Clinical features and prognosis of malignant small bowel tumors: Experience from a university hospital in Chile / Características clínicas y pronóstico de los tumores malignos de intestino delgado: experiencia en un hospital universitario de Chile

Background Small bowel tumors (SBT) are infrequent and represent a small proportion of digestive neoplasms. There is scarce information about SBT in Latin America.AimTo describe the epidemiology, clinical characteristics, diagnostic methods, and survival of malignant SBTs.MethodsRetrospective observational study of adult patients with histopathological diagnosis of SBT between 2007 and 2021 in a university hospital in Chile.ResultsA total of 104 patients [51.9% men; mean age 57 years] with SBT. Histological type: neuroendocrine tumor (NET) (43.7%, n=38), gastrointestinal stromal tumors (GIST) (21.8%, n=19), lymphoma (17.2%, n=15) and adenocarcinoma (AC) (11.5%, n=10). GIST was more frequent in duodenum (50%; n=12) and NET in the ileum (65.8%; n=25). Metastasis was observed in 17 cases, most commonly from colon and melanoma. Nausea and vomiting were significantly more often observed in AC (p=0.035), as well as gastrointestinal bleeding in GIST (p=0.007). The most common diagnostic tools were CT and CT enteroclysis with an elevated diagnostic yield (86% and 94% respectively). The 5-year survival of GIST, NET, lymphoma and AC were 94.7% (95%CI: 68.1–99.2), 82.2% (95%CI: 57.6–93.3), 40.0% (95%CI: 16.5–82.8) and 25.9% (95%CI: 4.5–55.7%), respectively. NET (HR 6.1; 95%CI: 2.1–17.2) and GIST (HR 24.4; 95%CI: 3.0–19.8) were independently associated with higher survival compared to AC, adjusted for age and sex.ConclusionsMalignant SBT are rare conditions and NETs are the most common histological subtype. Clinical presentation at diagnosis, location or complications may suggest a more probable diagnosis. GIST and NET are associated with better survival compared to other malignant subtypes. (AU)

Introducción Los tumores del intestino delgado (TID) son infrecuentes y la información sobre ellos es escasa en Latinoamérica.ObjetivoDescribir la epidemiología, características clínicas, métodos diagnósticos y supervivencia de los TID malignos.MétodosEstudio observacional retrospectivo de pacientes adultos con diagnóstico histopatológico de TID entre 2007-2021 en un hospital universitario de Chile.ResultadosSe observaron 104 pacientes (51,9% hombres; edad media 57 años) con TID. El tipo histológico fue tumor neuroendocrino (TNE) (43,7%, n=38), tumor estromal gastrointestinal (GIST) (21,8%, n=19), linfoma (17,2%, n=15) y adenocarcinoma (AC) (11,5%, n=10). Los GIST fueron más frecuentes en el duodeno (50%; n=12) y los TNE en el íleon (65,8%; n=25). Hubo 17 casos de metástasis, más comúnmente de colon y melanoma. Las náuseas y los vómitos se observaron con mayor frecuencia en AC (p=0,035), así como el sangrado gastrointestinal en GIST (p=0,007). Las herramientas de valoración más comunes fueron TC y enteroclisis por TC con un rendimiento diagnóstico alto (86% y 94%, respectivamente). La supervivencia a cinco años de los GIST, TNE, linfoma y AC fue 94,7% (intervalo de confianza [IC] 95%: 68,1-99,2), 82,2% (IC 95%: 57,6-93,3), 40,0% (IC 95%: 16,5-82,8) y 25,9% (IC 95%: 4,5-55,7), respectivamente. Los TNE (hazard ratio [HR] 6,1; IC 95%: 2,1-17,2) y GIST (HR 24,4; IC 95%: 3,0-19,8) se asociaron de forma independiente con una mayor supervivencia en comparación con AC, ajustado por edad y sexo.ConclusionesLos TID malignos son enfermedades poco frecuentes y los TNE son el subtipo histológico más común. La presentación clínica en el momento del diagnóstico, localización o complicaciones pueden sugerir un dictamen más probable. Los GIST y TNE se asocian a una mejor supervivencia en comparación con otros subtipos malignos. (AU)

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.

Clinical features and prognosis of malignant small bowel tumors: Experience from a university hospital in Chile.

BACKGROUND: Small bowel tumors (SBT) are infrequent and represent a small proportion of digestive neoplasms. There is scarce information about SBT in Latin America. AIM: To describe the epidemiology, clinical characteristics, diagnostic methods, and survival of malignant SBTs. METHODS: Retrospective observational study of adult patients with histopathological diagnosis of SBT between 2007 and 2021 in a university hospital in Chile. RESULTS: A total of 104 patients [51.9% men; mean age 57 years] with SBT. Histological type: neuroendocrine tumor (NET) (43.7%, n=38), gastrointestinal stromal tumors (GIST) (21.8%, n=19), lymphoma (17.2%, n=15) and adenocarcinoma (AC) (11.5%, n=10). GIST was more frequent in duodenum (50%; n=12) and NET in the ileum (65.8%; n=25). Metastasis was observed in 17 cases, most commonly from colon and melanoma. Nausea and vomiting were significantly more often observed in AC (p=0.035), as well as gastrointestinal bleeding in GIST (p=0.007). The most common diagnostic tools were CT and CT enteroclysis with an elevated diagnostic yield (86% and 94% respectively). The 5-year survival of GIST, NET, lymphoma and AC were 94.7% (95%CI: 68.1-99.2), 82.2% (95%CI: 57.6-93.3), 40.0% (95%CI: 16.5-82.8) and 25.9% (95%CI: 4.5-55.7%), respectively. NET (HR 6.1; 95%CI: 2.1-17.2) and GIST (HR 24.4; 95%CI: 3.0-19.8) were independently associated with higher survival compared to AC, adjusted for age and sex. CONCLUSIONS: Malignant SBT are rare conditions and NETs are the most common histological subtype. Clinical presentation at diagnosis, location or complications may suggest a more probable diagnosis. GIST and NET are associated with better survival compared to other malignant subtypes.

Gastrointestinal stromal tumor in North Africa and the middle east: updates in presentation and management from an 11-year retrospective cohort.

OBJECTIVES: This study described the epidemiological, clinical, and survival profiles of patients with gastrointestinal stromal tumor (GIST) in North Africa and the Middle East (AfME). METHODS: This regional, multicenter, observational, retrospective study collected 11-year data on demographics, medical history, disease characteristics, current treatment approaches of GIST, the safety of the most common tyrosine kinase inhibitors (TKIs), second cancers, and survival status. RESULTS: Data of 201 eligible patients were analyzed: mean age was 56.9 ± 12.6 years; 111 (55.2%) patients were men, 21 (10.4%) patients had previous personal malignancy. The most common clinical presentation of GIST was dysphagia [92 (45.8%) patients]. The stomach was the most common primary site in 120 (60.7%) patients, 171 (85.1%) patients had localized disease at diagnosis. 198 (98.5%) GIST cases were CD117/CD34-positive. Imatinib was used in the neoadjuvant (18/21 patients), adjuvant (85/89 patients), and first-line metastatic treatment (28/33 patients) settings. The most common non-hematological toxicity associated with TKIs was vomiting in 32/85 (37.6%) patients. Overall, 100 (49.8%) patients (95%CI: 42.8-56.7%) were alive and disease-free while 30 (14.9%) patients were alive with active disease. CONCLUSION: Presentation of GIST in our AfME population is consistent with global reports, being more frequent in patients >50 years old and having the stomach as the most common primary site. Unlike what is usually reported, though, we did have more patients with lymphatic spread of the disease. Despite the global trend and advances in the treatment of GIST according to molecular profile, this is still far to happen in our population given the lack of access to molecular profiles and the high associated cost.

Hallmarks and novel insights for gastrointestinal stromal tumors: A bibliometric analysis.

BACKGROUND: Due to the increasing recognition of gastrointestinal stromal tumor (GIST), novel insights have appeared in both preclinical and clinical research and begun to reshape the field. This study aims to map the research landscape through bibliometric analysis and provide a brief overview for the future of the GIST field. METHODS: We searched the Web of Science Core Collection without publication data restrictions for GISTs and performed a bibliometric analysis with CiteSpace and VOSviewer software. RESULTS: In sum, 5,911 of 13,776 records were included, and these studies were published in 948 journals and written by 24,965 authors from 4,633 institutions in 100 countries. Referring to published reviews and bibliometric analysis, we classified the future trends in four groups. In epidemiological study, precise incidence and clinicopathological features in different regions and races might become potential hotspots. Novel therapy, such as drugs, modified strategies, radioligand therapy, was persistent hotspots in GIST fields, and ctDNA-guided diagnosis, monitoring, and treatment might meet future clinical needs. The debate over serosa surgery vs. mucosa surgery will remain active for a long time in GIST surgery, and function reserve surgery, biology-based surgery will play an important role in future. Moreover, rare GIST type, like NF-1-associated GIST, Carney triads and SDH mutant GIST, need more studies in pathogenesis and genetic mutation to provide appropriate treatment for this orphan GIST patients. CONCLUSIONS: Potential hotspots in future GIST trends might involve epidemiology, agents, resection therapy and rare type GIST, moreover, researchers could pay more attention in these four fields.

Synchronous and metachronous neoplasms of different histogenesis with gastrointestinal stromal tumor (GIST): 10 years experience of a single institution.

AIM: Gastrointestinal stromal tumors (GISTs) are the most common primary mesenchymal neoplasms of the gastrointestinal tract. Significant advances have been made in its pathogenesis, diagnosis, and treatment over the past few decades. However, little is known about the occurrence of synchronous or methacronous tumors with other histogenesis in addition to GISTs. The aim of this study was to present a series of 15 patients diagnosed with a second primary neoplasm in addition to GIST. MATERIAL AND METHODS: Patients who were diagnosed with both GIST and other primary neoplasm between January 2010 and December 2019 were included in the study. Demographic, clinicopathologic and immunohistochemical parameters of the patients were analyzed along with the follow-up results RESULTS: This study included 12 men and 3 women with a median age of 68 years (range: 57-83 years). Of the GISTs, 93.3% were localized in the stomach and 73.3% were at very low / low risk category. Of the second primary tumors, 66.6% were in the gastrointestinal tract. Detection of the GIST was synchronous in 9 cases, metachronous in 2 cases and preceded the GIST diagnosis in 4 cases. GIST was incidentally found intra-operatively in 3 of the cases. The mean size of the synchronous GISTs was 20 mm while the most common GIST-associated malignancy was gastric adenocarcinoma. The median follow-up times was 62 months (range: 13-129 months). CONCLUSIONS: The prevalence of secondary malignancies in GIST patients is significantly higher than the healthy population. The high occurrence rate of additional primary tumors in GIST patients has focused the attention of surgeons on this problem. While it is not yet clear if there is a causal association or a common genetic mechanism for the concomitant occurrence of GIST with other malignancies, a closer surveillance of GIST patients is needed due to their proved increased prevalence of a second primary tumor especially during the first year after diagnosis. KEY WORDS: Gastrointestinal stromal tumor, Coexistence, Synchronous malignancy, Second neoplasm, Gastric adenocarcinoma.

[Gastrointestinal stromal tumors: a retrospective study]. / Tumeurs stromales gastro-intestinales: une étude rétrospective.

Introduction: although rare, gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the digestive tract. The aim of this work is to study the clinical, paraclinical, therapeutic and evolutionary features of GIST. Methods: we conducted a descriptive retrospective, monocentric study (April 2017-April 2021) collecting data from the medical records of all patients with GIST treated at the medical oncology department of the University Hospital of Mostaganem. Results: we collected data from the medical records of 23 patients, with a median age of 54.4 years, sex ratio 1.8, over a period of 4 years. Abdominal pain was the most frequent symptom (78.3%, n=18); 47.8% of patients (n=11) had a tumor in the small bowel. The diagnosis was made at an early stage in 69.6% of cases (n=16). Surgical treatment was performed in 20 of the 23 patients, 18 of whom with R0. Of the 15 operated patients with a localized tumor, 13 received adjuvant medical treatment (Imatinib). Disease progression was reported in three patients treated with imatinib, then 2nd line therapy (Sunitinib) was started. During the study period, all patients were alive except two who died due to disease progression. Conclusion: the diagnosis of GIST is mainly based on histology and immunohistochemistry, which is often not performed by our pathologists. Molecular biology makes it possible to predict the prognosis and consequently adapt the therapies. The outcome of patients with GIST is often favorable but marked by recurrences despite a supposedly curative treatment requiring prolonged monitoring.

Update of epidemiology, survival and initial treatment in patients with gastrointestinal stromal tumour in the USA: a retrospective study based on SEER database.

OBJECTIVES: An updated epidemiological analysis of gastrointestinal stromal tumour (GIST), the change of cancer-specific survival (CSS) and patterns of initial treatment are of interest. DESIGN: A retrospective study using data from the Surveillance, Epidemiology and End Results (SEER) database. SETTING AND PARTICIPANTS: A total of 5625 patients with GIST diagnosed between 2010 and 2019 were identified. PRIMARY OUTCOME MEASURES: Age-standardised incidence rate (ASIR) and annual prevalence rate were calculated. SEER combined stage, period CSS rate and initial treatment were summarised. All the data were calculated by SEER*Stat software. RESULTS: From 2010 to 2019, the ASIR of GIST increased from 0.79 to 1.02 per 100 000 person-years, with an increase of 2.4% annually. The increase was across age and sex subgroups. The prevalence trend was similar with the ASIR trend in each subgroup. The stage distributions were similar between different age groups, but varied among different primary tumour sites. More importantly, a stage shift from regional stage to localized stage at diagnosis was found, which may result in the improvement of CSS over years. Overall, the 5-year CSS rate of GIST was approximately 81.3%. Even for metastatic GIST, the rate exceeded 50%. Surgery was the most common treatment regimen for GIST, followed by surgery and systemic treatment. Whereas approximately 7.0% patients were undertreated, which was more pronounced among patients with distant and unknown stages. CONCLUSIONS: The findings of this study suggest an improving early detection of GIST and an improving ability of accurate staging. Though most patients are effectively treated and perform good survivals, approximate 7.0% patients may be undertreated.

The association of genitourinary cancer among Saudi patients with gastrointestinal stromal tumors and a systematic literature review.

The gastrointestinal tract's most commonly occurring primary mesenchymal tumor is the gastrointestinal stromal tumor (GIST). However, few cases worldwide were reported associated with renal cell carcinoma (RCC). Therefore, we aimed to identify the association of genitourinary tumors in patients with GIST in our tertiary care hospital in Saudi Arabia and compare it to the literature. We identified all patients in the pathology department database with the diagnosis of GIST. We excluded duplicate and recurrent cases. We examined patients' files for the presence of RCC, adrenal tumors, or other genitourinary cancer. A systematic review of the association was conducted. From 2003 to 2020, 170 patients had a histopathologic diagnosis of primary GIST, 100 men and 70 women, median age of 57 (range 9-91) years at the time of diagnosis. The site of primary GIST was gastric 103, small bowel 43, mesenteric 5, omentum/peritoneum 7, abdomen 4, isolated adrenal 1, and other 7. Six patients had associated primary genitourinary cancer. Three patients had RCC (two clear cell RCC and one radiologic diagnosis only), and three had adrenal tumors (one adrenal carcinoma, one an isolated adrenal GIST, and one pheochromocytoma). In addition, two patients had a tumor invading the urinary bladder. Although the cohort included 63 men aged 60 or above (median 71 ± 8.7 years, range 60-94), none demonstrated clinical prostatic carcinoma. Data was compared to 69 systematic review articles. We report the rare association between GIST tumors and primary genitourinary cancer, mainly RCC and adrenal tumors. Also, we identified a secondary invasion of the urinary bladder. Unlike the reported series, none of the older male patients had clinical prostate cancer.

Gastrointestinal stromal tumors of the upper GI tract: population-based analysis of epidemiology, treatment and outcome based on data from the German Clinical Cancer Registry Group.

BACKGROUND: Gastrointestinal stromal tumors (GIST) are rare mesenchymal tumors. They are most frequently located in the stomach but are also found in the esophagus and the gastroesophageal junction (GEJ). Information regarding the prognostic factors associated with upper gastrointestinal GIST is still scarse. METHODS: In this study, datasets provided by the German Clinical Cancer Registry Group, including a total of 93,069 patients with malignant tumors in the upper GI tract (C15, C16) between 2000 and 2016 were analyzed to investigate clinical outcomes of GIST in the entire upper GI tract. RESULTS: We identified 1361 patients with GIST of the upper GI tract. Tumors were located in the esophagus in 37(2.7%) patients, at the GEJ in 70 (5.1%) patients, and in the stomach in 1254 (91.2%) patients. The incidence of GIST increased over time, reaching 5% of all UGI tumors in 2015. The median age was 69 years. The incidence of GIST was similar between males and females (53% vs 47%, respectively). However, the proportion of GIST in female patients increased continuously with advancing age, ranging from 34.7% (41-50 years) to 71.4% (91-100 years). Male patients were twice as likely to develop tumors in the esophagus and GEJ compared to females (3.4% vs. 1.9% and 6.7% vs. 3.4%, respectively). The median overall survival of upper gastrointestinal GIST was 129 months. The 1-year, 5-year, and 10-year OS was 93%, 79%, and 52% respectively. Nevertheless, tumors located in the esophagus and GEJ were associated with shorter OS compared to gastric GIST (130 vs. 111 months, p = 0.001). The incidence of documented distant metastasis increased with more proximal location of GIST (gastric vs. GEJ vs. esophagus: 13% vs. 16% vs. 27%) at presentation. CONCLUSION: GIST of the esophagus and GEJ are rare soft tissue sarcomas with increasing incidence in Germany. They are characterized by worse survival outcomes and increased risk of metastasis compared to gastric GIST.

Cardiovascular-Specific Mortality among Gastrointestinal Stromal Tumor Patients: A Population-Based Analysis.

Background: The overall risk of cardiovascular mortality (CVM) in cancer survivors has increased with time. The trend of CVM in patients with gastrointestinal stromal tumors (GISTs) remains unclear. This study is aimed at assessing the risks and independent predictors of CVM in GIST patients. Methods: Data of the GIST patients were extracted from the Surveillance, Epidemiology, and End Results (SEER) database (2000-2019). The standardized mortality ratio (SMR) was used to evaluate the risk of CVM, and a multivariate competing risk model was utilized to identify the predictors for CVM. Results: A total of 12,058 patients with GIST were included in this study, of whom 477 (4.0%) patients died of cardiovascular disease (CVD). The SMR for CVM among GIST patients was significantly higher than in the general population (SMR, 3.23, 95% CI: 2.97-3.52), and all categories of CVD were associated with a significantly elevated SMR. The cumulative mortality of CVD was the lowest among all causes of death, while the CVM was the second most common cause of death in patients ≥ 80 years when stratified by age at diagnosis. Furthermore, male sex, older age at diagnosis, White race, unmarried, earlier year of diagnosis, and not receiving chemotherapy were the poor prognostic factors for CVM. Conclusions: The CVM risk in GIST patients was significantly higher relative to the general US population. Timely screening and cardioprotective interventions should be implemented to prevent the occurrence of CVM in patients with GIST.

Clinicopathological and immunohistochemical characterization of gastrointestinal stromal tumour at four tertiary health centers in Nigeria using CD117, DOG1, and human epidermal growth factor receptor-2 biomarkers.

Aims: Gastrointestinal stromal tumors (GISTs) are neoplastic lesions that primarily affect the digestive tract and develop from interstitial cells of Cajal. These lesions require histopathological and immunohistochemical characterization due to their malignant potential and personalized treatment. In this investigation, the sex, age, lesional sites of origin, histopathological types, the prevalence of human epidermal growth factor receptors (HER-2) expression, prognostic indices (based on tumor size and mitotic figures), expression of CD117 and DOG1, and characteristics of patients with GIST were all characterized. Materials and Methods: This was a retrospective cross-sectional analysis of GIST cases seen at four tertiary health-care centers in Nigeria over a 10-year period (2008-2017) and investigated utilizing histopathological and immunohistochemical (CD117, DOG1, and HER-2) methods. Results: In this investigation, there were twenty GIST cases. Notably, the majority (40%) of the cases had tumors with sizes between 7.0 and 8.0 cm; the stomach was the most frequent site (70%) and the spindle cell type of GIST was the most prevalent (80%) histopathological type. In addition, the stomach was significantly associated with GIST as an origin site (with a P = 0.001), and 100% and 50% of these tumors were immunoreactive with CD117 and DOG1, respectively. Conclusions: In our study, GISTs most frequently develop in the stomach, and CD117 and DOG1 are essential for correctly diagnosing these tumors. However, HER-2 immunoreactivity is a predictive marker of survival for personalized care.

Résumé Objectifs: Les tumeurs stromales gastro-intestinales (GIST) sont des lésions néoplasiques qui affectent principalement le tube digestif et se développent à partir des cellules interstitielles de Cajal. Ces lésions nécessitent une caractérisation histopathologique et immunohistochimique en raison de leur potentiel malin et d'un traitement personnalisé. Dans cette enquête, le sexe, l'âge, les sites d'origine des lésions, les types histopathologiques, la prévalence de l'expression des récepteurs du facteur de croissance épidermique humain (HER-2), les indices pronostiques (basés sur la taille de la tumeur et les chiffres mitotiques), l'expression de CD117 et DOG1, et les caractéristiques des patients atteints de GIST ont toutes été caractérisées. Matériels et méthodes: Il s'agissait d'une analyse transversale rétrospective de cas de GIST observés dans quatre centres de soins de santé tertiaires au Nigeria sur une période de 10 ans (2008-2017) et étudiée à l'aide d'analyses histopathologiques et immunohistochimiques (CD117, DOG1 et HER). 2) méthodes. Résultats: Dans cette enquête, il y a eu vingt cas de GIST. Notamment, la majorité (40 %) des cas présentaient des tumeurs mesurant entre 7,0 et 8,0 cm ; l'estomac était le site le plus fréquent (70 %) et le type de GIST à cellules fusiformes était le type histopathologique le plus répandu (80 %). De plus, l'estomac était significativement associé au GIST comme site d'origine (avec un P = 0,001) et 100 % et 50 % de ces tumeurs étaient immunoréactives avec CD117 et DOG1, respectivement. Conclusions: Dans notre étude, les GIST se développent le plus souvent dans l'estomac, et CD117 et DOG1 sont essentiels pour diagnostiquer correctement ces tumeurs. Cependant, l'immunoréactivité HER-2 est un marqueur prédictif de survie pour une prise en charge personnalisée. Mots-clés: Biomarqueurs, tumeurs stromales gastro-intestinales, histopathologie, immunohistochimie.

Challenge of gastro-intestinal stromal tumor management in low-income countries: example of Benin.

BACKGROUND: GISTs are rare tumors but the most frequent mesenchymal tumors of the digestive tract. Diagnosis and treatment are challenging in low-income countries due to relatively poor access to immunohistochemistry and targeted therapy. In Africa, there are few studies about it. Imatinib, an oral targeted therapy, has been available in Benin since 2010 and free since 2016. This study describes the diagnosis and therapeutic management of GIST in Cotonou, Benin. METHODS: This is a descriptive cross-sectional study, with retrospective data collection over a 10-year period from 2010 to 2020, focused on patients with histological confirmed gastro-intestinal stromal tumor (GIST). Cases were identified using the registry database and the archival files of the Hubert Koutoukou Maga National University Hospital of Cotonou (CNHU-HKM). RESULTS: Fifteen GISTs were identified during the study period. The median age was 52 and the sex ratio was 2:1 (10 males and 5 females). The most frequent symptom was abdominal pain (n = 12). Delay in care seeking after onset of symptoms ranged from 24 h to 15 years. The most common site for GISTs was the stomach (n = 8). The median tumor size was 11 cm and the majority (n=10) was metastatic or locally advanced at the time of diagnosis. The tumors were often spindle-shaped at histology (n = 13) and the majority expressed KIT (n = 14). Most of the tumors (n = 12) were at high risk of recurrence according to the Joensuu scoring system. The availability of imatinib has improved the outcome of GIST with response in all cases it was used in neoadjuvant setting (n = 7). CONCLUSION: GISTs are rare tumors and preferentially affect the stomach in Cotonou). Most of the tumors were large, unresectable at the time of diagnosis and at high risk of recurrence. Access to imatinib has revolutionized the management of those tumors in our country.

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.

Distress in soft-tissue sarcoma and gastrointestinal stromal tumours patients-Results of a German multicentre observational study (PROSa).

OBJECTIVE: Soft tissue sarcomas (STS) and gastrointestinal stromal tumours (GIST) are a group of rare malignant tumours with a high and heterogenous disease burden. As evidence is scarce, we analysed the prevalence of increased emotional distress and identified distress-associated factors in these patients. METHODS: The PROSa-study (Burden and medical care of sarcoma) was conducted between 2017 and 2020 in 39 study centres. Cross-sectional data from adult STS and GIST patients were analysed. Distress was measured with the Patient Health Questionnaire (PHQ-4). The relation of socioeconomic and clinical factors with distress was explored in adjusted logistic regression models. RESULTS: Among 897 patients, 17% reported elevated anxiety and 19% reported depression. Unemployed patients (odds ratio [OR] 6.6; 95% CI 2.9-15.0), and those with a disability pension (OR 3.1; 95% CI 1.9-5.0) were more likely to experience distress compared to employed patients. Also, patients with a disability pass had higher odds of increased distress than those without (OR 1.8; 95% CI 1.2-2.7). Lowest distress was observed in patients 2 to <5 years and ≥5 years after diagnosis (comparison: <6 months) (OR 0.4; 95% CI 0.2-0.6) and (0.3; 95% CI 0.2-0.6). Patients with thoracic STS (vs. lower limbs) had twice the odds to experience distress (OR 2.0; 95% CI 1.1-3.6). Distress was seen almost twice as often in patients with progressive disease (vs. complete remission) (OR 1.7; 95% CI 1.1-2.8). CONCLUSION: The prevalence of elevated distress in STS and GIST patients is high. In unemployed patients, in those with a disability pension and in newly diagnosed patients a noticeable increase was observed. Clinicians should be aware of these factors and consider the social aspects of the disease.

Descriptive epidemiology of soft tissue sarcomas and gastrointestinal stromal tumors in Thailand.

This study aimed to analyze burden of STS and GIST in population and survival rate which represented the current situation of treatment in Thailand. The data was collected from five population-based cancer registries around the country for the period 2001 through 2015. The Segi world standard population was used to calculated age-standardized incidence rates (ASR). Standardized rate ratios (SRR) were used to compare populations. Joinpoint Trend Analysis was used to assess changes in incidence. STATA was used to examine patient survival rates. During the study period, 4080 cases of STS and 457 cases of GIST were reported. The ASR of STS and GIST was 2.14/100,000 person-years and 0.22/100,000 person-years, respectively. The most common histological types of STS were unspecified sarcoma (24.8%), leiomyosarcoma (19.0%) and liposarcoma (11.4%). The overall ASR of STS in Thailand was relatively low compared to Western countries. The five-year survival rate was 62.6% for STS and 63.4% for GIST, which was comparable to the rates reported in other countries. This is the first report of STS and GIST from PBCRs in Thailand. Based on current healthcare service, an overall survival rates of STS and GIST are comparable to those reported from others.

Patient demographics, clinicopathologic features, and outcomes in wild-type gastrointestinal stromal tumor: a national cohort analysis.

Wild-type KIT and PDGFRA gastrointestinal stromal tumors (GIST) are rare tumors with limited treatment options. We sought to determine the clinicopathologic features of wild-type GIST and identify factors that influence overall survival (OS) using a large national database. Retrospective evaluation of patients with wild-type GIST in the National Cancer Database (NCDB) was performed. Demographic, clinicopathologic, and treatment data were analyzed. Features associated with OS were investigated using Kaplan-Meier analysis and Cox proportional hazards model. 244 patients with median diagnosis age of 59 years (95% CI 57-63) were identified. The stomach was the most common primary site (57%) followed by the small intestine (35%). Surgical resection was performed on 85% of patients and 53% of patients received systemic therapy. Factors associated with decreased OS on multivariable analysis included small intestine primary (HR 2.72, 95% CI 1.13-6.69, P = 0.026) and > 5 mitoses per 50 HPF (HR 4.77, 95% CI 1.86-13.2, P = 0.001). Wild-type GISTs may be identified in older patients, with most arising in the stomach and small bowel. Surgery remains the principal treatment modality. Small intestine primary site and high mitotic count were associated with abbreviated OS.

Agricultural exposure and risk of soft tissue sarcomas and gastrointestinal stromal sarcoma in the AGRIculture and CANcer (AGRICAN) cohort.

Sarcomas are a heterogeneous group of tumors whose incidence is nearly 5 per 100 000 inhabitants in Europe. Their causes are poorly understood, although occupational exposures (especially farming and pesticides) are suspected. The AGRICAN cohort is a prospective study of 181 842 individuals enrolled in 2005 to 2007 who completed an enrolment questionnaire with data on lifelong agricultural exposure. Associations between agricultural exposure and sarcoma overall, gastrointestinal stromal tumors (GIST) and myomatous and fibrous sarcoma together, were analyzed with a Cox model. Until 2015, 188 incident cases of sarcoma were identified. Increased risks were observed (a) among cattle farmers working <10 years (HR<10years  = 2.45, 95% CI 1.36-4.43) and breeding ≥50 livestock (HR≥50animals  = 3.84, 95% CI 1.60-9.22), especially if involved in animal care and building disinfection, (b) in greenhouse production (HR = 1.82, 95% CI 1.01-3.30) and (c) in field-grown vegetable production (HR = 1.49, 95% CI 0.96-2.32). Concerning histological subtypes, GIST were positively associated with pesticide use in vineyards (HR = 2.24, 95% CI 0.95-5.30). For myomatous and fibrous sarcoma, the only increase was seen in field-grown vegetable production (HR = 2.37, 95% CI 1.16-4.85). In AGRICAN, the risk of sarcomas was increased in several farming activities with differences according to histological subtype.

Multicenter analysis of gastrointestinal stromal tumors in inner Mongolia of China: A study of 804 cases.

INTRODUCTION: To analyze the clinicopathological characteristics, immunohistochemistry, genotyping and prognosis of patients in the multicenter GIST data in Inner Mongolia, China. METHODS: Retrospective analysis was performed on GIST data from January 2013 to January 2018 in Inner Mongolia. Descriptive statistics were used to analyze the clinical characteristics of GIST patients. The Chi-square test was performed on the modified NIH criteria by age distribution, and Kaplan-Merie method was used for survival analysis. RESULTS: A total of 804 patients were included in the GIST database in Inner Mongolia, with a male to female ratio of 1.1102:1. The most common location was the gastric (465). Mitotic count ≤5/50HPFs was found in 67.3 % patients. There were 276 patients with tumor diameter of 2-5 cm and 354 patients with tumor diameter of 5.1-10 cm.The modified NIH criteria was mainly of intermediate risk (210) and high risk (342). The recurrence and metastasis of patients were related to the tumor location, mitotic index, tumor size, and modified NIH criteria. All patients were followed up for 1-10 years, in which 63.1 % of them were followed up for at least three years. The 3-year survival rates of patients with modified NIH criteria of very low risk, low risk, intermediate risk, and high risk were 100 %, 100 %, 100 %, and 96.3 %, respectively. CONCLUSIONS: The incidence of GIST in middle-aged and elder people in Inner Mongolia is high, and the long-term prognosis of patients after surgical treatment is good, which can objectively reflect the incidence, diagnosis and treatment of GIST in Inner Mongolia.