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@#OBJECTIVE To provide reference for guiding the individualized drug therapy management of imatinib for gastrointestinal stromal tumor （GIST）， with the goal of enhancing patient survival rates and improving their quality of life. METHODS Using a nominal group technique， a multidisciplinary （clinical， pharmaceutical and evidence-based） expert panel was formed to create the Consensus of Chinese Experts on Individualized Medication Management of Imatinib for Gastrointestinal Stromal Tumors outline through joint discussions. The expert panel conducted systematic retrieval， analysis， and summarization of the outline’s content， and reached relevant consensus based on China’s current situation， clinical needs， and research evidence. An external expert panel was also formed， comprising experienced multidisciplinary experts in clinical practice. Delphi method questionnaire was employed to openly collect the external experts’ opinions， which were then organized， summarized， analyzed， provided with feedback， revised， and finally formed into a consensus. RESULTS & CONCLUSIONS The drafting of this consensus included the clinical application of imatinib in neoadjuvant therapy for GIST patients， adjuvant therapy for adult patients with significant risk of recurrence after surgical resection， and drug therapy for patients with recurrent， metastatic， or unresectable tumors； pharmaceutical monitoring and long-term medication management. This consensus provides standardized processes and methods for medical institutions in individualized drug therapy management for GIST patients and holds significant importance in improving the clinical efficacy of imatinib and ensuring drug safety.
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Objective To investigate the value of multiparametric CT features for predicting the risk classification of gastric stro-mal tumor(GST).Methods The clinical data from 139 patients with GST were retrospectively collected.According to the patho-logical risk results,the patients were divided into two groups:a low-risk GST group(including very low-and low-risk)with 75 patients and a high-risk GST group(including medium and high-risk)with 64 patients.The CT features between low-risk GST group and high-risk GST group were compared using chi-squared test or t-test.The risk factors of high-risk GST were identified by univariate analysis.The prediction models were built by multivariate logistic regression.The performance of models were evaluated by receiver oper-ating characteristic(ROC)curve.Results There were significant differences in the maximum tumor diameter,minimum tumor diameter,arterial phase enhancement degree,venous phase enhancement rate,arterial phase enhancement degree rate,venous phase enhance-ment degree rate,cystic,and necrosis between low-risk GST group and high-risk GST group,which were associated with the risk classification of GST.The area under the curve(AUC)of the quantitative features-based model that combined maximum tumor diam-eter,minimum tumor diameter,arterial phase enhancement degree,venous phase enhancement rate,arterial phase enhancement degree rate and venous phase enhancement degree rate,showed a significantly higher performance than the qualitative features-based model that incorporated cystic and necrosis(0.981 vs 0.850,P<0.001).Conclusion Maximum tumor diameter,minimum tumor diameter,arterial phase enhancement degree,venous phase enhancement rate,arterial phase enhancement degree rate,venous phase enhance-ment degree rate,as well as cystic and necrosis,are associated with the risk classification of GST and can predict the high-risk GST.
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Tyrosine kinase inhibitors （TKIs） represent a class of small-molecule targeted drugs that improve the survival time of patients with gastrointestinal stromal tumor （GIST）. Imatinib， sunitinib， regorafenib， ripretinib， and avapritinib are commonly used TKIs in the clinical treatment of various types of GIST. This article provides a comprehensive review of the pharmacokinetics and therapeutic drug monitoring （TDM） of these five drugs， finding that there is significant individual variability in the pharmacokinetics of these drugs. Among them， the absorption of imatinib， regorafenib， and avapritinib are influenced by food intake. Imatinib should be taken with meals and 200 mL of water， regorafenib is taken with a low-fat meal， while avapritinib is taken on an empty stomach. TKIs are mainly metabolized by cytochrome P450 3A4 （CYP3A4）， and when used in combination with CYP3A4 inducers or inhibitors， drug exposure levels will significantly change； apart from metabolic enzymes， the exposure levels of TKIs are also influenced by interactions with the transporter proteins P-glycoprotein and breast cancer resistance protein. Currently， research on TDM for TKIs is still in the exploratory stage， with a substantial amount of literature reporting the effective concentrations of imatinib， sunitinib and regorafenib. However， the precise relationship between exposure levels and efficacy/ toxicity needs further exploration. Currently， there is a lack of research on the correlation between exposure levels and efficacy/ toxicity of ripretinib and avapritinib. It is recommended to implement TDM in patients taking these drugs and explore their therapeutic window in combination with pharmacokinetic models. The commonly used methods for clinical TDM of TKIs include immunoassay， chromatography， and surface-enhanced Raman spectroscopy， providing a technical basis for clarifying the therapeutic window of TKIs.
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Gastrointestinal stromal tumor(GIST),with a certain malignant potential,are currently the most common subepithelial tumors of the gastrointestinal tract.Early diagnosis and prediction of malignant potential are very important for the formulation of a treatment plan and determining the prognosis of GIST.Deep learning technology has made significant progress in the diagnosis of digestive tract diseases,and it can also effectively assist physicians in diagnosing GIST and predicting their malignant potential,preoperatively.The application of deep learning technology in the diagnosis of GIST includes CT,gastrointestinal endoscopy and endoscopic ultrasound.This paper aims to review the application of deep learning technology in the diagnosis and prediction of malignant potential of GIST.
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Abstract Succinate dehydrogenase inhibitors (SDHIs), fungicides currently most used in agriculture in Brazil, act by blocking the enzyme succinate dehydrogenase (SDH) from plant pathogens. However, studies show that SDHIs can not only inhibit SDH activity in target fungi, but also block that activity in human cells. Considering the medical and agricultural implications of SDH, the purpose of this narrative review is to describe the relationship between exposure to fungicides SDHIs and epigenetic regulation of SDH associated with the development of gastrointestinal stromal tumor, pheochromocytoma/paraganglioma, and cancer. The results obtained with the research showed that the human SDH enzyme exhibited sensitivity to some tested SDHIs, which may cause microcephaly and defects in neurological development. Deficiency of SDH activity causes accumulation of succinate which can act as an oncometabolite inhibit-ing iron-dependent dioxygenases and alpha-ketoglutarate, eleven translocation -TET and histone demethylases, inducing epigenetic changes that lead to multiple cancers and other diseases. Therefore, further in vitro and in vivo analyzes should be performed to assess susceptibility to diseases influenced by the toxic effect of SDHIs.
Resumo Os inibidores da succinato desidrogenase (SDHIs), fungicidas atualmente mais utilizados na agricultura no Brasil, atuam bloqueando a enzima succinato desidrogenase (SDH) de fitopatógenos. No entanto, estudos mostram que SDHIs podem nao apenas inibir a atividade de SDH em fungos alvo, mas também bloquear essa atividade em células humanas. Considerando as implicares médicas e agrícolas do SDH, o objetivo desta revisao narrativa é descrever a relaqao entre a exposiqao a fungicidas SDHIs e a regulaqao epigenética do SDH associada ao desenvolvimento de tumor estromal gastrointestinal, feocromocitoma/paraganglioma e cáncer. Os resultados obtidos com a pesquisa mostraram que a enzima SDH humana apresentou sensibilidade a alguns SDHIs testados, que podem causar microcefalia e defeitos no desenvolvimento neurológico. A deficiencia da atividade da SDH causa acumulo de succinato que pode atuar como um oncometabólito inibindo as dioxigenases dependentes de ferro e alfa-cetoglutarato, onze translocaqóes -TET e histonas desmetilases, induzindo alteraqóes epigenéticas que levam a múltiplos canceres e outras doenqas. Portanto, análises adicionais in vitro e in vivo devem ser realizadas para avaliar a suscetibilidade a doenqas influenciadas pelo efeito tóxico dos SDHIs.
Resumen Los inhibidores de la succinato deshidrogenasa (SDHI), los fungicidas actualmente más utilizados en la agricultura en Brasil, actúan bloqueando la enzima succinato deshidrogenasa (SDH) de los patógenos de las plantas. Sin embargo, los estudios muestran que los SDHI no solo pueden inhibir la actividad de SDH en los hongos objetivo, sino que también bloquean esa actividad en las células humanas. Teniendo en cuenta las implicaciones médicas y agrícolas de SDH, el propósito de esta revisión narrativa es describir la relación entre la exposición a fungicidas SDHI y la regulación epigenética de SDH asociada con el desarrollo de tumores del estro-ma gastrointestinal, feocromocitoma/paraganglioma y cáncer. Los resultados obtenidos con la investigación mostraron que la enzima SDH humana mostró sensibilidad a algunos SDHI probados, lo que puede causar microcefalia y defectos en el desarrollo neurológico. La deficiencia de la actividad de SDH provoca la acumulación de succinato que puede actuar como un oncometabolito que inhibe las dioxigenasas dependientes de hierro y el alfa-cetoglutarato, once translocaciones -TET e histona desmetilasas, induciendo cambios epigenéticos que conducen a múltiples cánceres y otras enfermedades. Por lo tanto, se deben realizar más análisis in vitro e in vivo para evaluar la susceptibilidad a enfermedades influenciadas por el efecto tóxico de los SDHI.
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Objective:To investigate the efficacy of different laparoscopic surgeries for gastrointestinal stromal tumors (GIST) of gastric cardia and fundus.Methods:The retrospective cohort study was conducted. The clinicopathological data of 251 patients with GIST of gastric cardia and fundus who underwent laparoscopic radical resection in 14 medical centers, including Guangdong Provincial People′s Hospital et al, from December 2007 to December 2021 were collected. There were 123 males and 128 females, aged 58(24,87)years. Observation indicators: (1) treatment; (2) clinicopathological data of patients undergoing different laparoscopic surgeries; (3) subgroup analysis for special laparoscopic techniques. Measurement data with normal distribution were represented as Mean± SD, and comparison between groups was conducted using the t test or ANOVA. Measure-ment data with skewed distribution were represented as M( Q1, Q3), and comparison between groups was conducted using the Mann-Whitney U test or Kruskal-Wallis H test. Count data were described as absolute numbers or percentages. Comparison of ordinal data was conducted using the rank sum test. Results:(1) Treatment. Of the 251 patients,202 cases underwent gastric wedge resection, 26 cases underwent special laparoscopic techniques including 10 cases with serotomy and dissection and 16 cases with transluminal gastrectomy, 23 cases underwent structural gastrectomy including 6 cases with total gastrectomy and 17 cases with proximal partial gastrectomy. There were 24 patients had postoperative complications after surgery. (2) Clinicopathological data of patients undergoing different laparoscopic surgeries. The gender (male, female), age, tumor diameter, operation time, volume of intraoperative blood loss, length of incision, time to postoperative initial whole liquid food intake, time to postoperative initial semi-liquid food intake, duration of postoperative hospital stay, cases with perioperative complications, cases with mitotic count as ≤5/50 high power field, 6?10/50 high power field, >10/50 high power field, cases be classified as very low risk, low risk, medium risk, high risk according to the National Institutes of Health risk classification, cases with tumor located at fundus and gastric cardia were 93, 109, (59±11)years, 3.50(0.40,10.00)cm, 88.00(25.00,290.00)minutes,20.00(25.00,290.00)mL, 4.00(2.00,12.00)cm, 3.00(1.00,9.00)days, 4.00(1.00,16.00)days, 5.00(1.00,18.00)days, 14, 164, 31, 7, 47, 83, 50, 22, 30, 172 in patients undergoing gastric wedge resection, respectively. The above indicators were 19, 7, (49±14)years, 2.55(0.20,5.00)cm, 101.00(59.00,330.00)minutes, 27.50(2.00,300.00)mL, 4.50(0,6.00)cm, 2.50(1.00,10.00)days, 4.00(1.00,16.00)days, 6.00(1.00,18.00)days, 3, 20, 5, 1, 15, 5, 2, 4, 24, 2 in patients undergoing special laparos-copic techniques, and 11, 12, (52±10)years, 5.00(0.80,10.00)cm, 187.00(80.00,325.00)minutes, 50.00(10.00,300.00)mL, 6.00(4.00,12.00)cm, 4.00(2.00,8.00)days, 6.00(3.00,14.00)days, 8.00(2.00,18.00)days, 7, 11, 5, 7, 2, 6, 6, 9, 13, 10 in patients undergoing structural gastrectomy. There were significant differences in the above indicators among the three groups of patients ( χ2=6.75, F=10.19, H=17.71, 37.50, 35.54, 24.68, 16.09,20.20, 13.76, χ2=13.32, Z=28.98, 32.17, χ2=82.14, P<0.05). (3) Subgroup analysis for special laparoscopic techniques. The time to postoperative initial whole liquid food intake, time to postoperative initial semi-liquid food intake, classification of tumor location (endophytic type, exophytic type, parietal type) were 4.50(1.00,10.00)days, 8.00(3.00,12.00)days, 0, 8, 2 in patients undergoing serotomy and dissection, versus 2.00(1.00,4.00)days, 3.00(1.00,6.00)days, 16, 0, 0 in patients undergoing transluminal gastrectomy. There were significant differences in time to postoperative initial whole liquid food intake, time to postoperative initial semi-liquid food intake between them ( Z=-2.65, -3.16, P<0.05); and there was a significant difference in classification of tumor location between them ( P<0.05). Conclusions:Gastric wedge resection is the most commonly used laparoscopic technique for GIST of gastric cardia and fundus. The application of special laparoscopic techniques is focused on the GIST of cardia to preserve the function of the cardia.
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Objective:To investigate the prediction of National Institute of Healthy (NIH) risk stratification of gastrointestinal stromal tumor(GIST) based on clinical ultrasound model, ultrasonographic radiomics model and combined model by oral contrast enhanced ultrasonography.Methods:The clinical and ultrasound imaging data of 204 gastric GIST patients attending Tianjin Medical University Cancer Institute and Hospital from June 2021 to June 2022 were retrospectively analyzed, among whom a total of 101 patients with high and moderate NIH risk stratification GIST confirmed by postoperative pathology were included in the high risk group, and a total of 103 patients with low and extremely low NIH risk stratification GIST were in the low risk group. The ultrasound images of the largest diameter of the GIST were manually segmented by ITK-SNAP software, and Pyradiomics (v3.0.1) module in Python 3.8.7 was applied to extract ultrasonographic radiomics features from the ROI segmented images. The patients were randomly divided into training and validation sets in the ratio of 7∶3. The XGBoost of Sklearn module was applied to construct the clinical ultrasound imaging model, ultrasonographic radiomics model, and combined model. Then the area under ROC curve (AUC), sensitivity, specificity, and accuracy were evaluated; the predictive ability of the three models was compared by Delong test. Calibration Curve was applied to evaluate the model performance, and the clinical Decision Curve Analysis was applied to determine the net benefit to patients.Results:A total of 578 ultrasonographic radiomics features were extracted from ROI, and 8 ultrasonographic radiomics features were finally retained for modeling after regression and dimensionality reduction. Finally, test results showed that AUC, sensitivity, specificity and accuracy of clinical ultrasound imaging model, ultrasonographic radiomics model and combined model were 0.75, 69.3%, 68.9%, 69.1%; 0.87, 79.2%, 81.6%, 80.4%; 0.91, 80.2%, 83.5%, 81.9%, respectively. Delong test showed that the difference of AUC between ultrasonographic radiomics model and clinical ultrasound imaging model was statistically significant ( Z=2.698, P<0.001), and the combined model was significantly better than clinical ultrasound imaging model ( Z=4.062, P<0.001) and ultrasonographic radiomics model ( Z=2.225, P=0.026). Calibration Curve showed the high performance of combined model, and Decision Curve Analysis showed the superior clinical usefulness of combined model. Conclusions:It is feasible to construct an ultrasonographic radiomics model for GIST NIH risk stratification based on oral contrast enhanced ultrasonography images, and the combined model has more advantageous diagnostic performance, which can identify high risk NIH GIST objectively and stably for clinical purposes.
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Mixed epithelial and stromal tumors of the kidney (MESTK) are very rare in male kidney tumors. One case was reported in this article. The patient accidentally discovered a renal space occupying lesion during physical examination and underwent laparoscopic radical left nephrectomy. The postoperative pathological results were reported as MESKT, with immunohistochemical staining of ER (+ ), PR (+ ), SMA (+ ). After a follow-up of 4 months, the results showed that the patient had no signs of tumor recurrence or related complications.
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Gastrointestinal stromal tumor(GIST)is the most common mesenchymal tumor of the gastrointestinal tract.The pathogenesis of most GIST is driven by the gain-of-function mutations of KIT proto-oncogene receptor tyrosine kinase(c-KIT)or platelet-derived growth factor receptor alpha(PDGFRA)gene.The original clinical treatment for GIST was surgical resection only.With the advent of tyrosine kinase inhibitors(TKIs)represented by imatinib,GIST therapy has entered the era of targeted therapy.TKIs have achieved significant clinical efficacy in GIST treatment.To date,several TKI drugs have been approved for clinical application,which has greatly improved the survival time of GIST patients,but the ensuing drug resistance problem is a difficult problem that requires an urgent solution.Currently,it has been confirmed that the main reason for drug resistance to TKI in GIST is the secondary mutation of different exons of c-KIT or PDGFRA.However,even GIST patients with the same exon mutation still reacts very differently to TKIs,suggesting that there may be other mechanisms acting in parallel with c-KIT and PDGFRA.Thanks to the development and application of molecular biological technologies such as CRISPR gene editing technology,the genetic differences between TKI drug resistant and sensitive GIST are becoming clearer and clearer,and many more new mechanisms have been identified.This paper summarizes the latest research progress of the mechanism of drug resistance to the most commonly used TKIs in the clinical treatment of GIST.
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Objective:To explore the predictive value of multi-slice spiral CT (MSCT) arterial enhancement fraction (AEF) in the risk degree of gastrointestinal stromal tumors (GIST).Methods:The clinical data of 178 patients with GIST in Shanxi Province Cancer Hospital from January 2013 to May 2021 were retrospectively analyzed. According to tumor size, primary site, mitotic figure and whether the tumor was ruptured or not, the patients were divided into extremely low risk group (24 cases), low risk group (44 cases), medium risk group (48 cases), and high risk group (62 cases). One-way ANOVA was used to compare MSCT AEF value and conventional plain and enhanced scan values among the 4 groups. The receiver operating characteristics (ROC) curve was drawn to assess the predictive efficacy of AEF value, conventional plain value, enhanced scan value, Ki-67 and AEF value combined with Ki-67 for high-risk GIST.Results:Except for CT value in the venous phase and CT value in added arterial phase, the comparison of AEF value, plain scan CT value, arterial phase CT value, and added venous phase CT value of GIST patients in the 4 groups showed statistically significant differences (all P < 0.05). In the predictive efficacy evaluation of high-risk GIST, the area under the curve (AUC) of the ROC curve for AEF value was 0.753, which was higher than that for plain scan CT value (0.593), arterial phase CT value (0.592) and added venous phase CT value (0.631), lower than AEF combined with Ki-67 (0.799). Kappa consistency test showed that the AEF value, plain scan CT value and arterial phase CT value were consistent with the pathological examination results (Kappa values were 0.375, 0.168 and 0.168, respectively), however, the added venous phase CT value was inconsistent with the pathological examination results (Kappa = -0.224). The AUC of AEF value combined with Ki-67 for predicting high-risk GIST was 0.799, and the Kappar value was 0.528. Conclusions:MSCT AEF value varies in GIST with different risk degree, and its preoperative efficacy in predicting high-risk GIST is superior to that of conventional plain scan and enhanced scan; especially the combination of AEF value with Ki-67 shows a higher predictive efficacy.
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Objective:To investigate the clinical and MRI features of the mixed epithelial and stromal tumor family (MESTF) of the kidney.Methods:From January 2009 to September 2021, 42 patients with pathologically-proven MESTF from the First Medical Center of Chinese PLA General Hospital were collected in this retrospective study. Clinical information, MRI features, and pathological results were documented. According to the Bosniak classification (BC) version 2019, all MESTFs were divided into cystic MESTFs (36 cases) and solid-cystic MESTFs (6 cases). The R.E.N.A.L. nephrometry score (RNS), lesion size, laterality, location, margin, shape, growth pattern, presence of protruding into renal sinus, hemorrhage, and enhancement pattern were evaluated and documented. Based on BC versions 2005 and 2019, all the cystic MESTFs were assessed and divided into low (Ⅰ, Ⅱ, ⅡF) and high (Ⅲ, Ⅳ) grades. The independent sample t test or Mann-Whitney U test were performed to compare age, RNS, and lesion size between cystic MESTFs and solid-cystic MESTFs. Pearson χ 2 test, continuity-adjusted χ 2 test or Fisher exact probability test were utilized to evaluated the differences of clinical and MRI features and the distribution of low or high grades in two versions of BC. Results:Forty-two MESTFs were unilateral and solitary masses, 25 males and 17 females, with a mean age of (41±13) years old. Compared to solid-cystic MESTFs, cystic MESTFs were prone to demonstrate endophytic growth pattern (χ 2=17.77, P<0.001), and no significant differences in other clinical and MRI features were observed between cystic and solid-cystic MESTFs (all P>0.05). There were 7 low-grade and 29 high-grade tumors in the BC version 2005, respectively. Meanwhile, 24 low-grade and 12 high-grade tumors in the BC version 2019, respectively. The distribution of low or high-grade tumors in the two versions of BC had a statistically significant difference (χ 2=16.37, P<0.001). Conclusion:MESTFs demonstrated middle-age onset and no gender predilection. Cystic MESTFs are more likely to exhibit endophytic growth pattern with low-grade classification in BC system version 2019.
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Gastrointestinal stromal tumor (GIST) is a kind of mesenchymal tumor, most commonly found in the stomach, with unique immunophenotype and molecular genetic characteristics. Gastric GIST mostly originates from the musculi propria of the stomach wall. It often grows expansively with clear boundaries and is relatively easy to separate. Surgery is still the preferred treatment for gastric GIST. With the rapid development of laparoscopic technique, laparoscopic surgical treatment for gastric GIST has been gradually recognized. However, it still remains unclear whether laparoscopic surgery can be applied in gastric gastrointestinal stromal tumors located in unfavorable sites. Here, this paper will combine author center′s exploration and clinical application on laparoscopic surgery for gastric GIST located in unfavorable sites and make a brief summary in order to choose a better way for treatment of gastric GIST located in unfavorable sites.
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The diagnosis of myoid gonadal stromal tumor (MSGT) can represent a difficult challenge, both for the extreme rarity of this neoplasm and for the clinical?radiological characteristics similar to other neoplasms of the testicle. The case management we present suggests how a complete differential diagnosis can be obtained by integrating ultrasonographic and pathological data.
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Synchronous endometrial and ovarian carcinoma is a rare instance and it accounts for 50 to 70% of all synchronous female genital tract tumors. However, it is very rare to find synchronous endometrial carcinoma and ovarian sex cord–stromal tumor (thecoma). The present case is a 75-year-old woman with a complaint of post-menopausal vaginal bleeding. Radiologically, the magnetic resonance imaging (MRI) pelvis revealed altered signal intensity mass in the uterus. Frozen section and routine histopathological examination were done on radical hysterectomy. Microscopically, serous carcinoma involving uterine corpus and left Fallopian tube was identified along with the unusual finding of contralateral ovarian sex cord–stromal tumor (thecoma), which was confirmed on immunohistochemical examination. It is a very rare association and is first reported in the present study after a thorough search of the published literature. Their relationship based on a high level of estrogen produced by the hyperactive ovary is controversial as serous carcinomas are less hormone-dependent.
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Resumen Objetivos: Presentar caso clínico y revisión de la literatura sobre asociación de tumores poco frecuentes compatibles con diagnóstico de tríada de Carney. Paciente y Métodos: Revisión de ficha clínica de paciente de sexo femenino de 39 años de edad con antecedentes de asma, quien acude a servicio de urgencias por síntomas respiratorios. En estudio con imágenes se evidencia masa pulmonar en lóbulo superior derecho probablemente hamartoma y masa en la bifurcación carotídea izquierda compatible con posible paraganglioma. Se completó el estudio con endoscopia digestiva alta sin evidencia de tumor gástrico y PET-CT (tomografía de emisión de positrones-tomografía computarizada) que descartó otras lesiones. Resultados: La paciente fue sometida a resección quirúrgica de ambos tumores (pulmonar y carotídeo). En estudio histopatológico diferido, se plantean los diagnósticos de paraganglioma carotideo y hamartoma pulmonar, el cual, luego de una segunda revisión histopatológica, es corregido a condroma pulmonar. Discusión: La tríada de Carney se compone por la asociación de al menos 2 de 3 tumores: tumor estromal gastrointestinal (GIST), paraganglioma extra-adrenal y condroma pulmonar. Su expresión es variable, coexistiendo en forma completa en solo el 22% de los casos. Conclusión: Los pacientes con sospecha de tríada de Carney deben recibir evaluación multidisciplinaria, estudio completo en búsqueda de tumores asociados y seguimiento a largo plazo por posibles recurrencias o metástasis.
Objective: To present a clinical case and review of the literature on the infrequent association of pulmonary and extra thoracic tumors compatible with Carney's triad. Patient and Methods: Review of clinical records of a 39 years-old female patient with history of asthma who presented in the emergency department with respiratory symptoms. An imaging study showed a pulmonary mass in the right upper lobe with the aspect of hamartoma and a mass in the left carotid artery bifurcation compatible with a possible paraganglioma. Upper gastrointestinal endoscopy showed no evidence of gastric tumor and a PET-CT (Positron Emission Tomography - Computed Tomography) excluded other lesions. Results: Patient underwent surgical resection of both tumors (pulmonary and carotid). Diagnosis of carotid paraganglioma and pulmonary hamartoma were stated by histopathology. However, lung tumor after a second pathological analysis was confirmed to be a pulmonary chondroma. Discussion: Carney's triad is defined by the association of at least 2 of 3 tumors: Gastrointestinal Stromal Tumor (GIST), extraadrenal paraganglioma and pulmonary chondroma. Its expression is variable, coexisting completely in only 22% of cases. Conclusion: Patients with suspected Carney's triad should receive a multidisciplinary assessment, a complete study searching associated tumors and long-term follow-up for recurrences or metastases.
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Humans , Female , Adult , Paraganglioma/diagnostic imaging , Carotid Arteries/diagnostic imaging , Chondroma/diagnostic imaging , Lung Neoplasms/diagnostic imaging , Neoplasms, Multiple Primary , Paraganglioma/surgery , Radiography, Thoracic , Carotid Arteries/surgery , Chondroma/surgery , Gastrointestinal Stromal Tumors/surgery , Gastrointestinal Stromal Tumors/diagnostic imaging , Positron Emission Tomography Computed Tomography , Gastrointestinal Neoplasms/surgery , Gastrointestinal Neoplasms/diagnostic imaging , Lung Neoplasms/surgeryالملخص
SUMMARY OBJECTIVE: The stroma surrounding the tumor cells is important in tumor progression and treatment resistance, besides the properties of tumor cells. Studies on the tumor stroma characteristics will contribute to the knowledge for new treatment approaches. METHODS: A total of 363 breast cancer patients were evaluated for the tumor-stroma ratio. The percentage of stroma was visually assessed on hematoxylin-eosin stained slides. The cases of tumor-stroma ratio more than 50% were categorized as tumor-stroma ratio high, and those less than 50% and below were categorized as tumor-stroma ratio low. RESULTS: Tumor-stroma ratio-high tumors had shorter overall survival (p=0.002). Disease-free survival tended to be shorter in tumor-stroma ratio-high tumors (p=0.082) compared with tumor-stroma ratio-low tumors. Tumor-stroma ratio was an independent prognostic parameter for the total group of patients (p=0.003) and also axillary lymph node metastasis and tumor-stroma ratio was statistically associated (p=0.004). Also, tumor-stroma ratio was an independent prognostic parameter in node-positive Luminal A and B subgroups for overall survival (p<0.001). CONCLUSION: Tumor-stroma ratio is an independent prognostic parameter that can be evaluated quite easily in all molecular subtypes of all breast cancers and does not require extra cost and time to evaluate.
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Humans , Female , Breast Neoplasms/pathology , Triple Negative Breast Neoplasms/pathology , Prognosis , Stromal Cells/pathology , Receptor, ErbB-2 , Disease-Free Survival , Lymphatic Metastasis/pathologyالملخص
Gastrointestinal Stromal Tumours (GISTs) are one of the rare causes of Alimentary Tract Neoplasm. They arise from the Interstitial Cells of Cajal (ICC) with overexpression of proto-oncogenes like KIT, PDGFRA and BRAF-Kinase, etc1. The typical location of these tumors is the stomach with Jejunal GIST being the rarest variant. The maximal incidence of the disease is reported in the sixth decade of life. GISTs are often asymptomatic and clinicians are misdirected towards a different diagnosis because of its variable nature of presentation. The clinical feature typically ranges from non-specific abdominal symptoms like nausea, vomiting, bloating, etc, to abdominal emergencies like hemorrhage, anemia, or obstruction. Although mostly benign, there is a high probability of progression to malignancy2. Thus, in patients with no appreciable cause of gastrointestinal bleed or chronic abdominal discomfort, GIST should have high suspicion index. This can help to limit the progression and thereby prevent further complications. Due to its location, it is difficult to identify by Endoscopy or Colonoscopy. Single Balloon Enteroscopy (SBE) and CT Scan of Abdomen are the primary investigational modalities. The treatment aims at resection of the mass with continued Postsurgical surveillance and targeted molecular therapy in some cases. Herein, we report a 14-year-old boy with chronic paleness and gradual onset repeat episodes of Melaena. Despite repeated blood transfusions, the patient had a Hemoglobin
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Objective:To compare the safety and efficacy of laparoscopy and laparotomy for 5-10 cm intermediate-risk gastric stromal tumor, and to evaluate whether there was evident benefits of postoperative adjuvant treatment with imatinib.Methods:A retrospective study was conducted on 72 patients with moderate risk gastric stromal tumors (5-10 cm in diameter) who received operation in Nanjing Drum Tower Hospital from January 2010 to July 2020. There were 28 cases in the laparoscopy group and 44 cases in the laparotomy group. The clinical features, pathological data, perioperative results and hospitalization costs were compared between the two groups. The survival rates of postoperative adjuvant therapy with or without imatinib were analyzed and compared.Results:There was no significant difference in clinicopathological features between the two groups ( P>0.05). The incidences of postoperative complications in the laparoscopy group and the laparotomy group were 32.1% (9/28) and 52.3% (23/44) respectively, showing no significant difference ( P=0.094). Compared with the laparotomy group, both the hospital stay (12.5±3.2 days VS 15.0±3.5 days, P=0.004) and the median postoperative hospital stay (7.5 days VS 9.0 days, P=0.006) in the laparoscopy group were significantly shorter, and the first exhaust time was significantly shorter ( P=0.003). During the median follow-up period of 58 months (13-129 months), there was no tumor-related death. Two cases died of breast cancer and heart disease in the laparotomy group, and 1 case died irrelevant to gastric stromal tumor in the laparoscopy group. Of the 72 patients, 40 received postoperative imatinib adjuvant therapy, 22 cases (50.0%) in the laparotomy group and 18 cases (64.3%) in the laparoscopy group, with no significant difference in the proportion ( χ2=1.414, P=0.234). There was significant difference in the overall survival rate between the group treated with imatinib and the group without imatinib ( P=0.015). Conclusion:Laparoscopic resection is safe and effective for intermediate-risk gastric stromal tumor of 5-10 cm. Taking imatinib adjuvant treatment does not increase overall survival rate of patients with intermediate-risk gastric stromal tumors (5-10 cm), and there is no tumor-related death, recurrence or metastasis for those who did not accept imatinib adjuvant treatment after R0 resection.
الملخص
Objective:To construct enhanced computed tomography (CT)-based nomograph model, to assist physicians in differentiating gastric schwannoma from gastric stromal tumor.Methods:From January 1, 2012 to January 1, 2022, at the Second Affiliated Hospital of Zhejiang University School of Medicine and Ningbo Hwamei Hospital, University of Chinese Academy of Sciences, 57 patients with gastric schwannoma and 275 patients with gastric stromal tumor confirmed by surgical pathology were retrospectively collected, among whom 39 patients with gastric schwannoma and 201 patients with gastric stromal tumor were enrolled in the training set, and the other 18 patients with gastric schwannoma and 74 patients with gastric stromal tumor were enrolled in the validation set. The contrast-enhanced CT imaging features (tumor size index, arterial phase CT value, venous phase CT value, necrosis, calcification, integrity of mucosal surface, and uniform enhancement, etc.) and clinical data (history of gastritis, carbohydrate antigen 19-9 (CA19-9), carcinoembryonic antigen, and monocyte to lymphocyte ratio (MLR), etc.) were collected. The least absolute shrinkage and selection operator (LASSO) regression analysis was used to screen the independent predictive factors of imaging features in the differential diagnosis of gastric schwannoma and gastric stromal tumor, and a nomograph model was constracted. Logistic regression analysis was used to analyze and screen the independent predictive factors of clinical indicators to distinguish gastric schwannoma from gastric stromal tumor, and a clinical control model was established. The receiver operating characteristic curve(ROC) was used to analyze the area under the curve (AUC) of the nomograph model in the training set and the verification set, and concordance index (CI) and decision curve analysis (DCA) were used to evaluate the predictive efficiency and clinical application value of the nomograph model. DeLong test was used for statistical analysis.Results:The results of LASSO regression analysis showed that tumor size index, arterial phase CT value, venous phase CT value, necrosis, calcification, integrity of mucosal surface, and uniform enhancement were independent predictive factors of imaging features in the differential diagnosis of gastric schwannoma and gastric stromal tumor(all P<0.05). The results of logistic regression analysis indicated that the history of gastritis ( OR=0.280, 95% confidence interval 0.138 to 0.566), CA19-9 ( OR=0.940, 95% confidence interval 0.890 to 0.993), carcinoembryonic antigen ( OR=0.794, 95% confidence interval 0.661 to 0.952), and MLR ( OR=0.087, 95% confidence interval 0.009 to 0.860) were independent predictive factors of clinical indicators in the differential diagnosis of gastric schwannoma and gastric stromal tumor ( P<0.001, =0.028, 0.013 and 0.037). The AUCs of the nomograph model in the training and validation set were 0.881 and 0.850, respectively, and the AUCs of the clinical control model in the training and validation set were 0.814 and 0.772, respectively, and the differences were statistically significant ( Z=2.57 and 1.96, P=0.005 and 0.030). The average CI of the nomograph model was 0.885. The results of DCA analysis showed that the overall benefit of the nomograph model was higher than that of the clinical control model. Conclusion:The enhanced CT-based nomograph model can effectively distinguish gastric schwannoma from gastric stromal tumor, and can help physicians to make precise clinical decisions.
الملخص
Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor of the gastrointestinal tract. Twenty years ago, tyrosine kinase inhibitors targeting KIT/platelet-derived growth factor receptor α were applied to GIST treatment and achieved great success. Therefore, this rare tumor has attracted great attention of clinicians and researchers. After 20 years of exploration and practice, great progress has been made in the clinical diagnosis and research level of GIST in China. In terms of clinical diagnosis and treatment, the pathological diagnosis system has been established and deepened, and the surgical treatment has gradually tended to be standardized, minimally invasive and integrated. More and more targeted drugs continue to challenge the survival limit of GIST patients, and the standardized diagnosis and treatment model has been initially formed and continuously developed. In terms of GIST related researches, drug clinical research from China has experienced the exploration from imitation to innovation, the clinical research related to surgical treatment is undergoing preliminary exploration. The research related to endoscopic treatment has formed a distinctive style with unique Chinese characteristics, and the translational research has also helped solving the clinical problems. In the future, the clinical diagnosis and treatment of GIST in China will be more standardized, minimally invasive, precise, comprehensive and whole process. The research direction in the field of GIST will be to carry out high-quality multi-center clinical research and targeted laboratory researches for clinical focus issues.