Tailored management of primary gastrointestinal stromal tumors

Abstract

Gastrointestinal stromal tumors (GISTs) are the most common human sarcoma and can form along the entire gastrointestinal tract. Over the last 20 years, considerable advances have been made in our understanding of the biology of GISTs. The advent of tyrosine kinase inhibitors has provided effective medical therapy for the first time. In fact, given that GIST typically is driven by either a KIT or PDGFRA gene mutation, it has become a paradigm of targeted molecular therapy. In addition, diagnostic and surgical techniques have been refined. Here, the critical aspects of primary GISTs and how they are now managed with an integrated approach are summarized. Treatment plans are developed based on specific pathologic and molecular features of the tumor. The authors outline the general principles of therapy and highlight some of the nuances. Particular focus is given to diagnosis, surgical considerations, and the use of preoperative and postoperative tyrosine kinase inhibitors.

<p>Endoscopic or Surgical Resection for Patients with 2–5cm Gastric Gastrointestinal Stromal Tumors: A Single-Center 12-Year Experience from China</p>

“…The gene mutation analysis is helpful for the diagnosis of some ambiguous cases, to predict the effectiveness of targeted drugs and provide guidance for clinical treatment. 10 The analysis of gene mutations included exons 9, 11, 13 and 17 of C-KIT and exons 12 and 18 of PDGFRA. Mutation analysis of these genes was performed in 31 patients.…”

Evaluating Treatment Patterns of Gastrointestinal Stromal Tumors in the Elderly and Predicting Outcomes by a Validated Model: A Population-based Study

“…As expected, a similar trend was observed in patients aged 65-74 years, of whom cases with surgery alone or combined therapy contributed to a better prognosis. Despite previous ndings have revealed signi cant bene ts from adjuvant therapy [9,32], as for the discrepancy that patients treated with chemotherapy alone did not derive survival bene t, this is partly because localized GISTs in this subgroup accounted for 87% in which surgery inclined to provide a clear survival advantage compared to chemotherapy alone [33]. Of importance, in our present study, treatment patterns, perceived as an independent predictor of OS, further manifested the durable improvement in survival of surgery and combined therapy.…”

Pretreatment Tumor DNA Sequencing of KIT and PDGFRA in Endosonography-Guided Biopsies Optimizes the Preoperative Management of Gastrointestinal Stromal Tumors

“…The criteria to be used to recommend neoadjuvant treatment in GIST remain to be determined [43,44]. Treatment decisions are dependent on numerous factors, such as tumor size, tumor position, the existence of metastases, and the performance status of the patient.…”