Korean J Radiol.  2017 Feb;18(1):84-93. 10.3348/kjr.2017.18.1.84.

Update on Gastrointestinal Stromal Tumors for Radiologists

• Tirumani S
• Baheti AD
• Tirumani H
• O'Neill A
• Jagannathan JP

• KMID: 2468124
• DOI: http://doi.org/10.3348/kjr.2017.18.1.84

Abstract

The management of gastrointestinal stromal tumors (GISTs) has evolved significantly in the last two decades due to better understanding of their biologic behavior as well as development of molecular targeted therapies. GISTs with exon 11 mutation respond to imatinib whereas GISTs with exon 9 or succinate dehydrogenase subunit mutations do not. Risk stratification models have enabled stratifying GISTs according to risk of recurrence and choosing patients who may benefit from adjuvant therapy. Assessing response to targeted therapies in GIST using conventional response criteria has several potential pitfalls leading to search for alternate response criteria based on changes in tumor attenuation, volume, metabolic and functional parameters. Surveillance of patients with GIST in the adjuvant setting is important for timely detection of recurrences.

Keyword

Gastrointestinal stromal tumor; Imatinib; Risk stratification; Choi criteria

MeSH Terms

Antineoplastic Agents/therapeutic use
Benzamides/therapeutic use
Chemotherapy, Adjuvant
Combined Modality Therapy
Exons
Gastrointestinal Neoplasms/*diagnostic imaging/drug therapy/genetics
Gastrointestinal Stromal Tumors/*diagnostic imaging/drug therapy/genetics
Humans
Imatinib Mesylate/therapeutic use
Mutation
Neoplasm Recurrence, Local
Pyrimidines/therapeutic use
Succinate Dehydrogenase/genetics
Tomography, X-Ray Computed
Antineoplastic Agents
Benzamides
Pyrimidines
Imatinib Mesylate
Succinate Dehydrogenase

Figure

• 

  Fig. 1 60-year-old man with gastric gastrointestinal stromal tumor with exon 11 mutation. A. Axial contrast-enhanced CT image at time of diagnosis demonstrates large 13 cm gastric mass (arrows). Biopsy of mass revealed gastrointestinal stromal tumor with exon 11 mutation. Patient was treated with imatinib in neoadjuvant setting to downsize tumor. B. Follow-up CT after 3 months of imatinib therapy shows marked decrease in enhancing component in mass with no significant change in size (arrows). Patient underwent surgery with no evidence of recurrence at time of last follow-up 4 years later. CT = computed tomography

• 

  Fig. 2 53-year-old woman with small bowel gastrointestinal stromal tumor with exon 9 mutation. A. Axial contrast-enhanced CT image of lower abdomen reveals large cavitating mass in lower abdomen surrounded by small bowel loops (arrows). Patient underwent surgery which revealed small bowel mass. Histopathology revealed gastrointestinal stromal tumor with exon 9 mutation. B. Three months after surgery follow-up CT scan demonstrated recurrent pelvic mass (arrows). Patient was treated with high dose imatinib. C. CT scan after 6 months of treatment showed significant decrease in size of pelvic mass (arrows). Pelvic mass was excised and patient was restarted on high dose imatinib. D. Repeat CT scan after 4 months of treatment showed recurrence in form of multiple peritoneal masses (arrows). Patient was switched to sunitinib. E. CT scan performed 2 months after start of sunitinib therapy showed decrease in density of peritoneal deposits with mild increase in size (arrows). F. Follow-up CT scan three months later showed significant increase in peritoneal sarcomatosis. Patient died two months later. CT = computed tomography

• 

  Fig. 3 41-year-old man with gastric GIST with SDH mutation. A. Axial contrast-enhanced CT image at time of diagnosis demonstrates large lobulated gastric mass (arrow). There is enlarged lymph node in gastrohepatic ligament (arrowhead). Patient was treated with neoadjuvant imatinib for one month. Follow-up CT scan showed no response to treatment and dose of imatinib was doubled. B. Repeat CT scan performed 3 months after therapy with high-dose imatinib showed no change in size of gastric mass and lymph node. Biopsy of mass at this time revealed SDH-deficient GIST. Patient was switched to sunitinib. C. CT scan after three months of treatment showed no response to treatment, instead new peritoneal nodule. Patient was taken up for surgery. D, E. Two years after surgery surveillance CT scan showed new liver metastasis (arrow, D) and peritoneal and bowel metastases (arrows, E). Patient was restarted on sunitinib. At time of last follow-up 7 years after initial diagnosis continues to have liver and peritoneal metastases which are stable in response to regorafenib therapy. CT = computed tomography, GIST = gastrointestinal stromal tumor, SDH = succinate dehydrogenase

• 

  Fig. 4 32-year-old man with anorectal gastrointestinal stromal tumor. A. Axial T2-weighted MR image of pelvis reveals 3.3 cm mass (arrow) in anal canal. Biopsy of mass showed gastrointestinal stromal tumor with mitotic count of 1 per 50 high power fields. Tumor is low risk according to NIH consesus criteria, AFIP criteria and Joensuu criteria. Patient was treated with imatinib in neoadjuvant setting to downsize tumor. B. Follow-up MRI after 3 months of imatinib therapy shows decrease in size of mass (arrow). AFIP = Armed Forces Institute of Pathology, MRI = magnetic resonance imaging, NIH = National Institute of Health

• 

  Fig. 5 67-year-old man with gastric GIST metastatic to liver. A. Axial contrast-enhanced CT image of abdomen at time of initial presentation reveals 8 cm partly necrotic gastric mass with irregular outline and enhancing solid internal component (arrow). There is large 14 cm necrotic liver mass consistent with metastasis (arrowhead). Presence of irregular outline and enhancing solid internal component on CT scan are predictive of increased risk of metastasis in GIST. Patient was treated with imatinib 400 mg. B. Follow-up CT after 12 months of imatinib therapy shows decrease in size and density of primary gastric mass (arrow) and also liver metastasis (arrowhead). C. Another CT scan performed 3 months later showed new enhancing nodule (balck arrowhead) in cystic liver metastasis (white arrowhead) consistent with recurrence. Primary gastric mass is again noted (arrow). Patient was switched to sunitinib. CT = computed tomography, GIST = gastrointestinal stromal tumor

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