|
1
|
Kim Y, Lee SH. Pathologic diagnosis and molecular features of gastrointestinal stromal tumors: a mini-review. Front Oncol 2024; 14:1487467. [PMID: 39629000 PMCID: PMC11611718 DOI: 10.3389/fonc.2024.1487467] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/28/2024] [Accepted: 10/29/2024] [Indexed: 12/06/2024] Open
Abstract
Gastrointestinal stromal tumors (GISTs) arise from the interstitial cells of Cajal, predominantly affecting the stomach and small intestine, with rare occurrences in the duodenum, rectum, and extraintestinal sites. Histologically, GISTs can present as spindle cells, epithelioid cells, or mixed morphologies, with immunohistochemical staining revealing expression of KIT (CD117) and discovered on GIST 1 (DOG1). Approximately 80% of GISTs harbor activating mutations in KIT or platelet derived growth factor receptor α (PDGFRA), which influence their clinical behavior and treatment response. SDH-deficient GISTs, associated with syndromes such as Carney triad and Carney-Stratakis syndrome, represent a distinct subgroup with unique characteristics and management challenges. The standard treatment includes surgery and imatinib for metastatic cases; however, resistance to tyrosine kinase inhibitors remains a significant hurdle, especially in pediatric and wildtype GISTs. This highlights the need for advanced therapeutic strategies and emphasizes the importance of molecular profiling in guiding treatment decisions and improving outcomes for GIST patients.
Collapse
Affiliation(s)
| | - Sung Hak Lee
- Department of Hospital Pathology, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
| |
Collapse
|
|
2
|
Augustine A, Issac R, Lakhani A, Kanamathareddy HV, John R, Simon B, Masih D, Eapen A, Chandramohan A. Anal and Perianal Masses: The Common, the Uncommon, and the Rare. Indian J Radiol Imaging 2024; 34:688-701. [PMID: 39318564 PMCID: PMC11419757 DOI: 10.1055/s-0044-1781459] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 09/26/2024] Open
Abstract
A variety of tumors involve the anal canal because the anal canal forms the transition between the digestive system and the skin, and this anatomical region is made of a variety of different cells and tissues. Magnetic resonance imaging (MRI) is the modality of choice for diagnosis and local staging of the anal canal and perianal neoplasms. In this pictorial review, we demonstrate the MRI anatomy of the anal canal and perianal region and display the imaging spectrum of tumors in the region along with an overview of its management. Imaging appearances of many tumorlike lesions that can cause diagnostic dilemmas are also demonstrated with pointers to differentiate between them.
Collapse
Affiliation(s)
- Antony Augustine
- Department of Radiology, Christian Medical College, Vellore, Tamil Nadu, India
| | - Rijo Issac
- Department of General Pathology, Christian Medical College, Vellore, Tamil Nadu, India
| | - Aisha Lakhani
- Department of Radiology, Christian Medical College, Vellore, Tamil Nadu, India
| | | | - Reetu John
- Department of Radiology, Christian Medical College, Vellore, Tamil Nadu, India
| | - Betty Simon
- Department of Radiology, Christian Medical College, Vellore, Tamil Nadu, India
| | - Dipti Masih
- Department of General Pathology, Christian Medical College, Vellore, Tamil Nadu, India
| | - Anu Eapen
- Department of Radiology, Christian Medical College, Vellore, Tamil Nadu, India
| | | |
Collapse
|
|
3
|
Huang W, Yuan W, Ren L, Liang H, Du X, Sun X, Fang Y, Gao X, Fu M, Sun Y, Shen K, Hou Y. Clinicopathological and therapeutic analysis of PDGFRA mutated gastrointestinal stromal tumor. Pathol Res Pract 2022; 239:154138. [PMID: 36183438 DOI: 10.1016/j.prp.2022.154138] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/07/2022] [Revised: 09/04/2022] [Accepted: 09/16/2022] [Indexed: 11/19/2022]
Abstract
BACKGROUND Platelet-Derived Growth Factor Receptor Alpha (PDGFRA) mutation has causes a rare subgroup of gastrointestinal stromal tumor (GIST) and not too much attention has been paid on it until the appearance of Avapritinib. This study aims to explore the clinicopathological features, therapy and prognosis of PDGFRA-mutant GIST for better understanding and clinical practice. METHOD 119 PDGFRA-mutant GIST patients were retrospectively collected from 2038 patients who underwent genetic testing. Kaplan-Meier method was used. RESULTS The incidence rate of PDGFRA-mutant GIST in our center was 5.8 %, with 79 males, 40 females, and a median age of 57 (25⁃80) years old. All the tumors were in the stomach, among which 60 were epithelioid type, 25 were spindle type and 34 were mixed type. There were 13 cases of exon 12 mutation and 106 cases of exon 18 mutation including 83 cases of D842V mutation (69.7 %). During a median follow⁃up of 49.6 (range, 1⁃154) months, progression could be observed in 12 patients with gene mutation at the codon 842 of exon 18, another case was V561D mutation in exon 12. The 5-year diseases⁃free survival (DFS) was 90.1 %, which was associated with the loss of CD34 expression (P＜0.001). Patients in D842V group showed a marginal worse prognosis than those in non-D842V group (P = 0.163). According to the NIH criteria, high risk group showed a poorer prognosis than non-high risk group (P = 0.003), however, there were no significant differences among the three non-high risk groups (P = 0.495, P = 0.652). Among 13 advanced patients, 5 cases (treated with Avapritinib) achieved partial remission. CONCLUSION PDGFRA-mutant GIST mostly derived from stomach, with a relative indolent behavior. D842V mutation and lose of CD34 expression were adverse prognostic factors. Avapritinib can effectively control advanced patients in a certain period of time.
Collapse
Affiliation(s)
- Wen Huang
- Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Wei Yuan
- Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Lei Ren
- Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Huaiyu Liang
- Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Xiangyang Du
- Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Xiangfei Sun
- Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Yong Fang
- Department of General Surgery, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Xiaodong Gao
- Department of General Surgery, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Min Fu
- Department of General Surgery, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Yihong Sun
- Department of General Surgery, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Kuntang Shen
- Department of General Surgery, Zhongshan Hospital, Fudan University, Shanghai, China.
| | - Yingyong Hou
- Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai, China.
| |
Collapse
|
|
4
|
Grazzini G, Guerri S, Cozzi D, Danti G, Gasperoni S, Pradella S, Miele V. Gastrointestinal stromal tumors: relationship between preoperative CT features and pathologic risk stratification. TUMORI JOURNAL 2021; 107:556-563. [PMID: 33620027 DOI: 10.1177/0300891621996447] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/31/2022]
Abstract
OBJECTIVE To investigate a relationship between contrast-enhanced computed tomography (CECT) features of gastrointestinal stromal tumors (GISTs) and risk of relapse according to Miettinen stratified risk classifications. METHODS After ethical committee approval, a retrospective analysis was conducted on the preoperative CECT of patients with pathologically proven GIST undergoing surgery between June 2009 and December 2019. Chi-square analysis was used to evaluate the correlation between Miettinen stratified risk categories and the following imaging features: tumor size and location, growth pattern, margins, type and degree of contrast enhancement, presence of calcifications, necrosis, signs of ulceration/fistulation, internal hemorrhagic foci, enlarged feeding or draining vessels (EFDV), ascites, peritoneal implants, lymphadenopathy, or metastasis. RESULTS A total of 54 patients (mean age 65 ± 11, 29 men) were included in the study with a total of 56 GISTs. Necrosis, ulceration/fistulation, hemorrhage, margins, enlarged vessels, type of contrast enhancement, and metastasis turned out to be associated with Miettinen risk categories (p < 0.005). Logistic regression analysis identified the presence of necrosis and EFDV as predictors of pathologic risk of relapse (overall accuracy of 89.3%). CONCLUSION Preoperative CECT may be helpful in predicting pathologic risk categories of GISTs, as determined by the Miettinen classification system.
Collapse
Affiliation(s)
- Giulia Grazzini
- Radiodiagnostica di Emergenza Urgenza, Azienda Ospedaliero Universitaria Careggi, Firenze, Italy
| | - Sara Guerri
- Radiodiagnostica di Emergenza Urgenza, Azienda Ospedaliero Universitaria Careggi, Firenze, Italy
| | - Diletta Cozzi
- Radiodiagnostica di Emergenza Urgenza, Azienda Ospedaliero Universitaria Careggi, Firenze, Italy
| | - Ginevra Danti
- Radiodiagnostica di Emergenza Urgenza, Azienda Ospedaliero Universitaria Careggi, Firenze, Italy
| | - Silvia Gasperoni
- SOD Oncologia Traslazionale Dipartimento Oncologico AOUC, Azienda Ospedaliero Universitaria Careggi, Firenze, Italy
| | - Silvia Pradella
- Radiodiagnostica di Emergenza Urgenza, Azienda Ospedaliero Universitaria Careggi, Firenze, Italy
| | - Vittorio Miele
- Radiodiagnostica di Emergenza Urgenza, Azienda Ospedaliero Universitaria Careggi, Firenze, Italy
| |
Collapse
|
|
5
|
Song LJ, Ge HJ, Shi XQ, Shen WW. Prognostic and predictive values of the KIT11-mutated grading system in patients with gastrointestinal stromal tumors: a retrospective study. Hum Pathol 2021; 110:31-42. [PMID: 33476644 DOI: 10.1016/j.humpath.2021.01.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/13/2020] [Revised: 01/05/2021] [Accepted: 01/11/2021] [Indexed: 01/10/2023]
Abstract
The KIT11 mutation is the most frequent mutation pattern in gastrointestinal stromal tumors (GISTs). However, few studies have investigated the correlation between the KIT11-mutated grading system and imatinib mesylate (IM) sensitivity (the first choice for adjuvant treatment of GISTs). Here, we elucidated the clinical value of the KIT11-mutated grading system for prognostic prediction in patients with GISTs treated with IM. A total of 106 patients with GIST were treated with IM (8: intermediate-risk, 98: high-risk; 10: KIT9-mutated, 86: KIT11-mutated, 5: wild-type, and 5: other mutations). KIT11-mutated patients were divided into 3 grades based on the KIT11-mutated site and type. Clinical backgrounds and prognostic outcomes were retrospectively compared between the 3 groups. Of 86 KIT11-mutated patients treated with IM, 32 (37.21%) had grade 1 tumors, 37 (43.02%) had grade 2 tumors, and 17 (19.77%) had grade 3 tumors. The 5-year disease-free survival (DFS) was significantly worse in patients with grade 3 KIT11-mutated GISTs (41.96%, p = 0.001) than in those with grade 1 (93%) and grade 2 (70.64%) cases. The multivariable analysis suggested that the KIT11-mutated grading system was an independent risk factor for DFS in patients treated with IM (hazard risk, 2.512; 95% confidence interval, 1.370-4.607; p = 0.003). In conclusion, the KIT11-mutated grading system provides good prognostic stratification for DFS in patients treated with IM. Grade 1 tumors predict a favorable response to IM.
Collapse
Affiliation(s)
- Ling-Jun Song
- Pathology Center, Shanghai General Hospital/Faculty of Basic Medicine, Shanghai Jiao Tong University School of Medicine, Shanghai, 200080 China.
| | - Hui-Juan Ge
- Department of Pathology, Fudan University Shanghai Cancer Center; Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, 200032 PR China
| | - Xiao-Qin Shi
- Pathology Center, Shanghai General Hospital/Faculty of Basic Medicine, Shanghai Jiao Tong University School of Medicine, Shanghai, 200080 China
| | - Wei-Wei Shen
- Pathology Center, Shanghai General Hospital/Faculty of Basic Medicine, Shanghai Jiao Tong University School of Medicine, Shanghai, 200080 China; Key Laboratory of Cell Differentiation and Apoptosis of Chinese Ministry of Education, Institutes of Medical Sciences, Shanghai Jiao Tong University School of Medicine, Shanghai, 200025 China
| |
Collapse
|
|
6
|
Surgical treatment of primary gastrointestinal stromal tumors (GISTs): Management and prognostic role of R1 resections. Am J Surg 2019; 220:359-364. [PMID: 31862107 DOI: 10.1016/j.amjsurg.2019.12.006] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/18/2019] [Revised: 11/24/2019] [Accepted: 12/03/2019] [Indexed: 12/15/2022]
Abstract
BACKGROUND Surgery represents the best treatment for primary gastrointestinal stromal tumors (GISTs). The aim of this study is to analyse outcomes of surgical management in order to evaluate the influence of microscopically R1 margins on survival and recurrence in patients affected by GISTs. METHODS The study reviewed retrospective data from 74 patients surgically treated for primary GISTs without metastasis at diagnosis. Clinical and pathological findings, surgical procedures, information about follow up and outcomes were analyzed. RESULTS Recurrence rate was low and no patients died in the R1 group during the follow up period. The difference in recurrence free survival for patients undergoing an R0 (n = 54) versus an R1 (n = 20) resections was not statistically significant (76% versus 85% at 3 years, logrank test p-value = 0,14; 63% versus 86% at 5 years, logrank test p-value = 0,48) CONCLUSIONS: Microscopically positive margin has no influence on overall and relapse-free survival in GIST patients. Thus, when R0 surgery implies major functional sequelae, it may be decided to accept possible R1 margins, especially for low risk tumors.
Collapse
|
|
7
|
Detection of ANO1 mRNA in PBMCs is a promising method for GISTs diagnosis. Sci Rep 2019; 9:9525. [PMID: 31266974 PMCID: PMC6606646 DOI: 10.1038/s41598-019-45941-2] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/12/2018] [Accepted: 06/17/2019] [Indexed: 01/14/2023] Open
Abstract
ANO1 is a calcium-activated chloride channel protein that has been used to diagnose GISTs after tissue biopsy. Recently, ANO1 mRNA amplification in the blood has received considerable attention as a useful method for the diagnosis of GISTs. The aim of this study was to evaluate the diagnostic ability of ANO1 mRNA in distinguishing GIST patients from healthy subjects. We constructed a logistic regression model for examining the diagnostic ability of ANO1 mRNA in comparison with conventional tumor markers, including CEA, CA199, and CA724. Our results showed that ANO1 mRNA was significantly amplified in PBMCs, the average expression level and range of ANO1 mRNA in the blood were increased along with the expression of ANO1 in the tissues, and the extent of amplification of ANO1 was associated with tumor size. In addition, ROC curve analysis showed that ANO1 mRNA in the blood had the highest specificity when compared with conventional tumor markers. Moreover, a combined analysis with ANO1 mRNA and conventional tumor markers had the highest sensitivity in diagnosing GISTs. Our study indicated that detection of ANO1 mRNA in PBMCs is a promising method for diagnosis of GISTs in vitro.
Collapse
|
|
8
|
Zhao B, Zhang J, Mei D, Zhang J, Luo R, Xu H, Huang B. The assessment of different risk classification systems for gastrointestinal stromal tumors (GISTs): the analytic results from the SEER database. Scand J Gastroenterol 2019; 53:1319-1327. [PMID: 30353759 DOI: 10.1080/00365521.2018.1515319] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
BACKGROUND Although various risk classification systems for GISTs have been proposed, the optimum one remains uncertain. In the present study, we compared the prognostic stratification of different risk classification systems for GIST patients. METHODS We reviewed those patients who were pathologically diagnosed with GISTs in the SEER database between 2009 and 2014. All patients were classified into different risk groups according to the NIH criteria, AFIP criteria and AJCC staging system, respectively. The prognostic differences between different risk groups were compared and clinicopathologic features were analyzed. RESULTS The prognosis of small intestinal GISTs was not significantly different from that of gastric GISTs. For gastric GIST patients, there was no significant prognostic difference between very low risk and low risk group according to the NIH and AFIP criteria. However, the prognostic stratification for two groups could be improved by the AJCC staging system. For small intestinal GIST patients, the prognostic difference between low risk and intermediate risk group was not stratified properly by the NIH and AFIP criteria. However, the prognostic difference between two groups could reach statistical significance according to the AJCC staging system. Unlike gastric GISTs, tumor size was not identified as an independent factor influencing the prognosis of small intestinal GISTs. CONCLUSIONS The AJCC staging system could provide a better prognostic stratification for GIST patients compared with the NIH and AFIP criteria, regardless of gastric or small intestinal tumor. However, primary tumor location and tumor size may be reconsidered and revised in the risk classification system.
Collapse
Affiliation(s)
- Bochao Zhao
- a Department of Surgical Oncology , First Affiliated Hospital of China Medical University , Shenyang , P.R. China
| | - Jingting Zhang
- a Department of Surgical Oncology , First Affiliated Hospital of China Medical University , Shenyang , P.R. China
| | - Di Mei
- a Department of Surgical Oncology , First Affiliated Hospital of China Medical University , Shenyang , P.R. China
| | - Jiale Zhang
- a Department of Surgical Oncology , First Affiliated Hospital of China Medical University , Shenyang , P.R. China
| | - Rui Luo
- a Department of Surgical Oncology , First Affiliated Hospital of China Medical University , Shenyang , P.R. China
| | - Huimian Xu
- a Department of Surgical Oncology , First Affiliated Hospital of China Medical University , Shenyang , P.R. China
| | - Baojun Huang
- a Department of Surgical Oncology , First Affiliated Hospital of China Medical University , Shenyang , P.R. China
| |
Collapse
|
|
9
|
Abdalla S, Oke T. Laparoscopic resection of a gastrointestinal stromal tumour in a Meckel's diverticulum. J Minim Access Surg 2019; 15:339-341. [PMID: 30618430 PMCID: PMC6839353 DOI: 10.4103/jmas.jmas_239_18] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
A Meckel's diverticulum (MD) is a congenital abnormality of the gastrointestinal tract which is estimated to be present in 2% of the population. Gastrointestinal stromal tumours (GISTs) are rare, soft-tissue tumours which represent 0.1%-3% of all gastrointestinal tumours. The association of an MD and a GIST is extremely unusual since fewer than 3% of MD harbour primary neoplasms and most of these neoplasms are carcinoid tumours. While MDs may remain asymptomatic throughout life, a small proportion may be complicated by occult gastrointestinal bleeding, inflammation, perforation and small bowel obstruction. A tumour in an MD may be asymptomatic or can cause vague abdominal pain and small bowel obstruction if it is larger in dimension. The authors present a rare case of a 5.5 cm GIST in an MD that was completely resected through a laparoscopic approach.
Collapse
Affiliation(s)
- Sala Abdalla
- Department of General Surgery, Queen Elizabeth Hospital, Lewisham and Greenwich NHS Trust, London, England
| | - Tayo Oke
- Department of General Surgery, Queen Elizabeth Hospital, Lewisham and Greenwich NHS Trust, London, England
| |
Collapse
|
|
10
|
Song S, Ren W, Wang Y, Zhang S, Zhang S, Liu F, Cai Q, Xu G, Zou X, Wang L. Tumor rupture of gastric gastrointestinal stromal tumors during endoscopic resection: a risk factor for peritoneal metastasis? Endosc Int Open 2018; 6:E950-E956. [PMID: 30083583 PMCID: PMC6070373 DOI: 10.1055/a-0619-4803] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/06/2017] [Accepted: 03/14/2018] [Indexed: 12/23/2022] Open
Abstract
BACKGROUND AND STUDY AIMS Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors in the gastrointestinal tract. Up to the present time, complete surgical excision has been the standard treatment for primary GISTs greater than 2 cm. It is well known that tumor rupture during surgery is an independent risk factor for peritoneal metastasis; however, it is not known whether the risk of peritoneal metastasis increases in cases where the tumor is ruptured during endoscopic resection. PATIENTS AND METHODS A total of 195 patients treated for GIST between January 2014 and December 2016 in our hospital were enrolled in this study. They were divided into two groups according to whether the tumor was ruptured during endoscopic resection. The rate of peritoneal metastasis in patients in the two groups who also suffered perforation was investigated from the follow-up results. RESULTS Approximately 55.4 % of all patients were female and the average age of the study group was 59.0 ± 10.3 years. Of the 195 patients, the tumors in 27 were ruptured and the remaining 168 patients underwent en bloc resection. There was no statistically significant difference in gender or age between the two groups. The median tumor size (maximum diameter) in all patients was 1.5 cm (0.3 - 5.0 cm): 2.5 cm (0.8 - 5.0 cm) and 1.4 cm (0.3 - 4.0 cm) in the tumor rupture group and en bloc resection group, respectively ( P < 0.001). Most of the tumors were located in the gastric fundus. At a median follow-up of 18.7 ± 10.2 months, neither tumor recurrence (liver metastasis, peritoneal metastasis, local recurrence) nor mortality related to GISTs were detected. CONCLUSIONS Tumor rupture during endoscopic resection of gastric GISTs may not be a risk factor for peritoneal metastasis.
Collapse
Affiliation(s)
- Shiyi Song
- Department of Gastroenterology, The Affiliated Drum Tower Hospital of Nanjing University, Medical School, Nanjing, China
| | - Wei Ren
- Department of Geriatrics, The Affiliated Drum Tower Hospital of Nanjing University, Medical School, Nanjing, China
| | - Yi Wang
- Department of Gastroenterology, The Affiliated Drum Tower Hospital of Nanjing University, Medical School, Nanjing, China
| | - Shu Zhang
- Department of Gastroenterology, The Affiliated Drum Tower Hospital of Nanjing University, Medical School, Nanjing, China
| | - Song Zhang
- Department of Gastroenterology, The Affiliated Drum Tower Hospital of Nanjing University, Medical School, Nanjing, China
| | - Fei Liu
- Department of Gastroenterology, The Affiliated Drum Tower Hospital of Nanjing University, Medical School, Nanjing, China
| | - Qiang Cai
- Digestive Diseases, Emory University, Atlanta, GA, USA
| | - Guifang Xu
- Department of Gastroenterology, The Affiliated Drum Tower Hospital of Nanjing University, Medical School, Nanjing, China
| | - Xiaoping Zou
- Department of Gastroenterology, The Affiliated Drum Tower Hospital of Nanjing University, Medical School, Nanjing, China
| | - Lei Wang
- Department of Gastroenterology, The Affiliated Drum Tower Hospital of Nanjing University, Medical School, Nanjing, China,Corresponding author Lei Wang Department of GastroenterologyThe Affiliated Drum Tower Hospital of Nanjing University, Medical SchoolNo. 321Zhongshan RoadNanjingJiangsu 210008China+86-138-51579216
| |
Collapse
|
|
11
|
Khoo CY, Chai X, Quek R, Teo MCC, Goh BKP. Systematic review of current prognostication systems for primary gastrointestinal stromal tumors. EUROPEAN JOURNAL OF SURGICAL ONCOLOGY 2018; 44:388-394. [PMID: 29422251 DOI: 10.1016/j.ejso.2017.12.006] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/18/2017] [Revised: 12/11/2017] [Accepted: 12/20/2017] [Indexed: 02/07/2023]
Abstract
BACKGROUND The advent of tyrosine kinase inhibitors as adjuvant therapy has revolutionized the management of GIST and emphasized the need for accurate prognostication systems. Numerous prognostication systems have been proposed for GIST but at present it remains unknown which system is superior. The present systematic review aims to summarize current prognostication systems for primary treatment-naive GIST. METHODS A literature review of the Pubmed and Embase databases was performed to identify all published articles in English, from the 1st January 2002 to 28th Feb 2017, reporting on clinical prognostication systems of GIST. RESULTS Twenty-three articles on GIST prognostication systems were included. These systems were classified as categorical systems, which stratify patients into risk groups, or continuous systems, which provide an individualized form of risk assessment. There were 16 categorical systems in total. There were 4 modifications of the National Institute of Health (NIH) system, 2 modifications of Armed Forces Institute of Pathology (AFIP) criteria and 3 modifications of Joensuu (modified NIH) criteria. Of the 7 continuous systems, there were 3 prognostic nomograms, 3 mathematical models and 1 prognostic heat/contour maps. Tumor size, location and mitotic count remain the main variables used in these systems. CONCLUSION Numerous prognostication systems have been proposed for the risk stratification of GISTs. The most widely used systems today are the NIH, Joensuu modified NIH, AFIP and the Memorial Sloan Kettering Cancer Center nomogram. More validation and comparison studies are required to determine the optimal prognostication system for GIST.
Collapse
Affiliation(s)
- Chun Yuet Khoo
- Department of Hepatopancreatobiliary and Transplant Surgery, Singapore General Hospital, Singapore; Yong Loo Lin School of Medicine, National University of Singapore, Singapore
| | - Xun Chai
- Department of Hepatopancreatobiliary and Transplant Surgery, Singapore General Hospital, Singapore; Yong Loo Lin School of Medicine, National University of Singapore, Singapore
| | - Richard Quek
- Division of Medical Oncology, National Cancer Center, Singapore
| | - Melissa C C Teo
- Division of Surgical Oncology, National Cancer Center, Singapore; Duke-NUS Medical School, Singapore
| | - Brian K P Goh
- Department of Hepatopancreatobiliary and Transplant Surgery, Singapore General Hospital, Singapore; Duke-NUS Medical School, Singapore.
| |
Collapse
|
|
12
|
Laparoscopic Versus Open Resection for Gastric Gastrointestinal Stromal Tumors (GISTs): A Size-Location-Matched Case-Control Study. World J Surg 2018; 41:2345-2352. [PMID: 28349318 DOI: 10.1007/s00268-017-4005-8] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Abstract
BACKGROUND Laparoscopic resection for gastric gastrointestinal stromal tumors (GISTs) is technically feasible, but the long-term effect remains uncertain. This study aims to compare the long-term oncologic outcomes of laparoscopic versus open resection of GISTs by larger cases based on tumor size-location-matched study. METHODS Between 2006 and 2015, 63 consecutive patients with a primary gastric GIST undergoing laparoscopic resection were enrolled in and matched (1:1) to patients undergoing open resection by tumor size and location. Clinical and pathologic parameters and surgical outcomes associated with each surgical type were collected and compared. RESULTS The operation time, intraoperative blood loss, return of bowel function and oral intake, nasogastric tube retention time and postoperative stay were all shorter/faster in laparoscopic group than those in open group (P < 0.001). Postoperative complications were comparable except for the higher incidence of abdominal/incision pain in open group (9.52 vs 27%, P = 0.01). There was no statistical difference in recurrence rate (9.52 vs 15.87%, P = 0.29) and long-term recurrence-free survival between the two groups (P = 0.39). CONCLUSIONS The long-term oncologic outcome of laparoscopic resection of primary gastric GISTs is comparable to that of open procedure, but laparoscopic procedure has the advantage of minimal invasion and is superior in postoperative recovery.
Collapse
|
|
13
|
Rosa F, Alfieri S, Tortorelli AP, Di Miceli D, Papa V, Doglietto GB. Gastrointestinal Stromal Tumors: Prognostic Factors and Therapeutic Implications. TUMORI JOURNAL 2018; 98:351-6. [DOI: 10.1177/030089161209800312] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/13/2023]
Abstract
Background Gastrointestinal stromal tumors (GISTs) are the most frequent mesenchymal tumors of the digestive tract. They have recently been recognized as a separate nosological entity and the literature on these stromal tumors has rapidly expanded. Materials and methods The surgical records of 50 patients with primary GISTs treated at the Digestive Surgery Department of the Catholic University of Rome from January 1993 to December 2010 were reviewed and the prognostic factors were analyzed. Results Surgery was performed in all patients with curative intent. The median age at presentation was 66.5 years (range, 28–81). Adjuvant therapy was administered in 26 (52%) cases. Median follow-up was 71 months (range, 5–208). There was an 8% recurrence rate. The actuarial 5-year overall and disease-free survival rates were 66.3% and 57.2%, respectively. High mitotic rate (P <0.001), tumor size greater than 10 cm (P = 0.007) and tumor rupture (P = 0.05) were the only prognostically significant negative factors for overall survival in multivariate analysis. Conclusions The present study confirmed the important role of aggressive surgical management of GISTs to offer these patients the most appropriate treatment for long-term survival.
Collapse
Affiliation(s)
- Fausto Rosa
- Department of Digestive Surgery, Agostino Gemelli
Hospital, Catholic University of Rome, Rome, Italy
| | - Sergio Alfieri
- Department of Digestive Surgery, Agostino Gemelli
Hospital, Catholic University of Rome, Rome, Italy
| | - Antonio Pio Tortorelli
- Department of Digestive Surgery, Agostino Gemelli
Hospital, Catholic University of Rome, Rome, Italy
| | - Dario Di Miceli
- Department of Digestive Surgery, Agostino Gemelli
Hospital, Catholic University of Rome, Rome, Italy
| | - Valerio Papa
- Department of Digestive Surgery, Agostino Gemelli
Hospital, Catholic University of Rome, Rome, Italy
| | | |
Collapse
|
|
14
|
Park CH, Kim GH, Lee BE, Song GA, Park DY, Choi KU, Kim DH, Jeon TY. Two staging systems for gastrointestinal stromal tumors in the stomach: which is better? BMC Gastroenterol 2017; 17:141. [PMID: 29207963 PMCID: PMC5718111 DOI: 10.1186/s12876-017-0705-7] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/19/2016] [Accepted: 11/24/2017] [Indexed: 12/23/2022] Open
Abstract
BACKGROUND The prognosis of a gastrointestinal stromal tumor (GIST) is influenced by its anatomic site; however, few studies on the prognosis of gastric GISTs have been reported. The aims of this study were to evaluate long-term prognoses of patients who underwent surgical resection for gastric GISTs and to compare the clinical efficacy of two staging systems: the National Institutes of Health (NIH) consensus criteria and the 7th Union for International Cancer Control/American Joint Committee on Cancer (UICC/AJCC) tumor-node-metastasis (TNM) staging system. METHODS We conducted a retrospective observational study of 145 patients who underwent surgical resection for gastric GISTs between February 2001 and June 2012 at Pusan National University Hospital (Busan, Korea). Recurrence and 5-year recurrence-free survival (RFS) rates were analyzed. RESULTS During a median follow-up period of 44 months (range, 6-144 months), 11 recurrent lesions were detected in 9 patients (6.4%). On multivariate analysis, tumor size (>5 cm), mitotic count (>5/50 high-power fields), and epithelioid and mixed pathological type were significantly associated with recurrence. The overall 5-year RFS rate was 93.4%. Although no statistically significant differences were detected (C-statistic difference P = 0.886), all metrics showed lower values for the UICC/AJCC TNM staging system than for the NIH consensus criteria, suggesting that the UICC/AJCC TNM staging system may be a better model. CONCLUSIONS The 5-year RFS rate in patients who underwent curative resection for gastric GISTs was excellent. The UICC/AJCC TNM staging system may be more useful than the NIH consensus criteria for risk categorization of patients with gastric GISTs.
Collapse
Affiliation(s)
- Chul Hong Park
- Department of Internal Medicine, Pusan National University School of Medicine, and Biomedical Research Institute Pusan National University Hospital, 179, Gudeok-ro, Seo-Gu, Busan, 49241 South Korea
| | - Gwang Ha Kim
- Department of Internal Medicine, Pusan National University School of Medicine, and Biomedical Research Institute Pusan National University Hospital, 179, Gudeok-ro, Seo-Gu, Busan, 49241 South Korea
| | - Bong Eun Lee
- Department of Internal Medicine, Pusan National University School of Medicine, and Biomedical Research Institute Pusan National University Hospital, 179, Gudeok-ro, Seo-Gu, Busan, 49241 South Korea
| | - Geun Am Song
- Department of Internal Medicine, Pusan National University School of Medicine, and Biomedical Research Institute Pusan National University Hospital, 179, Gudeok-ro, Seo-Gu, Busan, 49241 South Korea
| | - Do Youn Park
- Department of Pathology, Pusan National University School of Medicine, Busan, South Korea
| | - Kyung Un Choi
- Department of Pathology, Pusan National University School of Medicine, Busan, South Korea
| | - Dae Hwan Kim
- Department of Surgery, Pusan National University School of Medicine, Busan, South Korea
| | - Tae Yong Jeon
- Department of Surgery, Pusan National University School of Medicine, Busan, South Korea
| |
Collapse
|
|
15
|
Kalliakmanis V, Koutsouvas K, Dimakopoulou V, Siasos N. Exophytic gastrointestinal stromal tumor of the stomach. Report of two cases. ACTA ACUST UNITED AC 2017. [DOI: 10.1007/s13126-017-0389-z] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
|
|
16
|
Prasertcharoensuk S, Thanapongpornthana P, Bhudhisawasdi V, Pugkhem A, Jenwitheesuk K, Sookprasert A, Pairojkul C. Prognostic Factors for Large Symptomatic Gists: a Pragmatic Study of Experiences From a University Hospital Over 10 Years. Asian Pac J Cancer Prev 2017; 18:655-658. [PMID: 28440971 PMCID: PMC5464480 DOI: 10.22034/apjcp.2017.18.3.655] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022] Open
Abstract
Background: Gastrointestinal stromal tumors (GISTs), which are mesenchymal neoplasms in the gastrointestinal (GI) tract account for 0.2% of all GI tumors. Several factors have been reported (mostly from studies conducted in Western countries) to be associated with survival in GISTs cases such as tumor site, staging, and tumor size. We conducted a pragmatic study, looking at a 10-year period, aimed at understanding the prognostic factors related to GISTs in a university hospital. The study population consisted of patients with large symptomatic GISTs. Methods: This was a retrospective study conducted at the Department of Surgery in the Khon Kaen University Hospital (Thailand). All patients diagnosed with GISTs that were treated between 2006 and 2015 were consecutively enrolled. The diagnosis of GISTs was made by examining the pathological section and immunohistochemistry results. The outcome of this study was the rate of survival after surgical treatment. Prognostic factors were determined using Cox regression analysis. Results: There were 124 GISTs patients treated at the university hospital during the 10-year period of the study. The median age of all patients was 54 years (range 24-83 years). Of those, 119 (95.9%) were symptomatic. Rectosigmoid GISTs accounted for 20.2% of all tumors. The median tumor size was 8 cm. A total of 68 patients (54.8%) died. The median survival time for all patients was 7.18 years (1st -3rd quartile range 6.48-7.89). There were three significant factors associated with death including male gender, liver metastasis, and peritoneal metastasis. Conclusion: Male gender, liver metastasis, and peritoneal metastasis were prognostic factors for large symptomatic GISTs.
Collapse
|
|
17
|
Urgency surgical treatment for duodenal GISTs: analysis of aged patients and review of the literature. Aging Clin Exp Res 2017; 29:1-6. [PMID: 27844452 DOI: 10.1007/s40520-016-0641-3] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/31/2016] [Accepted: 10/12/2016] [Indexed: 12/17/2022]
Abstract
INTRODUCTION Gastrointestinal stromal tumours (GISTs) are the most common mesenchymal neoplasms (85%) of the gastrointestinal (GI) tract; duodenal GISTs constitute 3-5% of all GISTs and represent 10-30% of all malignant tumours of the duodenum. Rarely, patients present severe bowel obstruction, perforation or severe bleeding. The radical resection with complete removal of the tumour remains the main therapeutic approach. We performed a local resection in patients with suspected GIST admitted for emergency treatment for GI bleeding. CASES We present three cases of patients admitted for GI bleeding. The cause could be a GIST bleeding. In all cases, local resection was performed without a pancreaticoduodenectomy. Histological examination on surgical preparations showed that in two cases it was a GIST and in one case, it was a leiomyoma. DISCUSSION Surgery remains the treatment of choice in the case of a GIST primitive without evidence of metastases, even for patients who are hospitalized for a bleeding emergency. Wide resections are not needed; it is important to remove completely the disease. In the case of duodenal GIST, it is important to get negative margins near the head of the pancreas, and this could take a PD. According to our experience and to the literature review, we believe that if the duodenal papilla or the periampullary region is not interested, you must perform a local resection. This is also because non-malignant tumours may present as GISTs and in these cases it is not recommended to run a PD. CONCLUSION The treatment of choice for duodenal GISTs is complete surgical resection with negative resection margins. When the papilla or the periampullary region is involved we choose to perform pancreaticoduodenectomy; otherwise it is better to perform a local resection. In fact, local resection has lower morbidity and mortality, with a comparable outcome.
Collapse
|
|
18
|
Roy AC, Wattchow D, Astill D, Singh S, Pendlebury S, Gormly K, Segelov E. Uncommon Anal Neoplasms. Surg Oncol Clin N Am 2017; 26:143-161. [DOI: 10.1016/j.soc.2016.07.009] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/31/2023]
|
|
19
|
Schmieder M, Henne-Bruns D, Mayer B, Knippschild U, Rolke C, Schwab M, Kramer K. Comparison of Different Risk Classification Systems in 558 Patients with Gastrointestinal Stromal Tumors after R0-Resection. Front Pharmacol 2016; 7:504. [PMID: 28082898 PMCID: PMC5187374 DOI: 10.3389/fphar.2016.00504] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/19/2016] [Accepted: 12/07/2016] [Indexed: 12/12/2022] Open
Abstract
Background: Due to adjuvant treatment concepts for patients with R0-resected gastrointestinal stromal tumors (GIST), a reproducible and reliable risk classification system proved of utmost importance for optimal treatment of patients and prediction of prognosis. The aim of this study was to reevaluate the impact of five widely-applied and well-established GIST risk classification systems (i.e., scores by Fletcher, Miettinen, Huang, Joensuu, and TNM classification) on a series of 558 GIST patients with long-term follow-up after R0 resection. Methods: Tumor size, mitotic count and site were used in variable combination to predict high- and low risk patients by the use of the five risk classification models. For survival analyses disease-specific survival, disease-free survival and overall-survival were investigated. Patients with initial metastatic disease or incompletely resectable tumors were excluded. Results: All GIST classification models distinguished well between patients with high-risk and low-risk tumors and none of the five risk systems was superior to predict patient outcome. The models showed significant heterogeneity. There was no significant difference between the different risk-groups regarding overall-survival. Subdivision of GIST patients with very low- and low-risk appeared to be negligible. Conclusions: Currently applied GIST risk classification systems are comparable to predict high- or low-risk patients with initial non-metastatic and completely resected GIST. However, the heterogeneity of the high-risk group and the absence of differences in overall survival indicate the need for more precise tumor- and patient-related criteria for better stratification of GIST and identification of patients who would benefit best from adjuvant tyrosine kinase inhibitor therapy.
Collapse
Affiliation(s)
- Michael Schmieder
- Department of Internal Medicine, Alb-Fils-Kliniken Göppingen, Germany
| | - Doris Henne-Bruns
- Department of General and Visceral Surgery, University Hospital Ulm Ulm, Germany
| | - Benjamin Mayer
- Institute of Epidemiology and Medical Biometry, University of Ulm Ulm, Germany
| | - Uwe Knippschild
- Department of General and Visceral Surgery, University Hospital Ulm Ulm, Germany
| | - Claudia Rolke
- Department of General and Visceral Surgery, University Hospital Ulm Ulm, Germany
| | - Matthias Schwab
- Dr. Margarete Fischer-Bosch Institute of Clinical PharmacologyStuttgart, Germany; Department of Pharmacy and Biochemistry, University of TübingenTübingen, Germany; Department of Clinical Pharmacology, University Hospital TübingenTübingen, Germany
| | - Klaus Kramer
- Department of General and Visceral Surgery, University Hospital Ulm Ulm, Germany
| |
Collapse
|
|
20
|
Li Q, Zhi X, Zhou J, Tao R, Zhang J, Chen P, Røe OD, Sun L, Ma L. Circulating tumor cells as a prognostic and predictive marker in gastrointestinal stromal tumors: a prospective study. Oncotarget 2016; 7:36645-36654. [PMID: 27153560 PMCID: PMC5095028 DOI: 10.18632/oncotarget.9128] [Citation(s) in RCA: 23] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/16/2015] [Accepted: 04/16/2016] [Indexed: 12/11/2022] Open
Abstract
BACKGROUND Circulating tumor cells (CTC) are prognostic and predictive for several cancer types. Only limited data exist regarding prognostic or predictive impact of CTC on gastrointestinal stromal tumor (GIST) patients. The aim of our study was to elucidate the role of CTC in GIST patients. RESULTS A total of 121 GIST patients and 54 non-GIST samples were enrolled in the study. The cutoff value for ANO1 positive was 3*10-5 and 65 (54%) GIST patients were defined as ANO1 positive. ANO1s were more frequently detected in unresectable patients. Tumor size, mitotic count and risk level were associated with ANO1 detection in resectable GIST patients. The presence of ANO1 significantly correlated with poor disease-free survival (15.3 versus 19.6 months, p = 0.038). Most patients turned ANO1-negative after surgery and inversely, all 21 patients with recurrence turned ANO1-positive with high ANO1 expression levels. Moreover, in the neoadjuvant setting, decline of ANO1 expression level correlated with the response of imatinib. METHODS Cells from peripheral blood mononuclear cells tested positive for anoctamin 1, calcium activated chloride channel, ANO1 (DOG1) were considered as tumor CTC of GISTs. The expression levels of ANO1 were determined using quantitative real-time polymerase chain reaction (qRT-PCR). The highest level of ANO1 expression in non-GIST samples was used as the "cutoff" value. CONCLUSION ANO1 detection by qRT-PCR in peripheral blood is of clinical potential for monitoring recurrence and evaluating therapeutic efficacy of imatinib for GIST patients.
Collapse
Affiliation(s)
- Qiang Li
- Department of General Surgery, Affiliated Hospital of Nantong University, Nantong, China
| | - Xiaofei Zhi
- Department of General Surgery, Affiliated Hospital of Nantong University, Nantong, China
| | - Jianping Zhou
- Department of Gastrointestinal Surgery, Affiliated Yixing Hospital of Jiangsu University, Yixing, China
| | - Ran Tao
- Department of General Surgery, Affiliated Hospital of Nantong University, Nantong, China
| | - Jiaxuan Zhang
- Department of General Surgery, Affiliated Hospital of Nantong University, Nantong, China
| | - Peisheng Chen
- Department of General Surgery, Affiliated Hospital of Nantong University, Nantong, China
| | - Oluf Dimitri Røe
- Clinical Cancer Research Center, Aalborg University Hospital, Clinical Institute, Aalborg, Denmark
- Cancer Clinic, Levanger Hospital, Nord-Trøndelag Health Trust, Levanger, Norway
- Department of Cancer Research and Molecular Medicine, Norwegian University of Science and Technology, Trondheim, Norway
| | - Luning Sun
- Research Division of Clinical Pharmacology, First Affiliated Hospital of Nanjing Medical University, Nanjing, China
| | - Lilin Ma
- Department of General Surgery, Affiliated Hospital of Nantong University, Nantong, China
| |
Collapse
|
|
21
|
Valappil FK, Rajan R, Natesh B, Sindhu RS, Raviram S, Mathew J. Evaluation of Gold's nomogram for predicting recurrence-free survival in gastrointestinal stromal tumors in Indian patients. Indian J Gastroenterol 2016; 35:225-8. [PMID: 27256432 DOI: 10.1007/s12664-016-0667-7] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/29/2016] [Accepted: 05/01/2016] [Indexed: 02/04/2023]
Abstract
Gold described a nomogram for prediction of recurrence-free survival (RFS) after surgery for gastrointestinal stromal tumors (GIST). This retrospective study was intended to evaluate the utility of this nomogram for predicting a 2-year RFS in our patients. Twenty-eight consecutive eligible patients from January 2009 to January 2013 who underwent R0 resection and had histopathologically proven GIST were included in the study. Nomogram predicted RFS was compared with observed RFS in four groups as in the National Institutes of Health (NIH)-Fletcher classification. Calibration was assessed by plotting the predicted probabilities of RFS against the actual outcome. For validation of the nomogram, the graph obtained should be closer to the 45-degree line. The observed overall 2-year RFS was 85.7 % (24 patients). Four patients had recurrence within 2 years. The observed RFS was 87.5 %, 77.8 %, 90 %, and 100 % in the high, intermediate, low, and very low risk groups, respectively. The nomogram predicted the 2-year RFS was 40 %, 84.8 %, 88.6 %, and 90 % for high, intermediate, low, and very low risk groups, respectively. Thus, the predicted probabilities of the 2-year RFS in intermediate, low, and very low risk groups were similar to the observed outcomes. However, for the high risk group, the observed RFS was better than predicted RFS. This variation in the high risk group may be due to the use of adjuvant imatinib in our study.
Collapse
Affiliation(s)
- Fysal Kollanta Valappil
- Department of Surgical Gastroenterology, Government Medical College, Thiruvananthapuram, 695 011, India.
| | - Ramesh Rajan
- Department of Surgical Gastroenterology, Government Medical College, Thiruvananthapuram, 695 011, India
| | - Bonny Natesh
- Department of Surgical Gastroenterology, Government Medical College, Thiruvananthapuram, 695 011, India
| | - R S Sindhu
- Department of Surgical Gastroenterology, Government Medical College, Thiruvananthapuram, 695 011, India
| | - S Raviram
- Department of Surgical Gastroenterology, Government Medical College, Thiruvananthapuram, 695 011, India
| | - Jacob Mathew
- Department of Surgical Gastroenterology, Government Medical College, Thiruvananthapuram, 695 011, India
| |
Collapse
|
|
22
|
|
|
23
|
Lee S, Kim YN, Son T, Kim HI, Cheong JH, Hyung WJ, Noh SH. Oncologic Safety of Laparoscopic Wedge Resection with Gastrotomy for Gastric Gastrointestinal Stromal Tumor: Comparison with Conventional Laparoscopic Wedge Resection. J Gastric Cancer 2015; 15:231-7. [PMID: 26819802 PMCID: PMC4722990 DOI: 10.5230/jgc.2015.15.4.231] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/06/2015] [Revised: 10/17/2015] [Accepted: 10/19/2015] [Indexed: 12/13/2022] Open
Abstract
Purpose Various laparoscopic wedge resection (LWR) techniques requiring gastrotomy for gastrointestinal stromal tumors (GISTs) of the stomach have been applied to facilitate tumor resection and preserve the remnant gastric volume. However, there is the possibility of cancer cell dissemination during these procedures. The aim of this study was to assess the oncologic safety of LWR with gastrotomy (LWR-G) compared to LWR without luminal exposure. Materials and Methods Clinicopathologic and operative results of 193 patients who underwent LWR for gastric GIST were retrospectively analyzed from 2003 to 2013. We stratified the patients into two groups: LWR-G and LWR without gastrotomy (LWR-C). Clinicopathologic features, short-term outcomes, and long-term outcomes were compared. Results A total of 26 patients underwent LWR-G, and 167 patients underwent LWR-C. The LWR-G group showed significantly more anterior wall-located (n=10, 38.5%), intraluminal (n=20, 76.9%), and ulcerative (n=13, 50.0%) tumors than the LWR-C group (n=33, 19.8%; n=96, 57.5%; n=46, 27.5%, respectively). Postoperative short-term outcomes did not differ between the two groups. When tumor staging was compared, no statistical difference was noted. There was no recurrence in the LWR-G group, while 2 patients in the LWR-C group experienced recurrence. The two recurrences in the LWR-C group were found in the liver and in the remnant stomach at 63 and 12 months after the operation, respectively. No gastric GIST-related death was recorded in any group during the study period. Conclusions LWR-G for gastric GIST is an oncologically safe procedure even for masses with ulcerations.
Collapse
Affiliation(s)
- Sejin Lee
- Department of Surgery, Yonsei University College of Medicine, Seoul, Korea
| | - You Na Kim
- Department of Surgery, Yonsei University College of Medicine, Seoul, Korea
| | - Taeil Son
- Department of Surgery, Yonsei University College of Medicine, Seoul, Korea
| | - Hyoung-Il Kim
- Department of Surgery, Yonsei University College of Medicine, Seoul, Korea
| | - Jae-Ho Cheong
- Department of Surgery, Yonsei University College of Medicine, Seoul, Korea
| | - Woo Jin Hyung
- Department of Surgery, Yonsei University College of Medicine, Seoul, Korea.; Robot and Minimally Invasive Surgery Center, Yonsei University College of Medicine, Seoul, Korea
| | - Sung Hoon Noh
- Department of Surgery, Yonsei University College of Medicine, Seoul, Korea
| |
Collapse
|
|
24
|
Current management of gastrointestinal stromal tumors: Surgery, current biomarkers, mutations, and therapy. Surgery 2015; 158:1149-64. [DOI: 10.1016/j.surg.2015.06.027] [Citation(s) in RCA: 46] [Impact Index Per Article: 4.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/23/2015] [Revised: 06/18/2015] [Accepted: 06/24/2015] [Indexed: 12/11/2022]
|
|
25
|
Gastrointestinal Stromal Tumor Mimicking as Ovarian Tumor in Gynaecologic Oncology. Indian J Surg Oncol 2015; 7:56-61. [PMID: 27065683 DOI: 10.1007/s13193-015-0479-8] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/09/2015] [Accepted: 10/13/2015] [Indexed: 02/02/2023] Open
Abstract
To report the clinical presentation and outcomes of a series of patients who presented with abdominal/pelvic mass or pelvic pain and were diagnosed with a gastrointestinal stromal tumor (GIST). Retrospective data were collected of all patients who presented with an abdominal/pelvic mass or pelvic pain between January 2010 and July 2015 and who were ultimately diagnosed with a GIST. The patients' medical records were reviewed. A literature review was also conducted. The event free survival and overall survival was calculated for all patients using Kaplan Meier curve (SPSS19-SPSS Inc. USA). A total ten patients were identified with GIST during the study period. Eight of ten patients had a tumor in the small intestine, one in sigmoid colon and one in base of small bowel mesentry. The mean tumor size was 13.9 cm (range, 3.9 to 24 cm). A complete resection was achieved in all 10 patients. No patient had distance metastasis. There were no intraoperative complications. One patient developed postoperative intestinal fistula and was managed conservatively. All patients were treated with imatinib after surgery. The mean follow-up time was 18 months (range, 2 to 47 months). The seven of the 10 patients (70 %) with no evidence of disease, two (20 %) lost follow up and one patient developed recurrence during follow up period and was started on sunitinib and patient died during follow up period because of disease. Gastrointestinal stromal tumors should be considered in the differential diagnosis of patients presenting with an abdominal/pelvic mass or pelvic pain in Gynaecologic oncology department. In such unusual circumstances the complete resection and appropriate adjuvant treatment results in complete durable remission.
Collapse
|
|
26
|
Chok AY, Goh BKP, Koh YX, Lye WK, Allen JC, Quek R, Teo MCC, Chow PKH, Ong HS, Chung AYF, Wong WK. Validation of the MSKCC Gastrointestinal Stromal Tumor Nomogram and Comparison with Other Prognostication Systems: Single-Institution Experience with 289 Patients. Ann Surg Oncol 2015; 22:3597-3605. [PMID: 25652053 DOI: 10.1245/s10434-015-4400-z] [Citation(s) in RCA: 26] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/02/2014] [Indexed: 12/31/2022]
Abstract
PURPOSE To validate the Memorial Sloan Kettering Cancer Center (MSKCC) prognostic nomogram in a single-institution cohort of patients with gastrointestinal stromal tumors (GISTs), and to compare its predictive accuracy against other established risk classification systems, including the National Institutes of Health (NIH), Armed Forces Institute of Pathology (AFIP), and Joensuu criteria. METHODS We retrospectively reviewed 289 patients who underwent surgical resection for primary localized GISTs without adjuvant imatinib therapy and compared the actuarial recurrence-free survival (RFS) with the predicted RFS. RESULTS Tumors >5 cm in size, with high mitotic index, and which had ruptured were significantly associated with recurrent disease. The 2-year RFS was 77.2 % [95 % confidence interval (CI) 71.6-81.8], and the 5-year RFS was 67.9 % (95 % CI 61.7-73.4). The concordance probability of the nomogram of 2-year RFS was 0.71 (SE 0.02), and 5-year RFS was 0.71 (SE 0.19). The 2-year and 5-year MSKCC nomogram probability calculations and the AFIP criteria gave a better estimation of RFS compared to the NIH (p < 0.001) and Joensuu (p < 0.001) criteria. There was no significant difference between the predictive accuracy of the nomogram compared to the AFIP criteria. CONCLUSIONS The MSKCC nomogram slightly underestimated the probability of RFS after surgical resection of GISTs. It was associated with a significantly better predictive accuracy compared to the NIH and Joensuu. This study suggests that there is a wider than expected prognostic divergence between gastric GISTs versus GISTs arising from the small intestine.
Collapse
Affiliation(s)
- Aik-Yong Chok
- Department of Hepatopancreatobiliary and Transplantation Surgery, Singapore General Hospital, Singapore, Singapore
| | - Brian K P Goh
- Department of Hepatopancreatobiliary and Transplantation Surgery, Singapore General Hospital, Singapore, Singapore.
- Duke-NUS Graduate Medical School, Singapore, Singapore.
| | - Ye-Xin Koh
- Department of Hepatopancreatobiliary and Transplantation Surgery, Singapore General Hospital, Singapore, Singapore
| | - Weng-Kit Lye
- Duke-NUS Graduate Medical School, Singapore, Singapore
| | - John C Allen
- Duke-NUS Graduate Medical School, Singapore, Singapore
| | - Richard Quek
- Division of Medical Oncology, National Cancer Centre, Singapore, Singapore
| | - Melissa C C Teo
- Division of Surgical Oncology, National Cancer Centre, Singapore, Singapore
| | - Pierce K H Chow
- Department of Hepatopancreatobiliary and Transplantation Surgery, Singapore General Hospital, Singapore, Singapore
- Duke-NUS Graduate Medical School, Singapore, Singapore
| | - Hock-Soo Ong
- Department of Upper Gastrointestinal and Bariatric Surgery, Singapore General Hospital, Singapore, Singapore
| | - Alexander Y F Chung
- Department of Hepatopancreatobiliary and Transplantation Surgery, Singapore General Hospital, Singapore, Singapore
| | - Wai-Keong Wong
- Department of Upper Gastrointestinal and Bariatric Surgery, Singapore General Hospital, Singapore, Singapore
| |
Collapse
|
|
27
|
Marano L, Boccardi V, Marrelli D, Roviello F. Duodenal gastrointestinal stromal tumor: From clinicopathological features to surgical outcomes. EUROPEAN JOURNAL OF SURGICAL ONCOLOGY 2015; 41:814-822. [PMID: 25956211 DOI: 10.1016/j.ejso.2015.04.004] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/20/2015] [Revised: 03/01/2015] [Accepted: 04/08/2015] [Indexed: 02/07/2023]
Abstract
Duodenal gastrointestinal tumors represent an extremely rare subset of stromal tumors arising from interstitial cells of Cajal. In the last 30 years the comprehension of the pathophysiology and natural history of this previously misunderstood clinical entity, in association with developments in endoscopy, imaging technology, and immunohistochemistry has resulted in novel diagnostic and treatment approaches. This is a comprehensive review of the current data of the literature on the various aspects of the diagnosis and treatment of these tumors. The duodenum is the less commonly involved site for these tumors in the digestive tract. Endoscopy and computed tomography can usually establish the diagnosis, confirmed by immunohistochemical staining and occasionally molecular genetic analysis. Endoscopic ultrasound with fine needle aspiration has been recently found to be the gold diagnostic standard with high sensitivity and specificity rates, diagnosing GIST in up to 80% of patients. Due to the complex anatomy of the pancreatico-duodenal region optimal therapeutic strategy of duodenal GISTs are challenging. Nevertheless surgical resection with microscopically clear resection margins seems to be the only potentially curative treatment for non-metastatic primary GISTs of the duodenum. Imatinib mesylate plays a key role in the management of GISTs both as neoadjuvant therapy and in patients with recurrent and metastatic disease. Meanwhile, the advances in the comprehension of the pathophysiology and natural history of this previously misunderstood clinical entity as well as the treatment of these tumors may render feasible, in the near future, the advent of newer and more effective treatment options.
Collapse
Affiliation(s)
- L Marano
- Unit of General and Minimally Invasive Surgery, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100, Siena, Italy.
| | - V Boccardi
- Unit of General and Minimally Invasive Surgery, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100, Siena, Italy
| | - D Marrelli
- Unit of General and Minimally Invasive Surgery, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100, Siena, Italy
| | - F Roviello
- Unit of General and Minimally Invasive Surgery, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100, Siena, Italy
| |
Collapse
|
|
28
|
Theodoropoulos DG. Gastrointestinal stromal tumors of the colon and rectum. SEMINARS IN COLON AND RECTAL SURGERY 2015. [DOI: 10.1053/j.scrs.2015.01.006] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
|
|
29
|
Karaca N, Akpak YK, Tatar Z, Batmaz G, Erken A. Gastrointestinal Stromal Tumor: May Mimic Adnexal Mass. Glob J Health Sci 2015; 8:20-6. [PMID: 26383211 PMCID: PMC4803964 DOI: 10.5539/gjhs.v8n2p20] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/24/2015] [Accepted: 06/01/2015] [Indexed: 11/17/2022] Open
Abstract
Gastrointestinal stromal tumors (GISTs) are rare tumor of the gastrointestinal tract. GISTs occur in the entire gastrointestinal tract and may also arise from the retroperitoneum, omentum and mesenteries. They are originated from gastrointestinal pacemaker cells (Cajal's interstitial cells) and range from benign tumors to sarcomas at all sites of occurrence. Diagnosis of GIST could be deceptive because of their similarity in appearance to gynecological neoplasms. We would like to present a case of a woman with GIST in the small intestine giving a imprint of an adnexal mass was diagnosed correctly during surgery. The diagnosis and treatment of GIST has been reformed over the past years. It is crucial to separate GISTs from possible misdiagnosis because their prognosis and treatment could be unlike clearly. The purpose of this case is to evaluate this rarely seen clinical entity, and thus, make some contribution to the literature.
Collapse
Affiliation(s)
- Nilay Karaca
- Bezmi Alem Vakif University, Medical Faculty, Department of Obstetrics and Gynecology, Istanbul, Turkey.
| | | | | | | | | |
Collapse
|
|
30
|
Kasetsermwiriya W, Nagai E, Nakata K, Nagayoshi Y, Shimizu S, Tanaka M. Laparoscopic surgery for gastric gastrointestinal stromal tumor is feasible irrespective of tumor size. J Laparoendosc Adv Surg Tech A 2015; 24:123-9. [PMID: 24625346 DOI: 10.1089/lap.2013.0433] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022] Open
Abstract
PURPOSE To compare the outcomes of laparoscopic surgery and open surgery for gastric gastrointestinal stromal tumors (GISTs) by size-matched analysis and evaluate whether laparoscopic surgery for lesions of >5 cm is feasible. PATIENTS AND METHODS Data of 44 consecutive patients with gastric GIST who underwent surgery from 1988 to 2011 were reviewed. Twenty-three patients who underwent successful laparoscopic surgery were compared with 10 patients with similar tumor sizes who underwent open surgery. Among the 23 patients in the laparoscopic group, we compared postoperative results between GISTs of ≤5 cm and >5 cm. RESULTS There were no differences in clinicopathological characteristics between the laparoscopic surgery group (LG) and the open surgery group (OG). The operation time was not different, but the blood loss (5.5 mL [range, 0-425 mL] in LG and 125 mL [range, 0-676 mL] in OG) (P=.008) and postoperative hospital stay (21 days in OG and 8 days in LG) (P<.001) were significantly less in the LG. Postoperative complications and recurrence were not different. Comparison between patients with lesions of >5 cm and patients with smaller lesions in the LG found that smaller lesions were associated with a shorter postoperative hospital stay (7.5 days versus 11 days) (P=.037). CONCLUSIONS Laparoscopic resection of primary gastric GISTs is feasible even for tumors of >5 cm.
Collapse
Affiliation(s)
- Wisit Kasetsermwiriya
- Department of Surgery and Oncology, Graduate School of Medical Sciences, Kyushu University , Fukuoka, Japan
| | | | | | | | | | | |
Collapse
|
|
31
|
Jones RL. Practical aspects of risk assessment in gastrointestinal stromal tumors. J Gastrointest Cancer 2015; 45:262-7. [PMID: 24802226 PMCID: PMC4126997 DOI: 10.1007/s12029-014-9615-x] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
INTRODUCTION Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors of the gastrointestinal tract, which are characterized in the majority of cases by activating mutations in KIT and platelet-derived growth factor receptor alpha (PDGFRA). The introduction of tyrosine kinase inhibitors has revolutionized the management of patients with metastatic GIST. However, complete surgical resection remains the mainstay of management for those with localized disease. Recently, three large trials have confirmed the benefit of adjuvant imatinib therapy in patients who were at high risk of recurrence following complete resection. In this setting, it is critical that oncologists understand the various GIST risk assessment criteria and be able to apply these methods to accurately assess the risk of recurrence and the need for adjuvant imatinib therapy. PURPOSE The aim of this review is to outline the risk stratification systems currently available to oncologists who are treating patients with GIST, so they can be optimally applied for clinical decision-making.
Collapse
Affiliation(s)
- R L Jones
- Division of Medical Oncology, Fred Hutchinson Cancer Research Center, University of Washington, 825 Eastlake Avenue East, G-3630, Seattle, WA, 98109-1023, USA,
| |
Collapse
|
|
32
|
Kim IH, Kwak SG, Chae HD. Prognostic Factors of Patients with Gastric Gastrointestinal Stromal Tumor after Curative Resection: A Retrospective Analysis of 406 Consecutive Cases in a Multicenter Study. Eur Surg Res 2015; 55:12-23. [PMID: 25766570 DOI: 10.1159/000375234] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/14/2014] [Accepted: 01/13/2015] [Indexed: 11/19/2022]
Abstract
BACKGROUND/PURPOSE Gastric gastrointestinal stromal tumors (GISTs) have a highly variable clinical course, and recurrent disease sometimes develops despite curative surgery. This study was undertaken to investigate the surgical role in treating gastric GISTs and evaluate the clinicopathological features of a large series of patients who underwent curative resection for gastric GISTs to clarify which features were independent prognostic factors. METHODS The clinicopathological data of 406 patients with gastric GISTs who underwent curative resection at 4 university hospitals in Daegu, South Korea, from March 1998 to March 2012 were reviewed. All cases were confirmed as gastric GISTs by immunohistochemical staining, in which CD117 or CD34 was positive. Clinical follow-up was performed periodically, and disease-free survival rates were retrospectively investigated using the medical records. RESULTS The mean follow-up period was 42.9 months (range: 2-166). There were 11 recurrent patients (2.7%). Due to the small number of recurrences, age, sex and location were controlled using propensity score matching before performing any statistical analysis. Tumor size, mitotic count, NIH classification, and cellularity were judged to be independent prognostic factors for recurrence by univariate analysis. In a multivariate analysis, tumor size and mitotic count were significantly and independently related to recurrence, and tumor size was determined to be the most important prognostic factor for recurrence after curative resection (hazard ratio: 1.204; p < 0.01). CONCLUSIONS The results of this multicenter study demonstrate that disease-free survival rates are good. Tumor size was disclosed as the most important factor for recurrence in gastric GIST patients who underwent radical resection.
Collapse
Affiliation(s)
- In-Hwan Kim
- Department of Surgery, School of Medicine, Catholic University of Daegu, Daegu, South Korea
| | | | | |
Collapse
|
|
33
|
Kim IH, Kim IH, Kwak SG, Kim SW, Chae HD. Gastrointestinal stromal tumors (GISTs) of the stomach: a multicenter, retrospective study of curatively resected gastric GISTs. Ann Surg Treat Res 2014; 87:298-303. [PMID: 25485237 PMCID: PMC4255548 DOI: 10.4174/astr.2014.87.6.298] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/20/2014] [Revised: 06/18/2014] [Accepted: 07/03/2014] [Indexed: 12/13/2022] Open
Abstract
Purpose The standard treatment for primary localized gastric gastrointestinal stromal tumor (GIST) is surgical resection. The clinical behavior of gastric GIST after surgical resection is extremely variable. We conducted a multicenter, retrospective study of gastric GISTs patients who underwent curative surgical resection to evaluate clinical features and the prognosis of surgically treated gastric GISTs. Methods We performed a retrospective study on 406 consecutive patients who underwent curative resections for localized gastric GIST at four university hospitals in Daegu, Korea, between March 1998 and March 2012. The retrospectively collected medical records were reviewed with respect to clinical parameters including age, gender, tumor location, surgical approach, and recurrence. Results There were 406 patients: 157 males (38.7%) and 249 females (61.3%), with a mean age of 60.8 ± 10.8 (standard deviation) years. The mean tumor size was 4.9 cm (range, 0.3-29 cm). Curative surgical resection was performed in all patients without tumor rupture or spillage. Laparoscopic wedge resections were performed in 156 patients (38.4%) and open resections in 250 patients (61.6%). The tumor size of the laparoscopic wedge resection group was smaller than that of open resection group (3.45 cm vs. 5.46 cm; P < 0.001). There were 11 recurrent cases (2.7%). No recurrence was observed in patients who underwent laparoscopic wedge resections. Conclusion Gastric GISTs had a low recurrence rate after curative resection in our series. Laparoscopic gastric wedge resection is feasible for treating gastric GISTs in selected patients.
Collapse
Affiliation(s)
- In-Hwan Kim
- Department of Surgery, Catholic University of Daegu School of Medicine, Daegu, Korea
| | - In-Ho Kim
- Department of Surgery, Catholic University of Daegu School of Medicine, Daegu, Korea
| | - Sang-Gyu Kwak
- Department of Medical Statistics, Catholic University of Daegu School of Medicine, Daegu, Korea
| | - Se Won Kim
- Department of Surgery, Yeungnam University College of Medicine, Daegu, Korea
| | - Hyun-Dong Chae
- Department of Surgery, Catholic University of Daegu School of Medicine, Daegu, Korea
| |
Collapse
|
|
34
|
Ma GL, Murphy JD, Martinez ME, Sicklick JK. Epidemiology of gastrointestinal stromal tumors in the era of histology codes: results of a population-based study. Cancer Epidemiol Biomarkers Prev 2014; 24:298-302. [PMID: 25277795 DOI: 10.1158/1055-9965.epi-14-1002] [Citation(s) in RCA: 179] [Impact Index Per Article: 16.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022] Open
Abstract
To date, all population-based epidemiologic data on gastrointestinal stromal tumor (GIST) in the United States predate the 2001 implementation of GIST-specific histology coding. As such, results from previous studies were limited because of inclusion of non-GIST abdominal or gastrointestinal sarcomas. We used a national cancer registry with modern day histologic codes to gain greater insight into the true epidemiology of GIST in the United States. We identified 6,142 patients diagnosed with GIST between 2001 and 2011 in the Surveillance, Epidemiology, and End Results database. Incidence, survival, demographic risk factors, and prognostic factors were analyzed. Annual age-adjusted incidence rose from 0.55/100,000 in 2001 to 0.78/100,000 in 2011 and increased with age, peaking among 70- to 79-year-olds (3.06/100,000). GIST was also more common in males than females [rate ratio (RR), 1.35], non-Hispanics than Hispanics (RR, 1.23), and blacks (RR, 2.07) or Asians/Pacific Islanders (RR, 1.50) than whites. The study period had 5-year overall and GIST-specific survival rates of 65% and 79%, respectively. The 5-year overall survival rates for those with localized, regional, and metastatic disease at diagnosis were 77%, 64%, and 41%, respectively. Multivariate analyses demonstrated that older age at diagnosis, male sex, black race, and advanced stage at diagnosis were independent risk factors for worse overall survival. Multivariate analysis also showed the four aforementioned characteristics, along with earlier year of diagnosis, to be independent risk factors for worse GIST-specific survival. As the first population-based, epidemiologic study of histologically confirmed disease, our findings provide a robust representation of GIST in the era of immunohistochemical diagnoses.
Collapse
Affiliation(s)
- Grace L Ma
- Division of Surgical Oncology and Department of Surgery, Moores Cancer Center, University of California, San Diego, La Jolla, California
| | - James D Murphy
- Department of Radiation Medicine and Applied Sciences, Moores Cancer Center, University of California, San Diego, La Jolla, California
| | - Maria E Martinez
- Department of Family and Preventive Medicine, Moores Cancer Center, University of California, San Diego, La Jolla, California
| | - Jason K Sicklick
- Division of Surgical Oncology and Department of Surgery, Moores Cancer Center, University of California, San Diego, La Jolla, California.
| |
Collapse
|
|
35
|
The True Incidence of Gastric GIST—a Study Based on Morbidly Obese Patients Undergoing Sleeve Gastrectomy. Obes Surg 2014; 24:2134-7. [DOI: 10.1007/s11695-014-1336-5] [Citation(s) in RCA: 32] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
|
|
36
|
Iorio N, Sawaya RA, Friedenberg FK. Review article: the biology, diagnosis and management of gastrointestinal stromal tumours. Aliment Pharmacol Ther 2014; 39:1376-86. [PMID: 24749828 DOI: 10.1111/apt.12761] [Citation(s) in RCA: 42] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/25/2014] [Revised: 03/19/2014] [Accepted: 03/27/2014] [Indexed: 12/16/2022]
Abstract
BACKGROUND Gastrointestinal stromal tumours (GIST) are the most common mesenchymal tumours of the gastrointestinal tract with an increasing incidence. AIMS To review the biology, diagnosis and treatment of gastrointestinal stromal tumours. METHODS A PubMed search using the phrases 'Gastrointestinal stromal tumor', 'imatinib', 'c-kit'. RESULTS The diagnosis of GIST is established by histology supplemented by the immunohistochemical marker CD117, which is positive in 95% of cases. The most common site of the tumour is the stomach. Most GIST are benign with 20-30% malignant. Five-year survival for malignant GIST ranges between 35% and 65% and depends primarily on tumour size, mitotic index and location. The malignant behaviour of GIST is best assessed by invasion of adjacent structures and distant metastases. The gold standard for treatment is surgical resection. Imatinib, a tyrosine kinase inhibitor, is the primary therapy for unresectable, recurrent or metastatic disease. CONCLUSIONS Gastrointestinal stromal tumours are rare tumours of the gastrointestinal tract and they vary in presentation. When surgical resection is not achievable, imatinib is the treatment of choice.
Collapse
Affiliation(s)
- N Iorio
- Department of Medicine, Temple University Hospital, Philadelphia, PA, USA
| | | | | |
Collapse
|
|
37
|
Chiang NJ, Chen LT, Tsai CR, Chang JS. The epidemiology of gastrointestinal stromal tumors in Taiwan, 1998-2008: a nation-wide cancer registry-based study. BMC Cancer 2014; 14:102. [PMID: 24548660 PMCID: PMC3932802 DOI: 10.1186/1471-2407-14-102] [Citation(s) in RCA: 37] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/23/2013] [Accepted: 02/12/2014] [Indexed: 01/24/2023] Open
Abstract
Background To investigate the incidence of gastrointestinal stromal tumors (GISTs) in Taiwan and the impact of imatinib on the overall survival (OS) of GIST patients. Methods GISTs were identified from the Taiwan Cancer Registry (TCR) from 1998 to 2008. The age-adjusted incidence rates and the observed OS rates were calculated. Cox proportional hazards models were applied to examine the mortality risk in three time periods (1998–2001, 2002–2004, 2005–2008) according to the application and availability of imatinib. Results From 1998 to 2008, 2,986 GISTs were diagnosed in Taiwan. The incidence increased from 1.13 per 100,000 in 1998 to 1.97 per 100,000 in 2008. The most common sites were stomach (47-59%), small intestine (31-38%), and colon/rectum (6-9%). The 5-year observed OS was 66.5% (60.3% for men, 74.2% for women, P < .0001). GISTs in the stomach had a better 5-year observed OS (69.4%) than those in the small intestine (65.1%) (P < .0001). The outcome of GIST improved significantly after the more widespread use of imatinib; the 5-year observed OS increased from 58.9% during 1998–2001 to 70.2% during 2005–2008 (P < .0001). Younger age, female sex, stomach location, and later diagnostic years were independent predictors of a better survival. Conclusions The incidence of GIST has been increasing in Taiwan, partially due to the advancement of diagnostic technology/method and the increased awareness by physicians. The outcome of GIST has improved significantly with the availability and the wider use of imatinib.
Collapse
Affiliation(s)
| | - Li-Tzong Chen
- National Institute of Cancer Research, National Health Research Institutes, 2 F, No, 367, Sheng Li Road, Tainan 70456, Taiwan.
| | | | | |
Collapse
|
|
38
|
Lin M, Lin JX, Huang CM, Zheng CH, Li P, Xie JW, Wang JB, Lu J. Prognostic analysis of gastric gastrointestinal stromal tumor with synchronous gastric cancer. World J Surg Oncol 2014; 12:25. [PMID: 24479763 PMCID: PMC3917900 DOI: 10.1186/1477-7819-12-25] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/02/2013] [Accepted: 01/18/2014] [Indexed: 12/22/2022] Open
Abstract
BACKGROUND Many patients with gastric gastrointestinal stromal tumor (GIST) and synchronous gastric cancer have been described, most in single case studies. We retrospectively investigated the clinicopathologic features and prognostic effects of gastric GIST in patients with synchronous gastric cancer. METHODS The study enrolled 170 patients with gastric GIST, who had undergone complete surgical resection (R0) from January 2000 to December 2011. Forty-two patients had synchronous gastric cancer (CA Group), whereas 128 did not (Non-CA Group). The clinicopathologic features and potential prognostic factors in the two groups were compared. RESULTS Patients in the CA Group had more obvious symptoms, but a lower rate of preoperative diagnosis of gastric GIST (P <0.05). The two groups differed significantly in gender, age, greatest tumor diameter, risk stratification, tumor-associated ulcers, and CD117 and CD34 expression (P <0.05 each). Univariate analysis showed that age, risk stratification, postoperative oral imatinib and synchronous gastric cancer were predictive factors of survival (P <0.05). Cox regression analysis showed that risk stratification, postoperative oral imatinib and synchronous gastric cancer were independent predictors of survival (P <0.05). Stratified analysis showed that the 5-year overall survival rate was lower in patients with synchronous gastric cancer than in those without synchronous gastric cancer. CONCLUSIONS Gastric GIST with synchronous gastric cancer had a lower rate of preoperative diagnosis, with correct diagnosis often missed. Survival, however, depended primarily on the gastric cancer.
Collapse
Affiliation(s)
- Mi Lin
- Department of Gastric Surgery, Fujian Medical University Union Hospital, No.29 Xinquan Road, Fuzhou 350001, Fujian Province, China
| | - Jian-Xian Lin
- Department of Gastric Surgery, Fujian Medical University Union Hospital, No.29 Xinquan Road, Fuzhou 350001, Fujian Province, China
| | - Chang-Ming Huang
- Department of Gastric Surgery, Fujian Medical University Union Hospital, No.29 Xinquan Road, Fuzhou 350001, Fujian Province, China
| | - Chao-Hui Zheng
- Department of Gastric Surgery, Fujian Medical University Union Hospital, No.29 Xinquan Road, Fuzhou 350001, Fujian Province, China
| | - Ping Li
- Department of Gastric Surgery, Fujian Medical University Union Hospital, No.29 Xinquan Road, Fuzhou 350001, Fujian Province, China
| | - Jian-Wei Xie
- Department of Gastric Surgery, Fujian Medical University Union Hospital, No.29 Xinquan Road, Fuzhou 350001, Fujian Province, China
| | - Jia-Bin Wang
- Department of Gastric Surgery, Fujian Medical University Union Hospital, No.29 Xinquan Road, Fuzhou 350001, Fujian Province, China
| | - Jun Lu
- Department of Gastric Surgery, Fujian Medical University Union Hospital, No.29 Xinquan Road, Fuzhou 350001, Fujian Province, China
| |
Collapse
|
|
39
|
Clinicopathologic factors predicting outcomes in patients with gastrointestinal stromal tumors of the rectum and colon. Tumour Biol 2013; 35:4357-62. [PMID: 24375257 DOI: 10.1007/s13277-013-1572-7] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/07/2013] [Accepted: 12/17/2013] [Indexed: 12/19/2022] Open
Abstract
Limited information is available concerning the clinicopathologic profile of colorectal gastrointestinal stromal tumors (GISTs), which are relatively rare, as well as survival rates following surgical resection. The present study was designed to describe the clinicopathologic characteristics of patients with colorectal GISTs and identify potential factors that may predict postoperative survival outcomes. We reviewed the medical records of 67 patients with colorectal GISTs who underwent surgical resection between January 2000 and December 2012. Clinicopathologic factors affecting overall survival were assessed using the Kaplan-Meier method and multivariate Cox proportional hazards models. The median age at diagnosis was 57 years (range, 32-79 years), with a male-to-female ratio of 1.68. Tumor size varied from 0.2 to 11 cm, with a median size of 5.7 cm. Sixty-two tumors (93%) were positive for CD117, 53 (79%) for CD34, 6 (9%) for PDGFRA, 15 (22%) for SMA, 5 (8%) for S100, and 39 (58%) for vimentin. The overall median survival time was 54 months (95% confidence interval, 32-59 months), and the 1-, 3-, and 5-year overall survival rates were 89.6, 63.9, and 34.4%, respectively. High-risk tumors (determined by histologic grade, size, and other histologic variables) were associated with poor prognosis (hazard ratio, 1.83; 95% confidence interval, 1.21-2.78), and patients who received adjuvant treatment with imatinib had significantly longer median overall survival times than patients who did not (hazard ratio, 0.43; 95% confidence interval, 0.24-0.80). In patients with colorectal GISTs, high-risk tumor histologic grade can predict poor prognosis, and patients may benefit from adjuvant treatment with imatinib. Findings from the present study may provide information to establish evidence-based management strategies for colorectal GISTs.
Collapse
|
|
40
|
Tabrizian P, Sweeney RE, Uhr JH, Nguyen SQ, Divino CM. Laparoscopic resection of gastric and small bowel gastrointestinal stromal tumors: 10-year experience at a single center. J Am Coll Surg 2013; 218:367-73. [PMID: 24559952 DOI: 10.1016/j.jamcollsurg.2013.11.029] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/28/2013] [Revised: 11/18/2013] [Accepted: 11/27/2013] [Indexed: 12/17/2022]
Abstract
BACKGROUND Complete curative resection remains the treatment of choice for nonmetastatic gastrointestinal stromal tumors (GISTs). The safety and feasibility of laparoscopy in the treatment of this disease has been shown, however, the long-term oncologic outcomes of this technique remain unclear. STUDY DESIGN An ongoing prospectively maintained database including all laparoscopically resected gastric and small bowel GISTs (n = 116) at Mount Sinai Medical Center from July 1999 to December 2011 was retrospectively analyzed. Recurrence and survival outcomes were calculated using the Kaplan-Meier method and compared with log-rank test. RESULTS Tumors were of gastric (77.6%) and small bowel (22.4%) origins. Overall mean tumor size was 4.0 cm (±2.7 cm) and R0 resection was achieved in 113 (97.4%) cases. Overall perioperative complication rate was 14.7%, with a reoperative rate of 4.3% at 90 days. When comparing gastric with small bowel GISTs, a more acute presentation requiring emergent resections was noted in patients with small bowel GISTs (p = 008). However tumor size, operative data, and perioperative outcomes were comparable in both groups (p = NS). At a median follow-up of 56.4 months (range 0.1 to 162.4 months), recurrence rate was 7.8% and comparable in both gastric and small bowel GISTs (p = NS). Risk factors for recurrence on univariate analysis were presence of ulceration/necrosis (p < 0.001) and tumor size >5 cm (p = 0.05). Overall 10-year survival rate was 90.8%. Gastric and small bowel overall survival rates were similar (90.7% vs 91.3%, respectively). Overall 10-year disease-free survival was 80.0% (84.3% gastric vs 71.6% small bowel; p = NS). CONCLUSIONS Our series demonstrates the safety and feasibility of laparoscopy in patients undergoing resection of small bowel and gastric GISTs. Comparable long-term oncologic outcomes with a 10-year survival of 90.8% were achieved.
Collapse
Affiliation(s)
- Parissa Tabrizian
- Division of General Surgery, Department of Surgery, Icahn School of Medicine at Mount Sinai, New York, NY
| | - Robert E Sweeney
- Division of General Surgery, Department of Surgery, Icahn School of Medicine at Mount Sinai, New York, NY
| | - Joshua H Uhr
- Division of General Surgery, Department of Surgery, Icahn School of Medicine at Mount Sinai, New York, NY
| | - Scott Q Nguyen
- Division of General Surgery, Department of Surgery, Icahn School of Medicine at Mount Sinai, New York, NY
| | - Celia M Divino
- Division of General Surgery, Department of Surgery, Icahn School of Medicine at Mount Sinai, New York, NY.
| |
Collapse
|
|
41
|
Yang H, Shen C, Zhang B, Chen H, Chen Z, Chen J. Expression and clinicopathological significance of CD9 in gastrointestinal stromal tumor. J Korean Med Sci 2013; 28:1443-8. [PMID: 24133347 PMCID: PMC3792597 DOI: 10.3346/jkms.2013.28.10.1443] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/12/2013] [Accepted: 08/12/2013] [Indexed: 02/06/2023] Open
Abstract
This study investigated the expression and clinicopathological significance of CD9 in gastrointestinal stromal tumor (GIST). Immunohistochemistry staining for CD9 was performed on tumor tissues from 74 GIST patients. The correlation with clinicopathological features, risk classification and prognosis was analyzed. CD9-positive staining comprised 59.5% (44/74) of the GIST patients. The CD9-positive expression rate of the sample was significantly associated with diameter (P = 0.028), mitotic counts (P = 0.035), risk classification (P = 0.018) and three-year recurrence-free survival (RFS) (P < 0.001). Cox proportional hazards regression (HR = 0.352; P = 0.015) showed that CD9 is an independent factor for post-operative RFS. The subgroup analysis showed that CD9 expression in gastric stromal tumor (GST) is significantly associated with diameter (P = 0.031), risk classification (P = 0.023) and three-year RFS (P = 0.001). The Cox proportional hazards regression (HR = 0.104; P = 0.006) also showed that CD9 is an independent factor for RFS of GST. However, CD9 expression does not have a statistically significant correlation with clinicopathological features, risk classification, and prognosis in non-GST. In conclusion, CD9 expression in GIST appears to be associated with the recurrence and/or metastasis of GIST patients, especially in GST, which may indicate the important role of CD9 in the malignant biological behavior and prognosis of GST.
Collapse
Affiliation(s)
- Hongxin Yang
- Department of Gastrointestinal Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China
- Department of Gastrointestinal Surgery of the Affiliated Hospital of Guiyang Medical University, Guiyang, Guizhou Province, China
| | - Chaoyong Shen
- Department of Gastrointestinal Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China
| | - Bo Zhang
- Department of Gastrointestinal Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China
| | - Haining Chen
- Department of Gastrointestinal Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China
| | - Zhixin Chen
- Department of Gastrointestinal Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China
| | - Jiaping Chen
- Department of Gastrointestinal Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China
| |
Collapse
|
|
42
|
New fronts in the adjuvant treatment of GIST. Cancer Chemother Pharmacol 2013; 72:715-23. [PMID: 23934322 DOI: 10.1007/s00280-013-2248-0] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/20/2013] [Accepted: 07/26/2013] [Indexed: 12/19/2022]
Abstract
PURPOSE To review the prognostic factors and stratification systems used to determine the need for adjuvant therapy in the treatment of gastrointestinal stromal tumors (GIST), and to review recent clinical advances in investigation of the efficacy and safety of adjuvant imatinib mesylate treatment. METHODS Recent data from clinical trials of various durations of adjuvant imatinib in GIST are reviewed, with emphasis on key results from the Phase III American College of Surgeons Oncology Group (ACOSOG) Z9001 trial and the Scandinavian Sarcoma Group XVIII/Arbeitsgemeinschaft Internistische Onkologie (SSGXVIII/AIO) trial. RESULTS Complete surgical resection remains the standard of treatment for localized GISTs; however, disease recurrence occurs in up to 50 % of patients who undergo complete resection. The ACOSOG Z9001 trial established that 1 year of adjuvant imatinib reduces the risk of recurrence in patients with resected GIST. The SSGXVIII/AIO trial further demonstrated that 3-year adjuvant imatinib improves both recurrence-free survival and overall survival compared with 1-year therapy in patients at high risk of recurrence after surgery. Considering risk factors associated with tumor recurrence is essential for identifying the patients who are most likely to benefit from adjuvant imatinib. CONCLUSIONS Although the optimal duration of adjuvant therapy remains to be determined, results from these pivotal trials provide firm evidence that adjuvant imatinib improves recurrence-free survival and improved overall survival of patients in the SSGXVIII/AIO trial. Ongoing studies may shed further light on the benefits and harms of adjuvant therapy, as well as the most appropriate patient candidates for adjuvant imatinib treatment.
Collapse
|
|
43
|
Zhu H, Lu J, Wang X, Zhang H, Tang X, Zhu J, Mao Y. Upregulated ZO-1 correlates with favorable survival of gastrointestinal stromal tumor. Med Oncol 2013; 30:631. [PMID: 23820955 DOI: 10.1007/s12032-013-0631-7] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/23/2013] [Accepted: 06/10/2013] [Indexed: 12/14/2022]
Abstract
Zonula occludens-1 (ZO-1) is a membrane-scaffolding protein that plays an important role in maintaining tight-junction integrity, which is disrupted in many invasive cancers and intestinal diseases. However, the expression of ZO-1 in gastrointestinal stromal tumor (GIST) and its relationship with clinical characteristics of this disease remain poorly understood. In this study, immunohistochemical analysis using tissue microarray was employed to evaluate the expression of ZO-1 in GIST and to investigate the relationship between its expression and GIST prognosis. High ZO-1 expression was displayed in 71.8 % of GIST patients, which was related to tumor diameter (p < 0.05). The Kaplan-Meier method and log-rank test indicated that high ZO-1 expression, small tumor diameter, tumor position in the esophagus, and a borderline-to-intermediate tumor grade displayed significant correlations with longer survival of GIST patients. The data suggest that ZO-1 expression is correlated with malignant phenotypes of GIST and it may serve as a favorable prognostic factor for GIST. These results also support a role for ZO-1 as a tumor-suppressor gene in GIST.
Collapse
Affiliation(s)
- Huijun Zhu
- Department of Pathology and Laboratory Medicine, Nantong University Affiliated Hospital, Nantong, China.
| | | | | | | | | | | | | |
Collapse
|
|
44
|
Rutkowski P, Gronchi A. Efficacy and economic value of adjuvant imatinib for gastrointestinal stromal tumors. Oncologist 2013; 18:689-96. [PMID: 23709752 DOI: 10.1634/theoncologist.2012-0474] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022] Open
Abstract
OBJECTIVE This article presents the clinical effectiveness and cost-effectiveness of the use of adjuvant imatinib mesylate for treating patients with localized primary gastrointestinal stromal tumors (GISTs) and discusses the impact of prolonged treatment with adjuvant imatinib on health care costs. METHODS A systematic review of the medical literature was conducted to explore recently reported clinical trials demonstrating the clinical benefit of adjuvant imatinib in GISTs, along with analyses discussing the economic impact of adjuvant imatinib. RESULTS Two phase III trials have demonstrated a significant clinical benefit of adjuvant imatinib treatment in GIST patients at risk of recurrence after tumor resection. Guidelines now suggest adjuvant treatment for at least 3 years in patients at high risk of recurrence. Despite this clinical effectiveness, prolonged use of adjuvant imatinib can lead to an increase in the risk for adverse events and to increased costs for both patients and health care systems. However, the increased cost is partially offset by cost reductions associated with delayed or avoided GIST recurrences. Three years of adjuvant treatment in high-risk patients was concluded to be cost-effective. Therefore, the careful selection of patients who are most likely to benefit from treatment can lead to improved clinical outcomes and significant cost savings. CONCLUSION Although introducing adjuvant imatinib has an economic impact on health plans, this effect seems to be limited. Several analyses have demonstrated that adjuvant imatinib is more cost-effective for treating localized primary GISTs than surgery alone. In addition, 3 years of adjuvant imatinib is more cost-effective than 1 year of adjuvant therapy.
Collapse
Affiliation(s)
- Piotr Rutkowski
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie Memorial Cancer Center and Institute of Oncology, Warsaw, Poland.
| | | |
Collapse
|
|
45
|
Clinicopathological features and prognostic factors of rectal gastrointestinal stromal tumors. J Gastrointest Surg 2013; 17:793-8. [PMID: 23288714 DOI: 10.1007/s11605-012-2086-0] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/05/2012] [Accepted: 11/12/2012] [Indexed: 01/31/2023]
Abstract
AIM Rectal gastrointestinal stromal tumor (GIST) was a rare disease. This study sought to summarize clinicopathological features and prognostic factors of rectal GISTs. METHODS Clinicopathological characteristics and prognostic factors of rectal GISTs were investigated by reviewing patients undergoing curative resection at the Fudan University Shanghai Cancer Center between 1986 and 2010. RESULTS Twenty-one patients, 15 male and 6 female, were included. The mean age of onset was 51 years. The most common initial presentation was hematochezia (7 cases). Eleven patients underwent radical resection, and the other 10 received local resection. No lymph node metastases were identified. All cases were positive for CD117. Seventeen patients were classified as high National Institutes of Health (NIH) risk category. The 5-year disease-free survival (DFS) and overall survival were 33 and 46 %, respectively. Fifteen cases had recurrence postoperatively. Both univariate and multivariate analyses demonstrated the NIH risk category (p = 0.028) and hematochezia symptom (p = 0.014) were independent prognostic factors of the DFS of patients. CONCLUSIONS Hematochezia was the most common initial symptom. Over 50 % of patients received radical surgery. More than 80 % of patients were at high NIH risk of recurrence. Hematochezia symptom and high NIH risk category indicated poor prognosis of rectal GISTs.
Collapse
|
|
46
|
Risk stratification models and mutational analysis: keys to optimising adjuvant therapy in patients with gastrointestinal stromal tumour. Eur J Cancer 2012. [PMID: 23206668 DOI: 10.1016/j.ejca.2012.10.025] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/02/2023]
Abstract
Imatinib is a standard of care in the adjuvant treatment of patients with resected gastrointestinal stromal tumour (GIST). Two important trials have shown a reduction in GIST recurrence rates for patients treated with imatinib 400 mg daily for 1 year; one of these trials also demonstrated a significant improvement in overall survival for patients with GIST at high risk of recurrence who were treated for 3 years. However, not all patients will benefit from adjuvant treatment. Considering the patient types in both trials, treatment decisions must take into account a number of factors including risk of recurrence and mutational status. Tumour characteristics including tumour size, location and mitotic index are the main prognostic factors of recurrence-free survival (RFS) after surgical resection of GISTs. Research, much of it in the advanced/metastatic setting, shows that mutational analysis is definitely predictive of treatment efficacy and probably prognostic of RFS. Patients on imatinib whose tumours harbour mutations in exon 11 of the KIT gene tend to have superior RFS compared with patients with exon 9 mutations. In contrast, patients with wild-type GIST often have disease that follows an indolent course and has limited sensitivity to imatinib in most cases. As such, increased use of existing risk-stratification schemes and mutational analysis will be essential for optimising tailored treatment approaches. In this review, the development and prognostic/predictive utility of key risk stratification tools and mutational analysis of GIST are discussed herein with the goal of facilitating adjuvant treatment decisions for patients with GIST.
Collapse
|
|
47
|
Pornsuksiri K, Chewatanakornkul S, Kanngurn S, Maneechay W, Chaiyapan W, Sangkhathat S. Clinical outcomes of gastrointestinal stromal tumor in southern Thailand. World J Gastrointest Oncol 2012; 4:216-222. [PMID: 23444235 PMCID: PMC3581833 DOI: 10.4251/wjgo.v4.i11.216] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/11/2012] [Revised: 09/04/2012] [Accepted: 10/20/2012] [Indexed: 02/05/2023] Open
Abstract
AIM To review a single institutional experience in clinical management of gastrointestinal stromal tumors (GIST) and analyze for factors determining treatment outcome. METHODS Clinicopathological data of patients with a diagnosis of GIST who were treated at our institute during November 2004 to September 2009 were retrospectively reviewed. RESULTS Ninety-nine cases were included in the analysis. Primary tumor sites were at the stomach in and small bowel in 44% and 33%, respectively. Thirty-one cases already had metastasis at presentation and the most common metastatic site was the liver. Sixty-four cases (65%) were in the high-risk category. Surgical treatment was performed in 77 cases (78%), 3 of whom received upfront targeted therapy. Complete resection was achieved in 56 cases (73% of operative cases) and of whom 27 developed local recurrence or distant metastasis at a median duration of 2 years. Imatinib was given as a primary therapy in unresectable cases (25 cases) and as an adjuvant in cases with residual tumor (21 cases). Targeted therapy gave partial response in 7 cases (15%), stable disease in 27 cases (57%) and progressive disease in 13 cases (28%). Four-year overall survival was 74% (95% CI: 61%-83%). Univariate survival analysis found that low-risk tumor, gastric site, complete resection and response to imatinib were associated with better survival. CONCLUSION The overall outcomes of GIST can be predicted by risk-categorization. Surgery alone may not be a curative treatment for GIST. Response to targeted therapy is a crucial survival determinant in these patients.
Collapse
Affiliation(s)
- Kittima Pornsuksiri
- Kittima Pornsuksiri, Siripong Chewatanakornkul, Walawee Chaiyapan, Surasak Sangkhathat, Department of Surgery and Tumor Biology Research Unit, Faculty of Medicine, Prince of Songkla University, Hat Yai, Songkhla 90110, Thailand
| | | | | | | | | | | |
Collapse
|
|
48
|
McCarter MD, Antonescu CR, Ballman KV, Maki RG, Pisters PWT, Demetri GD, Blanke CD, von Mehren M, Brennan MF, McCall L, Ota DM, DeMatteo RP. Microscopically positive margins for primary gastrointestinal stromal tumors: analysis of risk factors and tumor recurrence. J Am Coll Surg 2012; 215:53-9; discussion 59-60. [PMID: 22726733 DOI: 10.1016/j.jamcollsurg.2012.05.008] [Citation(s) in RCA: 131] [Impact Index Per Article: 10.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/10/2012] [Revised: 05/08/2012] [Accepted: 05/08/2012] [Indexed: 12/15/2022]
Abstract
BACKGROUND Little is known about the outcomes of patients with microscopically positive (R1) resections for primary gastrointestinal stromal tumors (GIST) because existing retrospective series contain small numbers of patients. The objective of this study was to analyze factors associated with R1 resection and assess the risk of recurrence with and without imatinib. STUDY DESIGN We reviewed operative and pathology reports for 819 patients undergoing resection of primary GIST from the North American branch of the American College of Surgeons Oncology Group (ACOSOG) Z9000 and Z9001 clinical trials at 230 institutions testing adjuvant imatinib after resection of primary GIST. Patient, tumor, operative characteristics, factors associated with R1 resections, and disease status were analyzed. RESULTS Seventy-two (8.8%) patients had an R1 resection and were followed for a median of 49 months. Factors associated with R1 resection included tumor size (≥ 10 cm), location (rectum), and tumor rupture. The risk of disease recurrence in R1 patients was driven largely by the presence of tumor rupture. There was no significant difference in recurrence-free survival for patients undergoing an R1 vs R0 resection of GIST with (hazard ratio [HR] 1.095, 95% CI 0.66, 1.82, p = 0.73) or without (HR 1.51, 95% CI 0.76, 2.99, p = 0.24) adjuvant imatinib. CONCLUSIONS Approximately 9% of 819 GIST patients had an R1 resection. Significant factors associated with R1 resection include tumor size ≥ 10 cm, location, and rupture. The difference in recurrence-free survival with or without imatinib therapy in those undergoing an R1 vs R0 resection was not statistically significant at a median follow-up of 4 years.
Collapse
Affiliation(s)
- Martin D McCarter
- Department of Surgery, University of Colorado Denver School of Medicine, Aurora, CO 80045, USA.
| | | | | | | | | | | | | | | | | | | | | | | |
Collapse
|
|
49
|
Abstract
Gastrointestinal stromal tumors (GISTs) of the colon and rectum are the most common mesenchymal tumors of the gastrointestinal tract. GISTs of the colon and rectum constitute ~5% of all cases. Although colorectal GISTs can be small and found incidentally, the majority appear to be high risk and carry a significant likelihood of recurrent and metastatic disease. Surgery remains the mainstay of treatment for primary disease. There is now considerable interest in GISTs because they can be treated effectively with targeted molecular therapies, specifically tyrosine kinase inhibitors (TKIs), such as imatinib mesylate and sunitinib malate. GISTs are best treated by a multidisciplinary team comprised of the surgeon, medical oncologist, pathologist, and radiologist in the initial evaluation, management, and in continued follow-up. Increasing the number of resectable cases through pharmacologic debulking, optimizing the timing of surgery and organ preservation, reducing recurrence and surgical morbidity, prolonging survival, and possibly enhancing response to imatinib through surgical cytoreduction are all potential benefits of multidisciplinary management.
Collapse
|
|
50
|
Johnston FM, Kneuertz PJ, Cameron JL, Sanford D, Fisher S, Turley R, Groeschl R, Hyder O, Kooby DA, Blazer D, Choti MA, Wolfgang CL, Gamblin TC, Hawkins WG, Maithel SK, Pawlik TM. Presentation and management of gastrointestinal stromal tumors of the duodenum: a multi-institutional analysis. Ann Surg Oncol 2012; 19:3351-60. [PMID: 22878613 DOI: 10.1245/s10434-012-2551-8] [Citation(s) in RCA: 61] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/04/2012] [Indexed: 12/22/2022]
Abstract
BACKGROUND Duodenal gastrointestinal stromal tumors (GISTs) are a small subset of GISTs, and their management is poorly defined. We evaluated surgical management and outcomes of patients with duodenal GISTs treated with pancreaticoduodenectomy (PD) versus local resection (LR) and defined factors associated with prognosis. METHODS Between January 1994 and January 2011, 96 patients with duodenal GISTs were identified from five major surgical centers. Perioperative and long-term outcomes were compared based on surgical approach (PD vs LR). RESULTS A total of 58 patients (60.4%) underwent LR, while 38 (39.6%) underwent PD. Patients presented with gross bleeding (n = 25; 26.0%), pain (n = 23; 24.0%), occult bleeding (n = 19; 19.8%), or obstruction (n = 3; 3.1%). GIST lesions were located in first (n = 8, 8.4%), second (n = 47; 49%), or third/fourth (n = 41; 42.7%) portion of duodenum. Most patients (n = 86; 89.6%) had negative surgical margins (R0) (PD, 92.1 vs LR, 87.9%) (P = 0.34). Median length of stay was longer for PD (11 days) versus LR (7 days) (P = 0.001). PD also had more complications (PD, 57.9 vs LR, 29.3%) (P = 0.005). The 1-, 2-, and 3-year actuarial recurrence-free survival was 94.2, 82.3, and 67.3%, respectively. Factors associated with a worse recurrence-free survival included tumor size [hazard ratio (HR) = 1.09], mitotic count >10 mitosis/50 HPF (HR = 6.89), AJCC stage III disease (HR = 4.85), and NIH high risk classification (HR = 4.31) (all P < 0.05). The 1-, 3-, and 5-year actuarial survival was 98.3, 87.4, and 82.0%, respectively. PD versus LR was not associated with overall survival. CONCLUSIONS Recurrence of duodenal GIST is dependent on tumor biology rather than surgical approach. PD was associated with longer hospital stays and higher risk of perioperative complications. When feasible, LR is appropriate for duodenal GIST and PD should be reserved for lesions not amenable to LR.
Collapse
Affiliation(s)
- Fabian M Johnston
- Department of Surgery, The Johns Hopkins University, Baltimore, MD, USA
| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |
Collapse
|