Multiple recurrences and metastases in abdominal cavity following surgery for a ruptured giant gastrointestinal stromal tumor: a case report

Background: Gastrointestinal stromal tumors (GISTs) are a rare subset of gastrointestinal neoplasms, originating from the interstitial cells of Cajal and exhibiting diverse tumor biology. The vast majority (85%) of GISTs are driven by mutations in the KIT or platelet-derived growth factor receptor alpha (PDGFRA) genes. Surgery remains the preferred treatment for GIST, yet recurrence and metastasis are common postoperatively. Gastrointestinal stromal tumours that recur in the abdominal cavity after surgery and are multifocal are a relatively complex condition. Appropriate and effective drug selection and timing of surgery can improve patients’ quality of life and reduce the risk of recurrence. Case Description: A 41-year-old male patient initially underwent surgical resection for a ruptured giant GIST and subsequently received adjuvant imatinib therapy for 3 years. One year following the discontinuation of imatinib, the patient was found to have multiple recurrences and metastases within the abdominal cavity. Ripretinib was administered after a second surgery, and no tumor recurrence was found on abdominal computed tomography (CT) 1 year after the surgery. Conclusions: Although most gastrointestinal stromal tumours grow indolently, it is very rare for patients to develop large tumours that rupture. At the same time, this patient was able to respond to medication and undergo microscopic residual tumor (R0) surgery after a recurrence, demonstrating the benefits of post-operative management and comprehensive treatment. For patients with high-risk or recurrent GISTs, adequate disease assessment, proper timing of surgery, and timely targeted therapy in accordance with gene mutation status are essential. Thus, our current case report may inform the management and prognostic assessment of GISTs.

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Publication Details

Journal
Gastrointestinal Stromal Tumor
Published
2024-12-01
DOI
https://doi.org/10.21037/gist-24-8
Primary Topic
Gastrointestinal Tumor Research and Treatment
Type
article
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article

Multiple recurrences and metastases in abdominal cavity following surgery for a ruptured giant gastrointestinal stromal tumor: a case report

Pan Nie, Xinxi Yang, Pengsen Guo, Nicolò Fabbri et al.
Gastrointestinal Stromal Tumor
Gastrointestinal Tumor Research and Treatment
article

Multiple recurrences and metastases in abdominal cavity following surgery for a ruptured giant gastrointestinal stromal tumor: a case report

Pan Nie, Xinxi Yang, Pengsen Guo, Nicolò Fabbri, Yanjun Liu, Yingxin Wu
article en

Abstract

Background: Gastrointestinal stromal tumors (GISTs) are a rare subset of gastrointestinal neoplasms, originating from the interstitial cells of Cajal and exhibiting diverse tumor biology. The vast majority (85%) of GISTs are driven by mutations in the KIT or platelet-derived growth factor receptor alpha (PDGFRA) genes. Surgery remains the preferred treatment for GIST, yet recurrence and metastasis are common postoperatively. Gastrointestinal stromal tumours that recur in the abdominal cavity after surgery and are multifocal are a relatively complex condition. Appropriate and effective drug selection and timing of surgery can improve patients’ quality of life and reduce the risk of recurrence. Case Description: A 41-year-old male patient initially underwent surgical resection for a ruptured giant GIST and subsequently received adjuvant imatinib therapy for 3 years. One year following the discontinuation of imatinib, the patient was found to have multiple recurrences and metastases within the abdominal cavity. Ripretinib was administered after a second surgery, and no tumor recurrence was found on abdominal computed tomography (CT) 1 year after the surgery. Conclusions: Although most gastrointestinal stromal tumours grow indolently, it is very rare for patients to develop large tumours that rupture. At the same time, this patient was able to respond to medication and undergo microscopic residual tumor (R0) surgery after a recurrence, demonstrating the benefits of post-operative management and comprehensive treatment. For patients with high-risk or recurrent GISTs, adequate disease assessment, proper timing of surgery, and timely targeted therapy in accordance with gene mutation status are essential. Thus, our current case report may inform the management and prognostic assessment of GISTs.

Gastrointestinal Stromal TumorVol. 7
Third People's Hospital of Chengdu (CN), Azienda Unità Sanitaria Locale di Ferrara (IT), Southwest Jiaotong University (CN)
Good health and well-being
Openalex Percentile: Top 56%
Gastrointestinal Tumor Research and Treatment
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