Financial difficulties experienced by patients with gastrointestinal stromal tumours (GIST) in the Netherlands: data from a cross-sectional multicentre study.

PURPOSE: This study aims to (1) explore the prevalence of patient-reported financial difficulties among GIST patients, differentiating between those currently undergoing tyrosine kinase inhibitor (TKI) treatment and those who are not; (2) investigate associations between financial difficulties and sociodemographic and clinical characteristics, work, cancer-related concerns, anxiety and depression and (3) study the impact of financial difficulties on health-related quality of life. METHODS: A cross-sectional study was conducted among Dutch GIST patients diagnosed between 2008 and 2018, who were invited to complete a one-time survey between September 2020 and June 2021. Patients completed nine items of the EORTC item bank regarding financial difficulties, seven work-related questions, the Hospital Anxiety and Depression Scale, Cancer Worry Scale and EORTC QLQ-C30. RESULTS: In total, 328 GIST patients participated (response rate 63.0%), of which 110 (33.8%) were on TKI treatment. Patients currently treated with TKIs reported significantly more financial difficulties compared to patients not on TKIs (17.3% vs 8.7%, p = 0.03). The odds of experiencing financial difficulties was 18.9 (95% CI 1.7-214.7, p = 0.02) times higher in patients who were less able to work due to their GIST diagnosis. Patients who experienced financial difficulties had significantly lower global quality of life and functioning, and more frequently reported psychological symptoms as compared to patients who did not report financial difficulties. CONCLUSION: Even in a country where the costs of TKIs and follow-up care are covered by health insurance, financial difficulties can be present in GIST patients, especially in patients on TKI treatment, and may negatively influence the quality of life.

Clinicopathological features, risk profile assessment, and the surgical outcome of gastrointestinal stromal tumors in Lagos, Nigeria.

Background: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. GISTs originate from the interstitial cells of Cajal and are most commonly found in the stomach. Most available reports on GISTs in the Sub-Sahara Africa were in case reports and case series. Aim: To report our local experience and challenges in the management of GISTs in 33 patients in Lagos, Nigeria. Methodology: This is a descriptive study of adult patients of 16 years and above managed for GISTs at the Lagos University Teaching Hospital and some Lagos private hospital facilities between January 2015 and March 2021. Information on the patients' demographic characteristics, clinicopathological features, surgery performed, and postoperative complications were retrieved from the hospital's medical records for analysis. Data analysis was carried out using IBM SPSS Statistics for Windows, Version 23.0., Armonk, NY, USA: IBM Corp. Results: Thirty-three patients comprising 19 males and 14 females with a male: female ratio of 1.4:1 were included in the study. The mean age at presentation was 52.5 years. Abdominal pain (69.7%) and anemic symptoms (45.4%) were the principal modes of presentation. Abdominal computed tomography (CT) scan revealed stomach as the primary source of GISTs in 75.8% of patients. Forty-five percent of the patients had CT features of local organ invasion and 27.2% had features of metastasis. Surgical resection was feasible in 28 (84.8%) patients. Postoperative mortality was recorded in two patients with recurrent GISTs. Histological cell types were spindle cell (57.6%), mixed spindle and epithelioid (24.2%), and epithelioid (18.2%). Joensuu high-risk tumors (64. 3%) were the most prevalent in our series. Conclusion: Advanced-stage disease and features of anemia were hallmarks of GISTs among patients in this series. Surgical resection of GIST may be possible in some cases of advanced disease. Spindle cell types and high-risk GISTs were the most common pathological varieties in our patients.

Résumé Contexte: Les tumeurs stromales gastro-intestinales (GIST) sont les tumeurs mésenchymateuses les plus courantes du tractus gastro-intestinal. Les GIST proviennent des cellules interstitielles de Cajal et se trouvent le plus souvent dans l'estomac. La plupart des rapports disponibles sur les GIST en Afrique subsaharienne étaient des rapports de cas et des séries de cas. Objectif: rendre compte de notre expérience locale et des défis dans la gestion des GIST chez 33 patients à Lagos, au Nigeria. Méthodologie: Il s'agit d'une étude descriptive de patients adultes de 16 ans et plus pris en charge pour des GIST à l'hôpital universitaire de Lagos et dans certains établissements hospitaliers privés de Lagos entre janvier 2015 et mars 2021. Informations sur les caractéristiques démographiques des patients, les caractéristiques clinicopathologiques, la chirurgie effectuée, et les complications postopératoires ont été extraites des dossiers médicaux de l'hôpital pour analyse. L'analyse des données a été effectuée à l'aide d'IBM SPSS Statistics pour Windows, version 23.0., Armonk, NY, États-Unis: IBM Corp. l'étude. L'âge moyen à la présentation était de 52,5 ans. Les douleurs abdominales (69,7 %) et les symptômes anémiques (45,4 %) étaient les principaux modes de présentation. La tomodensitométrie abdominale (TDM) a révélé que l'estomac était la principale source de GIST chez 75,8 % des patients. Quarante-cinq pour cent des patients présentaient des caractéristiques CT d'invasion d'organes locaux et 27,2 % présentaient des caractéristiques de métastases. La résection chirurgicale était réalisable chez 28 (84,8 %) patients. La mortalité postopératoire a été enregistrée chez deux patients avec des GIST récurrents. Les types de cellules histologiques étaient les cellules fusiformes (57,6 %), les cellules mixtes fusiformes et épithélioïdes (24,2 %) et les épithélioïdes (18,2 %). Les tumeurs à haut risque de Joensuu (64,3 %) étaient les plus répandues dans notre série. Conclusion: La maladie à un stade avancé et les caractéristiques de l'anémie étaient les caractéristiques des GIST chez les patients de cette série. La résection chirurgicale des GIST peut être possible dans certains cas de maladie avancée. Les types de cellules fusiformes et les GIST à haut risque étaient les variétés pathologiques les plus fréquentes chez nos patients. Mots clés: Bilan, tumeurs stromales gastro-intestinales, pathologie, risque, gestes chirurgicaux.

Changing Disparity of Gastric Cancer Incidence by Histological Types in US Race-Specific Populations.

BACKGROUND: The incidence pattern of gastric cancer by histological types across major race/ethnic groups is unknown. METHODS: Age-standardized rates from 1992-2016 by race/ethnicity were calculated using data from Surveillance, Epidemiology, and End Results Program (SEER). Annual percent changes (APCs) in rates and corresponding 95% confidence intervals (CIs) were calculated and pairwise comparison of rates between race/ethnic groups was performed using the Joinpoint Regression Program. Calendar periods of incidence rates of gastric cardia and non-cardia cancer by histological types across race/ethnicity groups were shown by figures. RESULTS: The White population has the highest incidence of gastric cardia adenocarcinoma and the incidence is keeping constant from 1992 through 2016 except the decreasing in the Asian population (AAPC = -1.4, 95%CI (-2.1, -0.8)). Although the incidence of non-cardia adenocarcinoma is decreasing in each group, the descending trend in the Asian population is the quickest (AAPC = -3.8, 95%CI (-4.0, -3.5)). Gastric carcinoids were observed to have statistically significant increasing trends in all race/ethnicity groups, especially in Hispanic women from 0.4 per 100,000 to 1.6 per 100,000 persons. The incidence of gastrointestinal stromal tumors (GISTs) is rising, with Non-Hispanic blacks having the highest incidence. CONCLUSION: This study demonstrated disparities in the incidence of gastric cancer by histological types among different race/ethnic groups. Further investigations are warranted to understand the changing incidence patterns by race/ethnicity.

Caregivers of patients receiving long-term treatment with a tyrosine kinase inhibitor (TKI) for gastrointestinal stromal tumour (GIST): a cross-sectional assessment of their distress and burden.

BACKGROUND: TKIs are a long-term treatment for GIST, and may have an impact on caregivers. MATERIAL AND METHODS: For this cross-sectional study, patients and caregivers were both included when patients had been treated with TKIs for at least six months. Caregivers completed questionnaires including demographics, distress (Hospital Anxiety and Depression scale), burden (Self-Perceived Pressure from Informal Care) general health (RAND-36), comorbidity (Self-administered Comorbidity Questionnaire), social support (Social Support List - Discrepancies) and marital satisfaction (Maudsley Marital Questionnaire). Patients completed similar questionnaires, without 'burden'. We conducted analyses to explore differences between caregivers with low/moderate versus high levels of burden and low versus high levels of distress. RESULTS: Sixty-one out of seventy-one eligible couples (84%) were included in the analysis. The median age of the caregivers was 60 years; 66% were female and 78% were the patients' spouse. The median age of the patients was 66 years; 43% were female. Caregivers experienced high levels of burden and distress in 10% and 23%, respectively. Caregivers with high levels of burden perceived significantly lower mental health, less vitality, lower general health and high levels of distress. Significantly higher levels of burden were found in non-spouses, caregivers of patients with more treatment-related side-effects, caregivers who spent more hours caring, and those caring for more than one person. For distress, caregivers with high levels of distress perceived significantly more burden, lower social functioning, more role physical and emotional problems, lower mental health, less vitality and lower general health. Furthermore, high levels of distress were found in caregivers of more dependent patients and those caring for more than one person. CONCLUSIONS: Caregivers of the patients with GIST treated with TKI are managing well. There is a small, vulnerable group of caregivers with high levels of burden and/or distress, show more health-related problems, both physical and mental, and require adequate support.

Gastrointestinal stromal tumors: treatment patterns of a population-based sample.

OBJECTIVES: The National Cancer Institute (NCI) annually confirms therapy with treating physicians on a sample of patients diagnosed with a specific cancer. METHODS: Using the NCI Patterns of Care data, treatment patterns were examined on a population-based sample of patients diagnosed with gastrointestinal stromal tumors (GIST) in 2008. RESULTS: A random sample of 323 of 405 GIST patients registered in SEER was selected. Most patients had gastric GISTs, were ≥ 65 years, white, had private insurance, and treated in a hospital with a residency program. Surgery was primarily performed in patients with non-metastatic disease (94%), in which: 26, 12, and 36% were at low, intermediate, and high-risk of recurrence, respectively. Amongst low-risk patients, ∼ 30% received adjuvant therapy. Amongst patients at higher risk, 26-40% did not receive adjuvant therapy. Imatinib was the most common targeted therapy administered. On multivariate analysis, age and risk-group were associated with receipt of adjuvant targeted therapy. CONCLUSIONS: Our study shows that in 2008, the majority of patients diagnosed with GIST received appropriate surgical and adjuvant therapies. However, a considerable subset may have been overtreated and undertreated. Future studies identifying factors that impact the delivery of adjuvant therapy should be conducted.

Correlation between mutational status and survival and second cancer risk assessment in patients with gastrointestinal stromal tumors: a population-based study.

BACKGROUND: Gastrointestinal stromal tumors are sarcomas of the digestive tract characterized by mutations mainly located in the c-KIT or in the platelet-derived growth factor receptor (PDGFR)-alpha genes. Mutations in the BRAF gene have also been described. Our purpose is to define the distribution of c-KIT, PDGFR and BRAF mutations in a population-based cohort of gastrointestinal stromal tumors (GIST) patients and correlate them with anatomical site, risk classification and survival. In addition, as most of the GIST patients have a long survival, second cancers are frequently diagnosed in them. We performed a second primary cancer risk assessment. METHODS: Our analysis was based on data from Tarragona and Girona Cancer Registries. We identified all GIST diagnosed from 1996 to 2006 and performed a mutational analysis of those in which paraffin-embedded tissue was obtained. Observed (OS) and relative survival (RS) were calculated according to risk classifications and mutational status. Multivariate analysis of variables for observed survival and was also done. RESULTS: A total of 132 GIST cases were found and we analyzed mutations in 108 cases. We obtained 53.7% of mutations in exon 11 and 7.4% in exon 9 of c-KIT gene; 12% in exon 18 and 1.9% in exon 12 of PDGFR gene and 25% of cases were wild type GIST. Patients with mutations in exon 11 of the c-KIT gene had a 5-year OS and RS of 59.6% and 66.3%, respectively. Patients with mutations in exon 18 of the PDGFR gene had a 5-year OS and RS of 84.6% and 89.7%. In multivariate analysis, only age and risk group achieved statistical significance for observed survival. GIST patients had an increased risk of second cancer with a hazard ratio of 2.47. CONCLUSIONS: This population-based study shows a spectrum of mutations in the c-KIT and PDGFR genes in GIST patients similar to that previously published. The OS and RS of GIST with the exon 18 PDGFR gene mutation could indicate that this subgroup of patients may be less aggressive and have a good prognosis, although less sensitive to treatment at recurrence. In our study, GIST patients have an increased risk of developing a second neoplasm.

Practical aspects of risk assessment in gastrointestinal stromal tumors.

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.

Cost-effectiveness of 3-years of adjuvant imatinib in gastrointestinal stromal tumors (GIST) in the United States.

BACKGROUND: Recent clinical trial data have demonstrated that 3 years vs 1 year of adjuvant imatinib therapy for patients with surgically resected Kit+ Gastrointestinal Stromal Tumors (GIST) leads to a significant improvement in recurrence-free survival and overall survival. This study assesses the cost-effectiveness of treating patients with 3 years vs 1 year of imatinib from a US payer's perspective. METHODS: A Markov model was developed to predict GIST recurrence and treatment costs. Patients enter the model after surgery and transition among three health states: free of recurrence, recurrence, and death. Recurrence, mortality, costs, and utilities were derived from clinical trial and published literature. Expected costs and quality-adjusted life years (QALYs) were estimated and discounted at 3%/year. Deterministic and probabilistic sensitivity analyses were conducted. RESULTS: Patients receiving 3 years of imatinib had higher QALYs (8.53 vs 7.18) than those receiving 1 year of imatinib. Total lifetime per-patient cost was $302,100 for 3 years vs $217,800 for 1 year of imatinib. Incremental cost effectiveness ratio of 3 years vs 1 year of imatinib was $62,600/QALY. Model results were sensitive to long-term rate of GIST recurrence (beyond 5 years) and cost of imatinib. At a threshold of $100,000/QALY, 3 years vs 1 year of imatinib was cost-effective in 100% of simulations. LIMITATIONS: The model is a simplified representation of disease natural history and may not account for all possible health states and complications associated with disease. Resource utilization on treatment was estimated using the resource use data from previous trials, therefore calculated medical costs might be over-estimated compared to the real-world setting. CONCLUSIONS: Model results suggest that treatment with 3 years vs 1 year of imatinib is cost-effective at a $100,000/QALY threshold. Clinical and economic results suggest treating surgically resected Kit+ GIST patients with 3 years of imatinib would result in improved quality-adjusted survival.

[Are sarcoma centers needed in Germany? Experience gained with the Bonner GIST register]. / Brauchen wir in Deutschland Sarkomzentren? Erfahrungen aus dem Bonner GIST-Register.

Due to their rarity and multiple subtypes, there is scant experience with sarcomas. Any effective targeted therapy depends on precise diagnosis of the tumor group using molecular markers and, increasingly, mutation testing. The necessary histopathological expertise and molecular diagnostic tools are usually only found at specialized centers. Using the Bonner GIST register as an example, the advantages of this kind of register from a diagnostic and therapeutic perspective will be discussed. Material submitted for gastrointestinal stromal tumors and other mesenchymal tumors, as well as the supervision of pathological referencing for national and international studies have made accurate diagnosis and appropriate therapy strategies ever more possible. The introduction of epidemiological as well as interdisciplinary sarcoma registers is a prerequisite for the improvement of sarcoma diagnostics and therapy.

Prevalence and management of gastrointestinal stromal tumors.

The prevalence and characteristics of patients with confirmed gastrointestinal stromal tumor (GIST) in a community hospital over a 6-year period are described. Our objective was to communicate our experience managing this rare tumor of the gastrointestinal tract. A retrospective chart review was performed. Patients were selected based on International Classification of Diseases, 9th Revision codes in correlation with their respective confirmational pathology. Patients with a diagnosis of GIST, cells of Cajal tumor, and/or different varieties of gastrointestinal sarcoma were included in this study. These tumors had to have a positive C-kit on immunohistochemistry. Demographic and clinical data were collected from medical records as well as pathology reports. Follow up from attendings' office records and telephone interviews complemented our data. A total of 61 patients was identified in our institution (averaging 10 patients per year). Females represented 63 per cent of our series. The average ages were 70.2 +/- 19.1 years for females and 59.4 +/- 13.5 years for males (P < 0.01). The most common clinical presentation was an intra-abdominal nonobstructing mass followed by an endoscopically detected mass or incidental tumors found during unrelated surgery. Surgical emergencies such as acute abdomen and gastrointestinal bleed were rare. Over half of these tumors were located in the stomach. Other sites were the small intestine, colon, esophagus, and rectal-vaginal septum. Opened surgical resection was performed in two-thirds of treated cases, whereas laparoscopic resection was done in the remainder. Only 18 per cent of these tumors were considered benign, whereas 35 per cent were considered to have some malignant potential and 47 per cent were of undetermined potential. In surgically resected tumors, we found a 42 per cent recurrence rate with a median average time of recurrence of 22 months. Pathologic grading and type of surgery were not predictors of rate and timing of recurrence. However, the disease tended to be more aggressive in white males and age older than 70 years. Imatinib was used mainly in attempts to downstage, control recurrent disease, and make surgery possible. With the improvement of immunohistochemical techniques, the diagnosis of GIST is increasing. Preoperative diagnosis is highly uncertain and dependent on clinical suspicion. Surgical resection is still the main form of curative therapy. Our experience is similar to large-volume centers. GIST, once recognized, can be treated in community hospitals without compromise of their care.

The epidemiologic, health-related quality of life, and economic burden of gastrointestinal stromal tumours.

BACKGROUND AND OBJECTIVES: Gastrointestinal stromal tumours (GIST) are uncommon tumours believed to arise from interstitial cells of Cajal or their precursors in the gastrointestinal (GI) tract, accounting for a small percentage of GI neoplasms and sarcomas. Given the recent recognition of GIST as a distinct cancer, as well as new treatment options available today, a review of the epidemiologic, health-related quality of life (HRQL), and economic burden of GIST is timely from a payer, provider and patient perspective and may provide guidance for treatment decision making and reimbursement. METHODS: A systematic literature review of PubMed and five scientific meeting databases, was conducted to identify published studies and abstracts describing the epidemiologic, HRQL and economic impact of GIST. Publications deemed worthy of further review, based on the information available in the abstract, were retrieved in full text. RESULTS AND DISCUSSION: Thirty-four publications met the review criteria: 29 provided data on GIST epidemiology, one provided cost data, three reported HRQL outcomes, and one reported cost and HRQL outcomes. The annual incidence of GIST (cases per million) ranged from 6.8 in the USA to 14.5 in Sweden, with an estimated 5-year survival rate of 45-64%. On the Functional Illness of Chronic Therapy-fatigue instrument, GIST patients scored 40.0 compared with 37.6 in anaemic cancer patients (0 = worst; 52 = least fatigue). Total costs over 10 years for managing GIST patients with molecularly targeted treatment was estimated at pounds 47 521- pounds 56 146 per patient compared with pounds 4047- pounds 4230 per patient with best supportive care. CONCLUSIONS: The incidence of GIST appears to be similar by country; the lower estimate in one country could be explained by differences in method of case ascertainment. Data suggest that the HRQL burden of GIST is similar to that with other cancers although this requires further exploration. The value of new therapies in GIST needs to consider not only cost but also anticipated benefits and the unmet medical need in this condition.

Adult sarcomas: a missed opportunity for sustained cooperative group translational and clinical research.

Preclinical advances offer an opportunity to further reduce morbidity and mortality from sarcomas over the next decade. Since no single institution or North American cooperative oncology group has the expertise or patient resources for histology-specific clinical and translational research on adult sarcomas, efforts have been made to develop funding from the National Cancer Institute (NCI). One such initiative was the Intergroup Coalition Against Sarcomas (ICAS), which, building upon the strengths of the multimodality cooperative oncology groups, provided an infrastructure for broad participation by investigators from all treatment disciplines in protocol development and patient entry. However, despite an excellent evaluation in formal peer review, the Division of Cancer Treatment of NCI has ended this initiative claiming insufficient available funds--to the detriment of adult sarcoma patients now and in the future.

[Current incidence and management of gastrointestinal stromal tumors].

Gastrointestinal stromal tumor (GISTs) are a new recognized tumor entity. The incidence increases with time for the greater awareness of GISTs. Latest reports of the epidemiology of GISTs have been collected and sorted. Besides, it is still a controversy on the management of GISTs. While surgical en-bloc resection is the treatment of choice, imatinib is still optimal for patients with recurrent or metastatic GISTs. Preliminary promising effects have been showed on neo-adjuvant and post-operational adjuvant therapies. Imatinib-resistant GISTs become common and more targeted medication have been investigated and tested.