Gastrointestinal stromal tumors: A multicenter study of 1160 Turkish cases

DOGUSOY, Gulen BULBUL; Group, Turkish GIST Working

doi:10.4318/tjg.2012.0342

Cited by 30 publications

(12 citation statements)

References 35 publications

(98 reference statements)

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“…Though a rare tumor, high rates of cure and improved OS is currently the norm with IM and multimodality therapy. The median age at diagnosis is usually 60 years while in our population median age is around 54 years which has been reported in other series as well (16,17). Our study shows male to female preponderance with a ratio of 2.4: while most population based studies shows no sex predilection (17)(18)(19).…”

Section: Discussionmentioning

confidence: 74%

Neoadjuvant imatinib: longer the better, need to modify risk stratification for adjuvant imatinib

Ramaswamy¹,

Jain²,

Sahu³

et al. 2016

J. Gastrointest. Oncol.

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Background: Multimodality treatment of gastrointestinal stromal tumor (GIST) with surgery and adjuvant imatinib mesylate (IM), along with an emerging role for neoadjuvant IM prior to evaluation for resectability has resulted in high survival rates. Methods: We conducted a retrospective analysis of prospectively collected data of patients who underwent surgery for GIST, prior to or followed by IM therapy. A total of 112 patients underwent surgery between January 2009 and March 2015 at our centre. This included 27 patients with upfront resectable disease, 76 patients with locally advanced GIST who received neoadjuvant IM followed by surgery and 9 patients with metastatic disease who had excellent response to IM and were taken for surgery. Results: The primary tumor in the non metastatic patients was in the stomach (53%), duodenum (16%), rectum (12%), jejunum (11%), ileum (7%), and others (2%). Median duration of neoadjuvant IM was 5 months with 4 patients showing disease progression during neoadjuvant IM. Ninety-three percent of all patients had R0 resections, while 7% had R+ resections. The estimated 3-and 5-year DFS in non-metastatic patients was 86.1% and 67% respectively with a 3-and 5-year median OS of 95.4% and 91.7% respectively.Five-year PFS and OS for the metastatic patients was 88.8% and 100% respectively. Lack of adjuvant IM was the only factor related to inferior PFS and OS. Conclusions: Longer duration of neoadjuvant IM should be considered in locally advanced GIST prior to surgery and resection may be considered in responding metastatic patients. J Gastrointest Oncol 2016;7(4):624-631 jgo.amegroups.com spillage of tumor cells (8-10). There is growing evidence for neoadjuvant IM therapy in terms of disease free survival (DFS) and overall survival (OS), with major evidence of benefit shown in the EORTC-STBSG retrospective analysis (11)(12)(13)(14)(15).The purpose of this study was to evaluate the demographic profile, presentation and outcomes of 112 patients with GIST who underwent surgery at our institution and were potential candidates for either neoadjuvant or adjuvant IM over a period of 6 years. We also attempted to identify potential prognostic factors with respect to outcomes and placed special emphasis on patients receiving neoadjuvant IM. MethodsA retrospective analysis of prospectively maintained database of all GIST patients who underwent surgery and presented between January 2009 to March 2015 at Department of GI & Hepatopancreaticobiliary Oncology, Tata Memorial Hospital, Mumbai, was performed. Clinical and radiological data were recorded from patient files and electronic medical records. Patients presenting in the above study period were subdivided on the basis of clinical presentation and treatment received into localized operable, locally advanced, operated elsewhere on adjuvant treatment and incidental GISTs. Locally advanced GISTs were defined by the size, need for multivisceral resections, anatomic proximity with major vessels and risk of intraoperative tumor spillage. Patients opera...

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Section: Discussionmentioning

confidence: 74%

Neoadjuvant imatinib: longer the better, need to modify risk stratification for adjuvant imatinib

Ramaswamy¹,

Jain²,

Sahu³

et al. 2016

J. Gastrointest. Oncol.

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“…The oldest patient is a 90-year-old male with intermediate-risk gastric GIST and he was relapse-free at last follow-up, six months after surgery. Although in most published documents there is no clear sex predilection [ 3 , 25 , 26 ], some studies revealed that there was a slight male predominance [ 27 – 29 ]. Our data agree with the latter.…”

Section: Discussionmentioning

confidence: 99%

Gastrointestinal stromal tumor: 15-years’ experience in a single center

Wang

Zhang

et al. 2014

BMC Surg

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BackgroundGastrointestinal stromal tumor (GIST) is known for its wide variability in biological behaviors and it is difficult to predict its malignant potential. The aim of this study is to explore the characteristics and prognostic factors of GIST.MethodsClinical and pathological data of 497 GIST patients in our center between 1997 and 2012 were reviewed.ResultsPatients were categorized into very low-, low-, intermediate- and high-risk groups according to modified National Institutes of Health (NIH) consensus classification system. Among the 401 patients untreated with imatinib mesylate (IM), 5-year overall survival (OS) in very low-, low-, intermediate- and high-risk groups was 100%, 100%, 89.6% and 65.9%; and 5-year relapse-free survival (RFS) was 100%, 98.1%, 90.9% and 44.5%, respectively. Univariate analysis revealed that sex, tumor size, mitotic rate, risk grade, CD34 expression, and adjacent involvement were predictors of OS or RFS. COX hazard proportional model (Forward LR) showed that large tumor size, high mitotic rate, and high risk grade were independent risk factors to OS, whereas high mitotic rate, high risk grade and adjacent organ involvement were independent risk factors to RFS. The intermediate-high risk patients who received IM adjuvant therapy (n = 87) had better 5-year OS and RFS than those who did not (n = 188) (94.9% vs. 72.1; 82.3% vs. 56.3%, respectively). Similarly, advanced GIST patients underwent IM therapy (n = 45) had better 3-year OS and 1-year progression-free survival (PFS) than those who didn’t (n = 42) (75.6% vs. 6.8%; 87.6% vs. 12.4%, respectively).ConclusionsVery low- and low-risk GISTs can be treated with surgery alone. Large tumor size, high mitotic rate, high risk grade, and adjacent organ involvement contribute to the poor outcome. IM therapy significantly improves the survival of intermediate-high risk or advanced GIST patients.Electronic supplementary materialThe online version of this article (doi:10.1186/1471-2482-14-93) contains supplementary material, which is available to authorized users.

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“…GISTs are considered to originate from the interstitial cells of Cajal , the pacemaker cells that regulate peristalsis in the digestive tract (Parkin and Chugh, 2011). Worldwide, GISTs occur at an incidence of around 11 to 19.6 per million (Bulbul Dogusoy, 2012; Chan et al, 2006; Goettsch et al, 2005; Nilsson et al, 2005; Vukobrat-Bijedic et al, 2012), equating to 3,300 to 6,000 new cases reported annually in the United States.…”

Section: Introductionmentioning

confidence: 99%

Carney triad, SDH-deficient tumors, and Sdhb+/− mice share abnormal mitochondria

Szarek¹,

Ball²,

Impériale³

et al. 2015

Endocrine-Related Cancer

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Carney Triad(CTr) describes the association of paragangliomas(PGL), pulmonary chondromas, and gastrointestinal(GI) stromal tumors(GISTs) with a variety of other lesions including pheochromocytomas and adrenocortical tumors. The gene(s) causing CTr remain(s) unknown. PGL and GISTs may be caused by loss-of-function mutations in succinate dehydrogenase (SDH) (a condition known as Carney-Stratakis syndrome (CSS)). Mitochondrial structure and function are abnormal in tissues carrying SDH defects but they have not been studied in CTr. For this study, we examined mitochondrial structure in human tumors and GI tissue(GIT) of mice with SDH deficiency. Tissues from 16 CTr tumors (n=12), and those with isolated GIST(n=1), with CSS caused by SDHC(n=1), SDHD(n=2) mutations were studied by electron microscopy (EM). GIT from mice with a heterozygous deletion in Sdhb (Sdhb+/−, n=4) were also studied by EM. CTr patients presented with mostly epithelioid GISTs that were characterized by plump cells containing a centrally located round nucleus and prominent nucleoli; these changes were almost identical to those seen in the GIST of patients with SDH. In tumor cells from patients, regardless of diagnosis or tumor type, cytoplasm contained increased mitochondria with “hypoxic” phenotype: mitochondria were devoid of cristae, exhibited structural abnormalities and of variable size. Occasionally, mitochondria were small/round, rarely thin, elongated with tubular cristae. Many mitochondria exhibited amorphous fluffy material with membranous whorls or cystic structures. Similar mitochondrial hypoxic phenotype was seen in Sdhb+/− mice. We conclude tissues from SDH-deficient tumors and mouse GIT and from CTr tumors shared identical abnormalities in mitochondrial structure and other features. Thus, the still elusive CTr defect(s) is(are) likely to affect mitochondrial function just like germline SDH-deficiency.

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Gastrointestinal stromal tumors: A multicenter study of 1160 Turkish cases

Cited by 30 publications

References 35 publications

Neoadjuvant imatinib: longer the better, need to modify risk stratification for adjuvant imatinib

Neoadjuvant imatinib: longer the better, need to modify risk stratification for adjuvant imatinib

Gastrointestinal stromal tumor: 15-years’ experience in a single center

Carney triad, SDH-deficient tumors, and Sdhb+/− mice share abnormal mitochondria

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