With deeper understanding of gastrointestinal stromal tumor(GIST), more and more patients are diagnosed as GIST. Although the prognosis of early GIST is satisfactory after complete surgical resection, there are still many problems in the treatment of advanced GIST. Variety of treatment options has been used in the treatment of GIST, such as surgery, targeted drug therapy, and surgery plus imatinib therapy. However, post-operative recurrence, imatinib-resistance, multi-targeted drug resistance are still challenges. Many clinical evidences show that a reasonable management strategy can improve the prognosis of patients with advanced GIST. All the doctors should have a clear mind to carry out appropriate interventions. Advanced GIST should not be simply considered to be either medical or surgical disease, but rather must be systematically managed by multidisciplinary team approach combining surgical oncology, medical oncology, pathology, and interventional medicine. This review will advocate suitable treatment strategies based on the most recent progresses in systemic treatment for advanced GIST and our clinical experience to achieve early detection, early prevention, proper management, and therefore improve the survival of these patients.
Gastrointestinal Stromal Tumors ; diagnosis ; drug therapy ; pathology ; surgery ; Humans ; Neoplasm Metastasis

OBJECTIVETo analyze the efficacy and prognosis of different treatments on small intestinal gastrointestinal stromal tumors(SIGIST).

METHODSClinical data of 63 patients with SIGIST who were admitted to the Chinese PLA General Hospital from January 2004 to December 2013 were analyzed retrospectively. According to resection procedure and postoperative use of imatinib, patients were divided into R0 resection plus imatinib group (13 cases), R0 resection without imatinib group (42 cases), non-R0 resection plus imatinib group (7 cases), non-R0 resection without imatinib group (1 case). Survival was compared among groups. Result All the patients were followed up with a median length of 24 months(3 to 120 months), and the over survival (OS) rates at 1-year, 3-year, 5-year were 97%, 94% and 80%. In R0 resection plus imatinib group, R0 resection without imatinib group, and non-R0 resection plus imatinib group, the progression free survival(PFS) time was 24, 24 and 23 months; the 1-year PFS were 100%, 97% and 83%; the 3-year PFS were 100%, 45% and 83%; the 5-year PFS were 100%, 28% and 42%. R0 resection plus imatinib group had significantly higher PFS(all P<0.05). The case of non-R0 resection without imatinib died 6 months after operation. Among 55 patients undergoing R0 resection, recurrence was found in 16 patients, whose recurrence rates of 1-year, 3-yeart and 5-year were 2%,43% and 58%. Local recurrence was found in 8 cases, hepatic recurrence in 3 cases and widespread recurrence in 5 cases, who received simple imatinib, operation plus imatinib and imatinib intervention, with median survival time of 66.5 months, 92.5 months and 48 months respectively. One patient initiatively abandoned treatment and died 17 months later.

CONCLUSIONThe total resection and postoperative imatinib administration can improve the prognosis and raise the progression free survival of patients with small intestinal stromal tumors.

Antineoplastic Agents ; therapeutic use ; Benzamides ; therapeutic use ; Disease-Free Survival ; Gastrointestinal Stromal Tumors ; drug therapy ; surgery ; Humans ; Imatinib Mesylate ; Intestinal Neoplasms ; drug therapy ; pathology ; surgery ; Intestine, Small ; pathology ; Neoplasm Recurrence, Local ; Piperazines ; therapeutic use ; Prognosis ; Pyrimidines ; therapeutic use ; Retrospective Studies

OBJECTIVETo investigate treatment and prognostic factors of gastrointestinal stromal tumor(GIST) in the small intestine.

METHODSThe clinicopathological data of 64 patients with GIST in the small intestine admitted to the Tianjin Medical University Affiliated Cancer Hospital between April 2002 and November 2010 were analyzed retrospectively and the prognostic factors were evaluated.

RESULTSNo patients in this cohort received chemotherapy or radiation therapy. Fourteen patients underwent post-operative imatinib targeted therapy. The overall 5-year survival rate was 51.2% and the post-operative recurrence rate of 61 cases undergoing R0 resection was 44.3%. Univariate analysis revealed that the complete tumor resection(P=0.001), tumor size(P=0.018), adhesion or invasion to surrounding tissue and organs (P=0.015), concurrent distant metastasis(P=0.000), tumor hemorrhage (P=0.032), Fletcher classification (P=0.027) and symptom(P=0.012) were associated with prognosis. Multivariate analysis demonstrated that adhesion or invasion to surrounding tissue and organs(P=0.026), concurrent distant metastasis(P=0.000) and symptom(P=0.019) were independent prognostic factors for survival.

CONCLUSIONThe survival evaluation of patients with small intestinal GIST depends on surgery, tumor size, adhesion or invasion to surrounding tissue and organs, concurrent distant metastasis, tumor hemorrhage, symptom, Fletcher classification, and use of targeted therapy.

Adult ; Aged ; Aged, 80 and over ; Female ; Follow-Up Studies ; Gastrointestinal Stromal Tumors ; drug therapy ; surgery ; Humans ; Intestinal Neoplasms ; drug therapy ; surgery ; Intestine, Small ; pathology ; Male ; Middle Aged ; Prognosis ; Retrospective Studies

Despite the rarity in incidence and prevalence, gastrointestinal stromal tumor (GIST) has emerged as a distinct pathogenetic entity. And the clinical management of GIST has been evolving very rapidly due to the recent recognition of its oncogenic signal transduction pathway and the introduction of new molecular-targeted therapy. Successful management of GIST requires a multidisciplinary approach firmly based on accurate histopathologic diagnosis. However, there was no standardized guideline for the management of Korean GIST patients. In 2007, the Korean GIST study group (KGSG) published the first guideline for optimal diagnosis and treatment of GIST in Korea. As the second version of the guideline, we herein have updated recent clinical recommendations and reflected changes in diagnosis, surgical and medical treatments for more optimal clinical practice for GIST in Korea. We hope the guideline can be of help in enhancing the quality of diagnosis by members of the Korean associate of physicians involving in GIST patients's care and subsequently in achieving optimal efficacy of treatment.
Antineoplastic Agents/therapeutic use ; Gastrointestinal Stromal Tumors/*pathology/surgery/*therapy ; Humans ; Neoadjuvant Therapy ; Piperazines/therapeutic use ; Proto-Oncogene Proteins c-kit/metabolism ; Pyrimidines/therapeutic use ; Recurrence ; Republic of Korea ; Tumor Markers, Biological/metabolism

OBJECTIVETo investigate the efficacy of targeted therapy combined with surgery in the treatment of recurrent and metastatic gastrointestinal stromal tumor(GIST).

METHODSClinicopathological and followed-up data of 318 patients with recurrent and metastatic GIST admitted in Zhongshan Hospital between January 2000 and December 2015 were analyzed retrospectively. According to different treatment methods, the patients were divided into four groups: surgery group (operation alone, 44 cases), target therapy group (imatinib alone, 108 cases), target therapy combined with surgery group (imatinib plus operation, 139 cases), other therapy group (chemotherapy, Chinese medicine and others, 27 cases). The progression-free survival (PFS) and overall survival (OS) of four groups were compared.

RESULTSThe baseline informations, such as age, gender, primary site, et al, were not significantly different (all P>0.05), but the recurrent and metastatic site was significantly different among 4 groups (P=0.000). The medial PFS of surgery group, target therapy group, target therapy combined with surgery was 16(95%CI: 4.9 to 27.0) months, 44 (95%CI: 30.9 to 57.1) months, 35 (95%CI: 26.5 to 43.5) months, respectively, and the latter 2 groups had significantly longer PFS than surgery group(P=0.000), while no significant difference was found between target therapy group and target combined with surgery group (P=0.251). The median OS of surgery group, target therapy group, target therapy combined with surgery, and other therapy group was 24 (95%CI: 9.0 to 39.0) months, 69(95%CI: 40.8 to 97.2) months, 92(95%CI: 78.0 to 106.0) months, 12(95%CI: 9.5 to 14.5) months. Target therapy group and target therapy combined with surgery group had significantly longer OS than surgery and other therapy groups (P=0.000), while the target therapy combined with surgery group had significantly longer OS than target therapy group(P=0.028).

CONCLUSIONTarget therapy combined with surgery can prolong the survival of recurrent and metastatic GIST patients.

Antineoplastic Agents ; therapeutic use ; Benzamides ; Combined Modality Therapy ; Disease-Free Survival ; Female ; Gastrointestinal Stromal Tumors ; drug therapy ; pathology ; surgery ; Humans ; Imatinib Mesylate ; therapeutic use ; Male ; Middle Aged ; Piperazines ; Pyrimidines ; Retrospective Studies

Adolescent ; Adult ; Antigens, CD34 ; metabolism ; Antineoplastic Agents ; therapeutic use ; Benzamides ; Chondroma ; drug therapy ; metabolism ; pathology ; surgery ; Female ; Follow-Up Studies ; Gastrectomy ; Gastrointestinal Stromal Tumors ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Imatinib Mesylate ; Lung Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Neoplasm Recurrence, Local ; Neoplasms, Multiple Primary ; drug therapy ; metabolism ; pathology ; surgery ; Piperazines ; therapeutic use ; Pneumonectomy ; Proto-Oncogene Proteins c-kit ; metabolism ; Pyrimidines ; therapeutic use

Gastrointestinal stromal tumor (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract arising from Cajal's cells, expressing CD 117. The standard treatment for primary GIST is complete surgical resection. Imatinib mesylate, a specific tyrosine kinase inhibitor, is effective against locally advanced and metastatic GIST. There are several reports of the effect of preoperative imatinib in patients with unresectable and locally advanced primary GIST. We report a case of unresectable primary GIST of the ampulla of Vater, which we were able to completely resect after treatment with a dosage of imatinib 400 mg daily for 5 months. Twelve months later, the patient was treated with imatinib and doing well with no evidence of recurrence.
Ampulla of Vater/*pathology ; Antineoplastic Agents/*therapeutic use ; Duodenoscopy ; Gastrointestinal Stromal Tumors/diagnosis/drug therapy/*surgery ; Humans ; Male ; Middle Aged ; Piperazines/*therapeutic use ; Pyrimidines/*therapeutic use ; Tomography, X-Ray Computed

Aged ; Antineoplastic Agents ; therapeutic use ; Benzamides ; therapeutic use ; Drug Resistance, Neoplasm ; Exons ; Gastrectomy ; methods ; Gastrointestinal Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Gastrointestinal Stromal Tumors ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Imatinib Mesylate ; Liver Neoplasms ; drug therapy ; secondary ; Male ; Piperazines ; therapeutic use ; Point Mutation ; Proto-Oncogene Proteins c-kit ; genetics ; metabolism ; Pyrimidines ; therapeutic use

Over the past 60 years, investigators of basic science, pathology, and clinical medicine have studied gastrointestinal stromal tumor (GIST) and made minor advances in patient care. Recent discoveries have led to an understanding of the biological role of KIT and platelet-derived growth factor receptor-α in GIST and the development of the tyrosine kinase inhibitor imatinib mesylate (Gleevec, formerly STI-571), one of the most exciting examples of targeted therapy to date. The success of targeted therapy in GIST has lead to new developments in our understanding of the medical and surgical management of the disease. Intense study of GIST may lead to new paradigms in the management of cancer.
Antineoplastic Agents ; therapeutic use ; Benzamides ; Combined Modality Therapy ; Drug Delivery Systems ; Gastrointestinal Neoplasms ; drug therapy ; genetics ; pathology ; surgery ; Gastrointestinal Stromal Tumors ; drug therapy ; genetics ; pathology ; surgery ; Humans ; Imatinib Mesylate ; Mutation ; Piperazines ; therapeutic use ; Protein Kinase Inhibitors ; therapeutic use ; Proto-Oncogene Proteins c-kit ; genetics ; metabolism ; Pyrimidines ; therapeutic use ; Receptor, Platelet-Derived Growth Factor alpha ; genetics ; metabolism

Antineoplastic Agents ; therapeutic use ; Benzamides ; Chemotherapy, Adjuvant ; Gastrointestinal Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Gastrointestinal Stromal Tumors ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Imatinib Mesylate ; Indoles ; therapeutic use ; Mutation ; Neoplasm Staging ; Piperazines ; therapeutic use ; Proto-Oncogene Proteins c-kit ; metabolism ; Pyrimidines ; therapeutic use ; Pyrroles ; therapeutic use ; Receptor, Epidermal Growth Factor ; genetics ; metabolism ; Vascular Endothelial Growth Factor A ; metabolism