedicineMemorial Sloan Kettering Cancer CenterNew YorkUSA
  4. 4.Department of PathologyVeterans’ Affairs HospitalWashingtonUSA

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