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1.
Zhonghua Wei Chang Wai Ke Za Zhi ; 22(9): 831-834, 2019 Sep 25.
Artigo em Chinês | MEDLINE | ID: mdl-31550821

RESUMO

Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor of the digestive tract. The diagnosis of GIST relies mainly on clinicopathological features, tumor cell morphology and immunohistochemical marker CD117 (c-Kit). However, some tumors (about 3%-4%) have clinicopathological features of GIST but do not express CD117. To determine whether these lesions are true GIST, it is necessary to raise awareness of CD117-negative GIST. This article discusses the immunohistochemical features, gene mutations, prognosis and efficacy of targeted drugs of CD117-negative GIST. Research results suggest that CD117-negative GIST lacks KIT expression but has typical clinical, histopathological and cytogenetic features. These tumors have KIT and/or PDGFRA mutations, or are wild type. Because most KIT-negative GISTs contain PDGFRA or KIT mutation, pathologists and oncologists should not exclude GIST diagnosis based on negative immunohistochemical staining of KIT. It is known that approximately 30% of PDGFRA mutations may be sensitive to imatinib, and patients with such tumors may benefit from imatinib, so imatinib treatment should not be empirically denied in these patients.


Assuntos
Tumores do Estroma Gastrointestinal , Proteínas Proto-Oncogênicas c-kit , Antineoplásicos/uso terapêutico , Tumores do Estroma Gastrointestinal/classificação , Tumores do Estroma Gastrointestinal/tratamento farmacológico , Tumores do Estroma Gastrointestinal/genética , Tumores do Estroma Gastrointestinal/patologia , Humanos , Mutação , Prognóstico , Proteínas Proto-Oncogênicas c-kit/metabolismo , Receptor alfa de Fator de Crescimento Derivado de Plaquetas
2.
J Cancer Res Clin Oncol ; 145(6): 1559-1568, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30923944

RESUMO

PURPOSE: Gastrointestinal stromal tumors (GISTs) are typically solid neoplasms with small cystic change detected occasionally but in rare instances may present predominantly as cystic lesions. The histopathologic features and prognoses of cystic GISTs (cGISTs) are poorly understood. METHODS: We herein reviewed 20 cGISTs resected or consulted in our institution from January 1, 2003 to December 31, 2014. RESULTS: Of the 20 patients included, the mean age was 61 years and the male-to-female ratio was 9:11. The original locations were the stomach (n = 10, 50%), the small intestine (n = 9, 45%) and the omentum (n = 1, 5%). Indistinct diagnosis or misdiagnosis was established in 15 cases based only on preoperative radiology. Grossly, the cystic component made up the bulk of masses and was filled by dark bloody fluid and necrotic debris in 18 cases. Microscopically, cyst wall was composed of neoplastic spindle (n = 14, 70%)/epithelioid cells (n = 6, 30%) and collagenous fiber, with necrotic debris and granulation tissue lining on the inner surface. cGISTs resembled their solid counterparts in terms of morphology and immunohistology but demonstrated fewer malignant parameters. c-kit or PDGFRα mutations were detected in eleven cases with the remaining being wild type for these two mutations. Although classified as intermediate or high (3 and 17, respectively) risk of recurrence according to modified National Institute of Health criterion, most patients with cGISTs experienced long-term recurrence-free survival without adjuvant imatinib. CONCLUSIONS: Cystic GISTs is a relatively indolent subset of GISTs with favorable prognoses and adjuvant imatinib should be a prudent consideration.


Assuntos
Cistos/patologia , Neoplasias Gastrointestinais/patologia , Tumores do Estroma Gastrointestinal/patologia , Adulto , Idoso , Cistos/diagnóstico por imagem , Cistos/genética , Feminino , Neoplasias Gastrointestinais/classificação , Neoplasias Gastrointestinais/diagnóstico por imagem , Neoplasias Gastrointestinais/genética , Tumores do Estroma Gastrointestinal/classificação , Tumores do Estroma Gastrointestinal/diagnóstico por imagem , Tumores do Estroma Gastrointestinal/genética , Humanos , Masculino , Pessoa de Meia-Idade , Mutação , Prognóstico
3.
Eur Radiol ; 29(3): 1074-1082, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30116959

RESUMO

OBJECTIVE: To develop and evaluate a radiomics nomogram for differentiating the malignant risk of gastrointestinal stromal tumours (GISTs). METHODS: A total of 222 patients (primary cohort: n = 130, our centre; external validation cohort: n = 92, two other centres) with pathologically diagnosed GISTs were enrolled. A Relief algorithm was used to select the feature subset with the best distinguishing characteristics and to establish a radiomics model with a support vector machine (SVM) classifier for malignant risk differentiation. Determinant clinical characteristics and subjective CT features were assessed to separately construct a corresponding model. The models showing statistical significance in a multivariable logistic regression analysis were used to develop a nomogram. The diagnostic performance of these models was evaluated using ROC curves. Further calibration of the nomogram was evaluated by calibration curves. RESULTS: The generated radiomics model had an AUC value of 0.867 (95% CI 0.803-0.932) in the primary cohort and 0.847 (95% CI 0.765-0.930) in the external cohort. In the entire cohort, the AUCs for the radiomics model, subjective CT findings model, clinical index model and radiomics nomogram were 0.858 (95% CI 0.807-0.908), 0.774 (95% CI 0.713-0.835), 0.759 (95% CI 0.697-0.821) and 0.867 (95% CI 0.818-0.915), respectively. The nomogram showed good calibration. CONCLUSIONS: This radiomics nomogram predicted the malignant potential of GISTs with excellent accuracy and may be used as an effective tool to guide preoperative clinical decision-making. KEY POINTS: • CT-based radiomics model can differentiate low- and high-malignant-potential GISTs with satisfactory accuracy compared with subjective CT findings and clinical indexes. • Radiomics nomogram integrated with the radiomics signature, subjective CT findings and clinical indexes can achieve individualised risk prediction with improved diagnostic performance. • This study might provide significant and valuable background information for further studies such as response evaluation of neoadjuvant imatinib and recurrence risk prediction.


Assuntos
Algoritmos , Tumores do Estroma Gastrointestinal/diagnóstico , Imageamento Tridimensional/métodos , Gradação de Tumores/métodos , Nomogramas , Tomografia Computadorizada por Raios X/métodos , Diagnóstico Diferencial , Feminino , Tumores do Estroma Gastrointestinal/classificação , Tumores do Estroma Gastrointestinal/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Período Pré-Operatório , Curva ROC , Máquina de Vetores de Suporte
4.
Scand J Gastroenterol ; 53(10-11): 1319-1327, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30353759

RESUMO

BACKGROUND: Although various risk classification systems for GISTs have been proposed, the optimum one remains uncertain. In the present study, we compared the prognostic stratification of different risk classification systems for GIST patients. METHODS: We reviewed those patients who were pathologically diagnosed with GISTs in the SEER database between 2009 and 2014. All patients were classified into different risk groups according to the NIH criteria, AFIP criteria and AJCC staging system, respectively. The prognostic differences between different risk groups were compared and clinicopathologic features were analyzed. RESULTS: The prognosis of small intestinal GISTs was not significantly different from that of gastric GISTs. For gastric GIST patients, there was no significant prognostic difference between very low risk and low risk group according to the NIH and AFIP criteria. However, the prognostic stratification for two groups could be improved by the AJCC staging system. For small intestinal GIST patients, the prognostic difference between low risk and intermediate risk group was not stratified properly by the NIH and AFIP criteria. However, the prognostic difference between two groups could reach statistical significance according to the AJCC staging system. Unlike gastric GISTs, tumor size was not identified as an independent factor influencing the prognosis of small intestinal GISTs. CONCLUSIONS: The AJCC staging system could provide a better prognostic stratification for GIST patients compared with the NIH and AFIP criteria, regardless of gastric or small intestinal tumor. However, primary tumor location and tumor size may be reconsidered and revised in the risk classification system.


Assuntos
Neoplasias Gastrointestinais/classificação , Tumores do Estroma Gastrointestinal/classificação , Estadiamento de Neoplasias/normas , Patologia Cirúrgica/normas , Idoso , China/epidemiologia , Feminino , Neoplasias Gastrointestinais/mortalidade , Neoplasias Gastrointestinais/patologia , Tumores do Estroma Gastrointestinal/mortalidade , Tumores do Estroma Gastrointestinal/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Prognóstico , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Programa de SEER , Análise de Sobrevida
5.
Ann Surg Oncol ; 25(5): 1133-1139, 2018 May.
Artigo em Inglês | MEDLINE | ID: mdl-29435684

RESUMO

BACKGROUND: In gastrointestinal stromal tumors (GISTs), rupture is a high-risk feature and an indication for adjuvant treatment; however, the independent impact of rupture on prognosis is uncertain and the term is inconsistently defined. In the present study, a previously proposed definition of 'tumor rupture' was applied on a population-based cohort of gastric GISTs. METHODS: Patients undergoing surgery for non-metastatic gastric GISTs from 2000 to 2015 were identified in the regional sarcoma database of Oslo University Hospital. Tumor rupture included spillage or fracture, piecemeal resection, incisional biopsy, blood-tinged ascites, gastric perforation, and microscopic adjacent infiltration. Minor defects of tumor integrity were not considered rupture, i.e. core needle biopsy, peritoneal tumor penetration, superficial peritoneal rupture, and R1 resection. Risk was assessed according to the modified National Institutes of Health consensus criteria. RESULTS: Among 242 patients, tumor rupture occurred in 22 patients and minor defects of tumor integrity occurred in 81 patients. Five-year recurrence-free survival (RFS) for patients with tumor rupture, minor defects of tumor integrity, and no defect was 37, 91, and 96%, respectively (p < 0.001). In the high-risk group, 5 year RFS for patients with rupture was 37%, versus 77% without rupture (hazard ratio 3.56, 95% confidence interval 1.57-8.08, p = 0.001). On multivariable analysis, tumor rupture and mitotic index were independently associated with recurrence. Of 13 patients who received adjuvant imatinib after tumor rupture, 11 relapsed. CONCLUSIONS: Tumor rupture according to the present definition was independently associated with recurrence. With tumor rupture, patients relapsed despite adjuvant treatment. Without rupture, prognosis was good, even in the high-risk group.


Assuntos
Tumores do Estroma Gastrointestinal/classificação , Tumores do Estroma Gastrointestinal/cirurgia , Neoplasias Gástricas/classificação , Neoplasias Gástricas/cirurgia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Antineoplásicos/uso terapêutico , Quimioterapia Adjuvante , Intervalo Livre de Doença , Feminino , Tumores do Estroma Gastrointestinal/tratamento farmacológico , Tumores do Estroma Gastrointestinal/patologia , Humanos , Mesilato de Imatinib/uso terapêutico , Masculino , Pessoa de Meia-Idade , Índice Mitótico , Terapia Neoadjuvante , Estudos Retrospectivos , Medição de Risco , Ruptura Espontânea/diagnóstico , Neoplasias Gástricas/tratamento farmacológico , Neoplasias Gástricas/patologia , Adulto Jovem
6.
Pathology ; 50(1): 37-48, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-28950990

RESUMO

Soft tissue tumours are relatively rare, but are diagnostically challenging as they comprise a large spectrum of diagnostic entities. Substantial advances have been made in recent years in identifying the underlying recurrent chromosomal and genomic alterations in a significant subset of soft tissue tumours, and this continues to enrich our understanding of the biological mechanisms of tumour development and progression. Ongoing validation and integration of these findings into existing pathological-diagnostic algorithms has led to re- or subclassification of diagnostic categories and will continue to shape a more nuanced (and hopefully clinically relevant) tumour classification system in the future. This review provides a selective overview of recent diagnostic or conceptual advances in the categories of peripheral nerve sheath tumours, vascular and adipocytic tumours, round cell and myogenic sarcomas, and gastrointestinal stromal tumours, as well as their underlying molecular mechanisms, some of which have been translated successfully into useful immunohistochemical stains. A thorough and critical validation of newly identified diagnostic markers-acknowledging the fact that some genetic alterations may not necessarily be tumour-specific-and ongoing correlation with clinical and prognostic implications will be necessary in this regard.


Assuntos
Neoplasias Gastrointestinais/diagnóstico , Tumores do Estroma Gastrointestinal/diagnóstico , Neoplasias da Bainha Neural/diagnóstico , Sarcoma/diagnóstico , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias Vasculares/diagnóstico , Biomarcadores Tumorais/análise , Neoplasias Gastrointestinais/classificação , Neoplasias Gastrointestinais/patologia , Tumores do Estroma Gastrointestinal/classificação , Tumores do Estroma Gastrointestinal/patologia , Humanos , Mutação , Neoplasias da Bainha Neural/classificação , Neoplasias da Bainha Neural/patologia , Prognóstico , Sarcoma/classificação , Sarcoma/patologia , Neoplasias de Tecidos Moles/classificação , Neoplasias de Tecidos Moles/patologia , Neoplasias Vasculares/classificação , Neoplasias Vasculares/patologia
7.
Endocr Relat Cancer ; 25(2): R49-R58, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29170162

RESUMO

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract, thought to derive from neoplastic outgrowth of the interstitial cells of Cajal. Building on recent advances in recognition, classification and diagnosis, the past two decades have seen a changing paradigm with molecular diagnostics and targeted therapies. KIT and PDGFRA mutations account for 85-90% of GIST carcinogenesis. However, the remaining 10-15% of GISTs, which until recently were called KIT/PDGFRA wild-type GISTs, have been found to have one of the several mutations, including in the SDHA, B, C, D, BRAF and NF1 genes. Though most of such GISTs are sporadic, a number of families with high incidence rates of GISTs and other associated clinical manifestations have been reported and found to harbor germline mutations in KIT, PDGFRA, SDH subunits and NF1 The goal of this review is to describe the mutations, clinical manifestations and therapeutic implications of syndromic and inherited GISTs in light of recent studies of their clinicopathologic range and pathogenesis.


Assuntos
Tumores do Estroma Gastrointestinal/classificação , Tumores do Estroma Gastrointestinal/genética , Tumores do Estroma Gastrointestinal/terapia , Humanos , Mutação , Neurofibromina 1/genética , Proteínas Proto-Oncogênicas c-kit/genética , Receptor alfa de Fator de Crescimento Derivado de Plaquetas/genética , Succinato Desidrogenase/genética , Síndrome
8.
Pathol Int ; 68(1): 7-11, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-29131458

RESUMO

We investigated the quantification of Ki-67 staining using digital image analysis (IA) as a complementary prognostic factor to the modified National Institutes of Health (NIH) classification in patients with gastrointestinal stromal tumor (GIST). We examined 92 patients, focusing on the correlation between age, sex, primary tumor site, tumor size, predominant histologic type, mitotic index, modified NIH classification (low/intermediate vs high), Ki-67 quantitation, and recurrence-free survival (RFS). We compared two IA processes for whole slide imaging (WSI) and manually captured image (MCI) methods. A Ki-67 quantitation cutoff was determined by receiver operator characteristics curve analysis. In the survival analysis, the high-risk group of a modified NIH classification, a mitotic count >5 per 20 high-powered fields, and Ki-67 cutoffs of ≥6% and ≥8% obtained by IA of the WSI and MCI methods, respectively, had an adverse impact on RFS. On multivariate analysis, each Ki-67 quantitation method strongly predicted prognosis, more strongly than the modified NIH classification. In addition, Ki-67 quantitation using IA of the MCI method could stratify low or intermediate risk and high risk GIST patients. Thus, IA is an excellent tool for quantifying Ki-67 to predict the prognosis of GIST patients, and this semiautomated approach may be preferable for patient care.


Assuntos
Biomarcadores Tumorais/análise , Tumores do Estroma Gastrointestinal/classificação , Interpretação de Imagem Assistida por Computador/métodos , Antígeno Ki-67/análise , Tumores do Estroma Gastrointestinal/patologia , Humanos , Índice Mitótico , Prognóstico
9.
Radiologe ; 57(11): 973-986, 2017 Nov.
Artigo em Alemão | MEDLINE | ID: mdl-29075871

RESUMO

Benign, intermediate and malignant soft tissue tumors can be differentiated histologically. Furthermore, the tumors can be subdivided according to their linear differentiation. In the new World Health Organization (WHO) classification of soft tissue tumors from 2013 changes have been made relating to the allocation of known entities, e. g. undifferentiated sarcomas have been formed into a new subgroup and are no longer assigned to the fibrohistiocytic tumors. The term malignant fibrous histiocytoma has been replaced by the undifferentiated sarcoma. Furthermore, two new subgroups were incorporated, the nerve sheath tumors and gastrointestinal stromal tumors. These were previously included in the tumor classification of other organ systems. These changes in the new classification are related to the rapid increase in knowledge of the genetics and the cell biology of soft tissue tumors. Malignant soft tissue tumors only represent 1% of all malignant tumors in adults. The largest subgroup of soft tissue tumors in adults is the adipocytic tumors. The liposarcoma, which belongs to this subgroup is one of the most common malignant soft tissue tumors in adults. In childhood malignant soft tissue tumors represent 15% of malignant tumors and rhabdomyosarcoma is the most common malignant soft tissue tumor.


Assuntos
Neoplasias de Tecidos Moles/epidemiologia , Neoplasias de Tecidos Moles/patologia , Adulto , Criança , Diagnóstico Diferencial , Tumores do Estroma Gastrointestinal/classificação , Tumores do Estroma Gastrointestinal/epidemiologia , Tumores do Estroma Gastrointestinal/patologia , Histiocitoma Fibroso Maligno/classificação , Histiocitoma Fibroso Maligno/epidemiologia , Histiocitoma Fibroso Maligno/patologia , Humanos , Lipoma/classificação , Lipoma/epidemiologia , Lipoma/patologia , Lipossarcoma/classificação , Lipossarcoma/epidemiologia , Lipossarcoma/patologia , Estadiamento de Neoplasias , Neoplasias da Bainha Neural/classificação , Neoplasias da Bainha Neural/epidemiologia , Neoplasias da Bainha Neural/patologia , Prevalência , Rabdomiossarcoma/classificação , Rabdomiossarcoma/epidemiologia , Rabdomiossarcoma/patologia , Sarcoma/classificação , Sarcoma/epidemiologia , Sarcoma/patologia , Neoplasias de Tecidos Moles/classificação , Terminologia como Assunto , Organização Mundial da Saúde
10.
Zhonghua Wei Chang Wai Ke Za Zhi ; 20(9): 1020-1024, 2017 Sep 25.
Artigo em Chinês | MEDLINE | ID: mdl-28900993

RESUMO

OBJECTIVE: To evaluate and compare the value of Modified NIH criteria and AFIP criteria for the risk classification of gastrointestinal stromal tumors (GIST). METHODS: Clinicopathological and follow-up data of 539 patients diagnosed as primary GIST with or without irregular tyrosine kinase inhibitors in the Nanfang Hospital(n=143), Sun Yat-sen University Cancer Center (n=138), Guangdong Provincial People's Hospital (n=102) and Wuhan Union Hospital (n=156) from January 2012 to December 2015 were retrospectively analyzed. Recurrence risks of these 539 patients were classified by the modified NIH criteria and AFIP criteria. Overall survival and tumor-free survival of patients with different risks were compared by Log-rank test and the accuracy of the two criteria in predicting postoperative recurrence was compared by receiver operating characteristic(ROC) curves. RESULTS: Of 539 GIST patients, 283 were male and 256 were female; the age was (56.5±12.5) years old; tumors of 390 cases (72.4%) located in the stomach; tumor diameter of 178 cases (33.0%) was more than 5 cm; nuclear division number of 164 cases(30.4%) was more than 5/50 high magnification. The mean follow-up time was (37.5±13.6) months. According to the modified NIH criteria, the mean overall survival time of patients with very low, low, intermediate, and high risk was 52.0, 57.0, 56.9 and 53.6 months respectively (P=0.002), and the mean tumor-free survival time was 56.0, 58.1, 58.2 and 51.2 months respectively (P=0.000). According to the AFIP criteria, the mean overall survival time of patients with very low, low, intermediate, and high risk was 54.1, 57.8, 55.5 and 52.0 months respectively(P=0.015), and the mean tumor-free survival time was 57.3, 56.6, 54.9 and 50.4 months respectively(P=0.000). While predicting the risk of postoperative recurrence, the ROC curve of AFIP criteria has a larger area under the curve compared to the curve of the modified NIH criteria(0.689 vs 0.641, P<0.05). CONCLUSION: Compared with the modified NIH criteria, AFIP criteria predicts the risk postoperative recurrence more accurately in GIST patients.


Assuntos
Tumores do Estroma Gastrointestinal/mortalidade , Recidiva Local de Neoplasia/mortalidade , Medição de Risco/métodos , Adulto , Idoso , Feminino , Tumores do Estroma Gastrointestinal/classificação , Tumores do Estroma Gastrointestinal/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Seleção de Pacientes , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida
11.
Arkh Patol ; 79(4): 48-55, 2017.
Artigo em Russo | MEDLINE | ID: mdl-28791999

RESUMO

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. Despite this, GISTS comprise about 2% in the structure of digestive tract cancers. They are usually localized in the stomach; however, they can be found in the small intestine and more rarely in the colon and esophagus. Although approximately 70% of the GISTs consist predominantly of spindle cells; the epithelioid cell tumors represent 20% of cases; there are also mixed variants. This variability in the morphological structure of GISTs complicates their diagnosis.


Assuntos
Neoplasias Gastrointestinais/patologia , Tumores do Estroma Gastrointestinal/patologia , Intestino Delgado/patologia , Estômago/patologia , Biomarcadores Tumorais/genética , Feminino , Neoplasias Gastrointestinais/classificação , Neoplasias Gastrointestinais/diagnóstico , Neoplasias Gastrointestinais/genética , Tumores do Estroma Gastrointestinal/classificação , Tumores do Estroma Gastrointestinal/diagnóstico , Tumores do Estroma Gastrointestinal/genética , Humanos , Masculino , Proteínas de Neoplasias/genética
12.
Zhonghua Zhong Liu Za Zhi ; 39(3): 184-189, 2017 Mar 23.
Artigo em Chinês | MEDLINE | ID: mdl-28316216

RESUMO

Objective: To evaluate the correlation between computed tomography (CT) features and malignancy risk category of small (≤5 cm) gastric stromal tumors (GST), in order to provide an image reference for preoperative assessment and intraoperative pathological diagnosis. Methods: Eighty-three patients with surgically and pathologically proven GST (≤5 cm) between January 2011 and November 2015 were recruited, and their clinical, pathological and CT data were retrospectively analyzed. According to the pathological results and malignancy risk category, the patients were divided into 2 groups, the benign biological behavior group (very low and low risk) and malignant biological behavior group (intermediate and high risk). The clinical, pathological and CT imaging findings of the two groups were analyzed. Based on the tumor diameter, the receiver operating characteristic curve (ROC) was applied to evaluate the sensitivity, specificity and the best cut-off point for distinguishing the malignancy risk between the two groups. Results: The lobulation and ulceration of the tumors presented statistically significant difference for the malignancy risk between the two groups (χ(2)=6.273 and 4.163, respectively; all P<0.05), but there was no significant difference in the sex, clinical symptoms, serum ferritin, tumor site, growth pattern, cystis degeneration and calcification (all P>0.05). No statistically significant differences were detected for the tumor CT value, arterial CT value, venous CT value, degrees of enhancement in arterial phase (DEAP), enhancement in portal venous phase (DEPP), and patient's age for distinguishing the malignancy risk between the two groups (all P>0.05). On the other hand, significant differences were found in the maximum diameter (Dmax) of tumor and the minimum diameter (Dmin) of tumor (t=-3.256 and -3.466, respectively; all P<0.05). When the cut-off point of Dmax was 1.6 cm, the area under the ROC curve, sensitivity and specificity were 0.704, 92.3% and 75.4%, respectively. When the cut-off point of Dmin was 1.5 cm, the area under the ROC curve, sensitivity and specificity were 0.713, 88.5% and 71.9%, respectively. Conclusion: CT features of the GST (≤5 cm) may predict, before surgery, the malignancy risk of small gastric stromal tumors, and provide the an image reference for preoperative assessment and intraoperative pathological diagnosis of the disease.


Assuntos
Tumores do Estroma Gastrointestinal/diagnóstico por imagem , Tumores do Estroma Gastrointestinal/patologia , Neoplasias Gástricas/diagnóstico por imagem , Neoplasias Gástricas/patologia , Feminino , Tumores do Estroma Gastrointestinal/classificação , Humanos , Masculino , Curva ROC , Estudos Retrospectivos , Sensibilidade e Especificidade , Neoplasias Gástricas/classificação , Tomografia Computadorizada por Raios X , Carga Tumoral
13.
Scand J Gastroenterol ; 52(3): 291-299, 2017 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-27817254

RESUMO

The purpose of the present review is to analyze the cytohistological and immunohistochemical characteristics of spindle-shaped mesenchymal gastrointestinal neoplams (MGNs), a group of unusual neoplastic conditions with different biological behavior. These tumors exhibit clinical pictures strictly related to the site of origin and dimensions, even if they appear generally with an intramural localization. This latter point may suggest an useful application of endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA), mainly followed by the cell-block procedure (CBP) in the differential diagnostic approach. First of all, we discuss the most common entity of MGNs represented by gastrointestinal stromal tumors (GISTs), analyzing the morphologic characteristics and stressing the strength of immunohistochemical algorithm for diagnostic purposes. Successively, we have reported the less common group of spindle-shaped MGNs comprehensive of those arising elsewhere the soft tissues, such as leiomyomas, leiomyosarcomas, schwannomas, inflammatory myofibroblastic tumor and intra-abdominal desmoid fibromatosis. Finally, very uncommon spindle-shaped MGNs, like clear cell, follicular dendritic cell, undifferentiated pleomorphic and radiation-induced sarcomas as well as spindle cell dedifferentiated liposarcomas, have been briefly mentioned.


Assuntos
Neoplasias Gastrointestinais/diagnóstico , Neoplasias Gastrointestinais/patologia , Tumores do Estroma Gastrointestinal/diagnóstico , Tumores do Estroma Gastrointestinal/patologia , Trato Gastrointestinal/patologia , Biópsia por Agulha Fina , Biologia Celular , Diagnóstico Diferencial , Neoplasias Gastrointestinais/classificação , Tumores do Estroma Gastrointestinal/classificação , Humanos , Imuno-Histoquímica
14.
Med. clín (Ed. impr.) ; 147(9): 405-409, nov. 2016. tab
Artigo em Espanhol | IBECS | ID: ibc-157031

RESUMO

La asociación de gastrointestinal stromal tumor (GIST, «tumor del estroma gastrointestinal») con otras neoplasias primarias en un mismo paciente es un hecho no solo frecuente, sino con un interés creciente en la literatura científica. Esta asociación tiene una enorme importancia tanto por el desafío clínico, diagnóstico y terapéutico como por el impacto pronóstico que implica. En las series publicadas existe una tendencia a agrupar a estos pacientes para determinar que los GIST asociados a otras neoplasias tienen características concretas y diferenciables. Por el contrario, no existe un consenso general ni una clasificación unificada. Esta clasificación sería de gran interés, pues permitiría unificar criterios, consensuar los grupos para comparar las distintas series y demostrar si realmente la etiopatogenia subyacente a ambos tumores y las características del propio GIST varían según el tipo de que se trate. Realizamos una revisión de la literatura médica actual y una propuesta de nueva clasificación para pacientes con GIST asociados a otros tumores (AU)


Additional primary malignancies in patients with gastrointestinal stromal tumor (GIST) is not only common but of growing interest in the scientific literature. This association is of great importance in terms of clinical challenge, diagnosis and therapy as well as for the prognosis impact it implies. In the published series there is a tendency to group these patients to determine the specific and distinguishable characteristics of GIST associated with other malignancies. On the other hand, there is no general consensus or unified classification. This classification would be of great interest, as it would unify criteria, agree groups to compare different series and demonstrate whether the aetiology underlying both tumours and the GIST’s own characteristics really vary according to the type in question. We undertook a medical literature review and proposed a new classification for patients with GIST associated with other tumours (AU)


Assuntos
Humanos , Tumores do Estroma Gastrointestinal/classificação , Neoplasias Primárias Múltiplas/classificação , Neoplasias Gastrointestinais/classificação , Tumores do Estroma Gastrointestinal/diagnóstico , Tumores do Estroma Gastrointestinal/etiologia , Neoplasias Primárias Múltiplas/etiologia , Neoplasias Primárias Múltiplas/diagnóstico , Neoplasias Gastrointestinais/diagnóstico , Neoplasias Gastrointestinais/etiologia , Prognóstico
15.
Pathol Int ; 66(8): 431-7, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27427238

RESUMO

Gastrointestinal stromal tumor (GIST) is a mesenchymal tumor of the gastrointestinal tract. Mutation of KIT and PDGFRA genes is implicated in the tumorigenesis. Approximately 10% of GISTs do not harbor mutation of these genes, and they are designated as "wild type" GIST. They are classified into succinate dehydrogenase (SDH)-deficient and non-SDH-deficient groups. SDH-deficient group includes Carney triad and Carney Stratakis syndrome. The patients are young women. Tumors occur in the antrum of the stomach, and tumor cells are epithelioid. Lymph node metastasis is frequent. The non-SDH-deficient group includes neurofibromatosis (NF) type 1 and GISTs with mutations of BRAF, KRAS, and PIK3CA and with the ETV6-NTRK3 fusion gene. GIST in NF occurs in the small intestine, and tumor cells are spindle shaped. GIST with BRAF mutation arises in the small intestine. Attention to the age, gender, family history and other neoplasms may raise the prediction of syndromic disease. Location of the tumor, morphology, and pleomorphism of the tumor cells are further informative. Lymphovascular invasion should be carefully evaluated. The determination of KIT expression is essential for the diagnosis. When wild type GIST is suspected, intensive genetic analysis is required. Further, a careful and long-time observation is recommended.


Assuntos
Tumores do Estroma Gastrointestinal/classificação , Tumores do Estroma Gastrointestinal/genética , Tumores do Estroma Gastrointestinal/patologia , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
16.
Med Clin (Barc) ; 147(9): 405-409, 2016 Nov 04.
Artigo em Espanhol | MEDLINE | ID: mdl-27431886

RESUMO

Additional primary malignancies in patients with gastrointestinal stromal tumor (GIST) is not only common but of growing interest in the scientific literature. This association is of great importance in terms of clinical challenge, diagnosis and therapy as well as for the prognosis impact it implies. In the published series there is a tendency to group these patients to determine the specific and distinguishable characteristics of GIST associated with other malignancies. On the other hand, there is no general consensus or unified classification. This classification would be of great interest, as it would unify criteria, agree groups to compare different series and demonstrate whether the aetiology underlying both tumours and the GIST's own characteristics really vary according to the type in question. We undertook a medical literature review and proposed a new classification for patients with GIST associated with other tumours.


Assuntos
Neoplasias Gastrointestinais/classificação , Tumores do Estroma Gastrointestinal/classificação , Neoplasias Primárias Múltiplas/classificação , Neoplasias Gastrointestinais/diagnóstico , Neoplasias Gastrointestinais/etiologia , Tumores do Estroma Gastrointestinal/diagnóstico , Tumores do Estroma Gastrointestinal/etiologia , Humanos , Neoplasias Primárias Múltiplas/diagnóstico , Neoplasias Primárias Múltiplas/etiologia , Prognóstico
17.
Rev. Col. Méd. Cir. Guatem ; 155(1): 42-45, jul. 2016.
Artigo em Espanhol | LILACS | ID: biblio-835552

RESUMO

Antecedentes: Los tumores del estroma gastrointestinal (TEG) son los tumores mesenquimales más comunes del tracto gastrointestinal (TGI), se considera que surgen de las células de Cajal, ocurren principalmente en adultos mayores (60-65 años) y se localizan en estómago (50%-70%), intestino delgado (25%-35%), colon-recto (5%-10%) y esófago (<5%). La mayoría se presenta de manera esporádica y hasta el 70% son clínicamente sintomáticos. El diagnóstico definitivo se realiza en el estudio anatomopatológico. El pronóstico de estos tumores se determina por el tamaño, recuento mitótico y localización del tumor, clasificandose: riesgo muy bajo, riesgo bajo, riesgo intermedio y riesgo alto. La cirugía es la opción terapéutica principal...


Background: Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract (GI), areconsidered to arise from the Cajal cells. Theyoccur mainly in older adults, 60-65 years. Theypresent in the stomach (50%-70%), small intestine (25%-35%), colon and rectum (5%-10%)and esophagus (<5%). Most GISTs are sporadicand are clinically symptomatic. The definitivediagnosis is made through anatomic pathology study. To determine the prognosis of this type of tumors we use the size, mitotic count and location of the tumor, classified them in: very low risk, low risk, intermediate risk and high risk. Surgery is the main treatment...


Assuntos
Humanos , Tumores do Estroma Gastrointestinal/cirurgia , Tumores do Estroma Gastrointestinal/classificação , Tumores do Estroma Gastrointestinal/diagnóstico
18.
JAMA Oncol ; 2(7): 922-8, 2016 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-27011036

RESUMO

IMPORTANCE: Wild-type (WT) gastrointestinal stromal tumors (GISTs), which lack KIT and PDGFRA gene mutations, are the primary form of GIST in children and occasionally occur in adults. They respond poorly to standard targeted therapy. Better molecular and clinical characterization could improve management. OBJECTIVE: To evaluate the clinical and tumor genomic features of WT GIST. DESIGN, SETTING, AND PARTICIPANTS: Patients enrolled in an observational study at the National Institutes of Health starting in 2008 and were evaluated in a GIST clinic held once or twice yearly. Patients provided access to existing medical records and tumor specimens. Self-referred or physician-referred patients younger than 19 years with GIST or 19 years or older with known WT GIST (no mutations in KIT or PDGFRA) were recruited; 116 patients with WT GIST were enrolled, and 95 had adequate tumor specimen available. Tumors were characterized by immunohistochemical analysis (IHC) for succinate dehydrogenase (SDH) subunit B, sequencing of SDH genes, and determination of SDHC promoter methylation. Testing of germline SDH genes was offered to consenting patients and families. MAIN OUTCOMES AND MEASURES: For classification, tumors were characterized by SDHA, B, C, or D (SDHX) mutations and other genetic and epigenetic alterations, including presence of mutations in germline. Clinical characteristics were categorized. RESULTS: Wild-type GIST specimens from 95 patients (median age, 23 [range, 7-78] years; 70% female) were classified into 3 molecular subtypes: SDH-competent (n = 11), defined by detection of SDHB by IHC; and 2 types of SDH-deficient GIST (n = 84). Of SDH-deficient tumors, 63 (67%) had SDH mutations, and in 31 of 38 (82%), the SDHX mutation was also present in germline. Twenty-one (22%) SDH-deficient tumors had methylation of the SDHC promoter leading to silencing of expression. Mutations in known cancer-associated pathways were identified in 9 of 11 SDH-competent tumors. Among patients with SDH-mutant tumors, 62% were female (39 of 63), median (range) age was 23 (7-58) years, and approximately 30% presented with metastases (liver [12 of 58], peritoneal [6 of 58], lymph node [15 of 23]). SDHC-epimutant tumors mostly affected young females (20 of 21; median [range] age, 15 [8-50] years), and approximately 40% presented with metastases (liver [7 of 19], peritoneal [1 of 19], lymph node [3 of 8]). SDH-deficient tumors occurred only in the stomach and had an indolent course. CONCLUSIONS AND RELEVANCE: An observational study of WT GIST permitted the evaluation of a large number of patients with this rare disease. Three molecular subtypes with implications for prognosis and clinical management were identified.


Assuntos
Complexo II de Transporte de Elétrons/genética , Tumores do Estroma Gastrointestinal/genética , Proteínas de Membrana/genética , Succinato Desidrogenase/genética , Adolescente , Adulto , Idoso , Criança , Metilação de DNA/genética , Feminino , Tumores do Estroma Gastrointestinal/classificação , Tumores do Estroma Gastrointestinal/patologia , Mutação em Linhagem Germinativa/genética , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Regiões Promotoras Genéticas , Proteínas Proto-Oncogênicas c-kit/genética , Receptor alfa de Fator de Crescimento Derivado de Plaquetas/genética
19.
Hepatogastroenterology ; 62(140): 919-23, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-26902028

RESUMO

BACKGROUND/AIMS: Adjuvant treatment with imatinib mesylate (IM) improves survival of patients with a high risk of recurrence of gastrointestinal stromal tumors (GISTs). However, the optimal adjuvant treatment strategy remains unknown. Thus, this study aimed to identify patients who do not require adjuvant IM treatment using the Ki-67 labeling index (LI). METHODOLOGY: The Ki-67 LI was calculated in 59 patients with gastric GISTs. A new risk classification using the Ki-67LI and tumor size was established using cut-off values determined by receiver operating characteristic analysis. This Ki-67 classification was compared with the modified Fletcher classification (MF). RESULTS: The best cut-off values for the Ki-67LI and tumor size were 8.6% and 80 mm, respectively. According to the MF, 42, 10, and 7 patients were categorized as low, intermediate, and high risk, respectively, while the Ki-67 classification rated 38, 17, and 4 patients as low, intermediate, and high risk, respectively. The 7 patients classified as high risk by MF were graded high risk (4 patients) and intermediate risk (3 patients) by the Ki-67 classification. Recurrence was not observed in patients classed intermediate risk by the Ki-67 classification. CONCLUSIONS: The Ki-67 classification is helpful for identifying patients for whom adjuvant IM treatment is not necessary.


Assuntos
Tumores do Estroma Gastrointestinal/metabolismo , Antígeno Ki-67/metabolismo , Recidiva Local de Neoplasia , Neoplasias Gástricas/metabolismo , Adulto , Idoso , Idoso de 80 Anos ou mais , Antineoplásicos/uso terapêutico , Quimioterapia Adjuvante , Estudos de Coortes , Feminino , Gastrectomia , Tumores do Estroma Gastrointestinal/classificação , Tumores do Estroma Gastrointestinal/tratamento farmacológico , Tumores do Estroma Gastrointestinal/patologia , Humanos , Mesilato de Imatinib/uso terapêutico , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Índice Mitótico , Prognóstico , Estudos Retrospectivos , Medição de Risco , Neoplasias Gástricas/classificação , Neoplasias Gástricas/tratamento farmacológico , Neoplasias Gástricas/patologia , Carga Tumoral
20.
Pathologe ; 36(1): 89-91, 2015 Feb.
Artigo em Alemão | MEDLINE | ID: mdl-25413680

RESUMO

The correct histopathological classification of a gastric mesenchymal tumor as a schwannoma is essential because in contrast to gastrointestinal stromal tumors (GIST) it is a definitive benign neoplasm which can be sufficiently treated by in sano (R0) resection. A (partial) gastrectomy is unnecessary. A clear radiological or sonographical differentiation between a schwannoma and GIST is not possible. The histomorphological and immunohistochemical features of this tumor entity are described.


Assuntos
Tumores do Estroma Gastrointestinal/patologia , Neurilemoma/patologia , Neoplasias Gástricas/patologia , Diagnóstico Diferencial , Endossonografia , Mucosa Gástrica/patologia , Mucosa Gástrica/cirurgia , Tumores do Estroma Gastrointestinal/classificação , Tumores do Estroma Gastrointestinal/cirurgia , Gastroscopia , Humanos , Masculino , Pessoa de Meia-Idade , Neurilemoma/classificação , Neurilemoma/cirurgia , Antro Pilórico/patologia , Antro Pilórico/cirurgia , Estômago/patologia , Estômago/cirurgia , Neoplasias Gástricas/classificação , Neoplasias Gástricas/cirurgia
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