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1.
BMC Cancer ; 21(1): 234, 2021 Mar 06.
Artigo em Inglês | MEDLINE | ID: mdl-33676450

RESUMO

BACKGROUND: Neuroendocrine tumours (NETs) arise from hormone-producing or nervous system cells and can develop from anywhere in the body. They have heterogeneous origins from skin to gastrointestinal track and a complicated histology. Thus, there is an inevitable need for genomic profiling to determine the exact genetics of each tumour for prognosis and treatment strategies to overcome the disease's complexity. For this purpose, next-generation-sequencing (NGS) is the most reliable methodology for both germ-line and somatic studies to make a clinical diagnosis. In this study, we analyse liquid biopsies, formalin fixed paraffin embedded (FFPE) tissues, and peripheral blood samples for their ability to provide information for actionability. METHODS: A customized multi-gene panel comprised of Succinate Dehydrogenase Complex Iron Sulfur Subunit B (SDHB), Succinate Dehydrogenase Complex Subunit C (SDHC), Cell Division Cycle 73(CDC73), Calcium Sensing Receptor (CASR), Platelet Derived Growth Factor Receptor Alpha (PDGFRA), Succinate Dehydrogenase Complex Flavoprotein Subunit A (SDHA), Ret Proto-Oncogene (RET), Succinate Dehydrogenase Complex Assembly Factor 2(SDHAF2), Menin 1(MEN1), Succinate Dehydrogenase Complex Subunit D (SDHD), MYC Associated Factor X (MAX) and Protein Kinase CAMP-Dependent Type I Regulatory Subunit Alpha (PRKAR1A) genes was constructed to assess multiple specimen types including: 3 liquid biopsies, 6 FFPE tissues, and 26 peripheral blood samples from 35 unique NET patients. Quality-control and bioinformatics analyses were performed using QCI-Analyze and QCI-Interpret. RESULTS: The three liquid biopsies and the 6 FFPE tissue samples were evaluated for somatic mutations; while the 26 peripheral blood samples were analysed using the germ-line pipeline. Five (55.6%) of the nine patients that were studied for somatic changes carried actionable mutations related to therapy sensitivities. Through the germ-line studies, we observed a 50% positivity rate for disease predisposition with 16 variants classified according to ACMG (American College of Medical Genetics) Standards and Guidelines. CONCLUSIONS: Genomic profiling medicine is an emerging area of clinical oncology and has become crucial for disease and patient management by providing a precision approach; this is especially true for rare diseases including rare cancers such as NETs. Notably, this study emphasized the relevance of multiple distinctive biological sample types for use in the genetic testing of cancers to help with the choice of therapy to maximize the likelihood of a positive clinical outcome.


Assuntos
Biomarcadores Tumorais/genética , Ácidos Nucleicos Livres/genética , Testes Genéticos , Tumores Neuroendócrinos/diagnóstico , Adolescente , Adulto , Idoso , Biomarcadores Tumorais/sangue , Ácidos Nucleicos Livres/sangue , Feminino , Predisposição Genética para Doença , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Biópsia Líquida , Masculino , Pessoa de Meia-Idade , Mutação , Tumores Neuroendócrinos/sangue , Tumores Neuroendócrinos/genética , Tumores Neuroendócrinos/terapia , Medicina de Precisão/métodos , Adulto Jovem
2.
BMC Cancer ; 21(1): 279, 2021 Mar 16.
Artigo em Inglês | MEDLINE | ID: mdl-33726691

RESUMO

BACKGROUND: Thymic tumors are unusual neoplasms, representing 0.2 to 1.5% of tumors in humans, but correspond to 20% of mediastinal tumors and 50% of those that occur in the anterior mediastinum. They tend to appear around the fourth and fifth decades of life without gender predilection. Up to 30% of patients are asymptomatic, therefore many are incidentally diagnosed. Radical thymectomy is the treatment of choice with high survival rates when detected in the early stages. METHODS: This was a retrospective descriptive study, including 18 adult patients' diagnosis of thymic neoplasm, who were managed with surgical resection from 2011 to 2019. Information about demographics, clinical characteristics, imaging findings, surgical and medical management, plus histological findings was obtained and reported. RESULTS: 18 patients with thymic tumors were included, of which specific histologic studies reveled thymomas, carcinomas, neuroendocrine tumors, thymolipoma and thymic cyst. Mean age was 52.7 years, with a predominance of male population. The main symptom was dyspnea, followed by cough and chest pain. Paraneoplastic syndromes such as myasthenia gravis, aplastic anemia and Cushing syndrome were reported. 89% of cases were treated by radical thymectomy alone, while only 2 cases required chemotherapy and radiotherapy. There were no surgical complications. Mean hospital stay length was 11. 9 days, with only 1 mortality during hospital admission. 5-year survival rate was 81%. CONCLUSIONS: The treatment of choice is radical thymectomy, which has been shown to positively impact patient mortality. Early detection is key to improve patient outcomes.


Assuntos
Síndromes Paraneoplásicas/epidemiologia , Timectomia , Timo/patologia , Neoplasias do Timo/cirurgia , Idoso , Carcinoma/complicações , Carcinoma/diagnóstico , Carcinoma/mortalidade , Carcinoma/cirurgia , Colômbia/epidemiologia , Feminino , Humanos , Tempo de Internação/estatística & dados numéricos , Lipoma/complicações , Lipoma/diagnóstico , Lipoma/mortalidade , Lipoma/cirurgia , Masculino , Pessoa de Meia-Idade , Tumores Neuroendócrinos/complicações , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/mortalidade , Tumores Neuroendócrinos/cirurgia , Síndromes Paraneoplásicas/etiologia , Estudos Retrospectivos , Taxa de Sobrevida , Timoma/complicações , Timoma/diagnóstico , Timoma/mortalidade , Timoma/cirurgia , Timo/diagnóstico por imagem , Timo/cirurgia , Neoplasias do Timo/complicações , Neoplasias do Timo/diagnóstico , Neoplasias do Timo/mortalidade
3.
Cancer Treat Rev ; 94: 102168, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33730627

RESUMO

OBJECTIVE: This systematic review and meta-analysis aimed to develop an evidence-based summary of current knowledge of bone metastases (BMs) in neuroendocrine neoplasms (NENs), inform diagnosis and treatment and standardise management between institutions. METHODS: PubMed, Medline, EMBASE and meeting proceedings were searched for eligible studies reporting data on patients with BMs and NENs of any grade of differentiation and site; poorly-differentiated large/small cell lung cancer were excluded. Data were extracted and analysed using STATA v.12. Meta-analysis of proportions for calculation of estimated pooled prevalence of BM and calculation of weighted pooled frequency and weighted pooled mean for other variables of interest was performed . RESULTS: A total of 149 studies met the eligibility criteria. Pooled prevalence of BMs was 18.4% (95% CI 15.4-21.5). BMs were mainly metachronous with initial diagnosis of NEN (61.2%) and predominantly osteoblastic; around 61% were multifocal, with a predisposition in axial skeleton. PET/CT seemed to provide (together with MRI) the highest sensitivity and specificity for BM detection. Almost half of patients (46.4%) reported BM-related symptoms: pain (66%) and skeletal-related events (SREs, fracture/spinal cord compression) (26.2%; weightedweighted mean time-to-SRE 9.9 months). Management of BMs was multimodal [bisphosphonates and bone-modifying agents (45.2%), external beam radiotherapy (34.9%), surgery (14.8%)] and supported by little evidence. Overall survival (OS) from the time of diagnosis of BMs was long [weighted mean 50.9 months (95% CI 40.0-61.9)]. Patients with BMs had shorter OS [48.8 months (95% CI 37.9-59.6)] compared to patients without BMs [87.4 months (95% CI 74.9-100.0); p = 0.001]. Poor performance status and BM-related symptoms were also associated with worse OS. CONCLUSIONS: BMs in patients with NENs remain underdiagnosed and undertreated. Recommendations for management of BMs derived from current knowledge are provided. Prospective studies to inform management are required.


Assuntos
Neoplasias Ósseas/secundário , Neoplasias Ósseas/terapia , Tumores Neuroendócrinos/patologia , Tumores Neuroendócrinos/terapia , Neoplasias Ósseas/diagnóstico , Humanos , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/secundário
4.
Medicine (Baltimore) ; 100(6): e24635, 2021 Feb 12.
Artigo em Inglês | MEDLINE | ID: mdl-33578581

RESUMO

RATIONALE: Duodenal neuroendocrine tumor (d-NET) is a rare tumor originating in the neuroendocrine system. The clinical manifestations of d-NET are similar to those of other digestive tract tumors, resulting in a lack of specificity and complex clinical symptoms. PATIENT CONCERNS: A 55-year-old female patient was admitted to our hospital with a chief complaint of an abdominal mass that had been present for more than 4 months. DIAGNOSES: The upper abdomen enhanced computed tomography scan showed an uneven density mass across the upper abdomen, and the tumor size was approximately 6.2 × 5.8 cm with obvious visible enhancement present in 1 area and a cystic nonenhanced area. The postoperative pathology showed the tumor cells to be positive for chromogranin, synaptophysin, cytokeratin, CD56 (partial weak), negative for vimentin, CD117, DOG-1, CD34, S-100, SMA, desmin, and Ki-67 approximately 2%, which confirmed the diagnosis of d-NETs. INTERVENTIONS: We preferred laparoscopic surgical exploration, but the tumor started at the ascending part of the duodenum and involved the mesenteric artery. As the branches of the superior mesenteric artery were intertwined with the tumor, it was difficult to operate with the endoscope, so we converted to open laparotomy. The postoperative pathology revealed the presence of d-NET. OUTCOMES: The patient recovered uneventfully and was discharged after the operation. One-month and 3-month follow-up after surgery, showed no evidence of recurrence. LESSONS: Radiological imaging studies are insufficient for the differential diagnosis of abdominal mass from other diseases, whereas surgery is the only radical treatment method, and the preferred surgical method is still active radical resection of the tumor.


Assuntos
Neoplasias Duodenais/diagnóstico , Tumores Neuroendócrinos/diagnóstico , Diagnóstico Diferencial , Neoplasias Duodenais/diagnóstico por imagem , Neoplasias Duodenais/cirurgia , Feminino , Humanos , Laparotomia , Pessoa de Meia-Idade , Tumores Neuroendócrinos/diagnóstico por imagem , Tumores Neuroendócrinos/cirurgia , Tomografia Computadorizada por Raios X
5.
Intern Med ; 60(3): 373-378, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33518610

RESUMO

A 60-year-old man had received octreotide for a metastatic neuroendocrine tumor (NET) in the rectum. Computed tomography and ultrasonography revealed a cardiac tumor, diffuse thickness of the ventricular wall and pericardial effusion, which was diagnosed as cardiac metastasis. The metastatic lesions continued to grow despite the alteration of chemotherapy, and the patient complained of repeated syncope and was admitted to our hospital at 11 months after the diagnosis of cardiac metastasis. An electrocardiogram during syncope showed sustained ventricular tachycardia, which was considered to be caused by the cardiac metastasis. We herein report a case of NET with cardiac metastasis which caused lethal arrhythmia along with a review of the pertinent literature.


Assuntos
Neoplasias Cardíacas , Tumores Neuroendócrinos , Neoplasias Pancreáticas , Neoplasias Retais , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/etiologia , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Tumores Neuroendócrinos/complicações , Tumores Neuroendócrinos/diagnóstico
6.
BMJ Case Rep ; 14(2)2021 Feb 04.
Artigo em Inglês | MEDLINE | ID: mdl-33541948

RESUMO

In this manuscript, we describe a rare case of neuroendocrine tumour metastatic to the testicle, presenting with testicular mass as an isolated symptom. We describe the investigations and management leading us to this uncommon histological diagnosis and explore its significance and impact on further management.


Assuntos
Tumores Neuroendócrinos , Neoplasias Testiculares/diagnóstico , Neoplasias Testiculares/cirurgia , Testículo/patologia , Colectomia , Humanos , Masculino , Pessoa de Meia-Idade , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/patologia , Tumores Neuroendócrinos/secundário , Orquiectomia , Compostos Organometálicos , Tomografia por Emissão de Pósitrons , Neoplasias Testiculares/patologia , Ultrassonografia
7.
Endocr Pract ; 27(2): 152-157, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33616046

RESUMO

OBJECTIVE: Adrenocorticotropic hormone-producing pancreatic neuroendocrine neoplasm (ACTHoma) is an exceedingly rare type of pancreatic neuroendocrine neoplasm (pNEN) that often causes ectopic adrenocorticotropic hormone syndrome. These neoplasms have been found to be very aggressive and challenging to treat. The current systematic review aimed to analyze the clinical features, immunohistochemical characteristics, diagnosis, therapy, and prognosis of ACTHoma. METHODS: A systematic review of the English- and Chinese-language literature was performed. PubMed, EMBASE, and Wanfang databases were searched to identify articles about ACTHoma in accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines. RESULTS: A total of 210 studies encompassing 336 patients diagnosed with ACTHoma were selected for the systematic review, including 16 Chinese patients. CONCLUSION: ACTHoma was more common in women (66.4%), and the mean age was 44.7 years. Tumors were generally large, and the mean tumor size was 4.43 cm. The incidence of clinical manifestations was: hypokalemia, 69.3%; diabetes, 63.2%; weakness, 60.1%, hypertension, 56.4%; moon face 41.1%; and edema, 37.4%. These tumors are more commonly found in the tail of pancreas, and the most frequent site of metastasis was the liver. The pNENs or other functioning pNENs could evolve into ACTHoma. ACTHoma is a very rare disease, and the mean follow-up time was 28.3 months.


Assuntos
Síndrome de ACTH Ectópico , Tumores Neuroendócrinos , Neoplasias Pancreáticas , Síndrome de ACTH Ectópico/diagnóstico , Síndrome de ACTH Ectópico/epidemiologia , Hormônio Adrenocorticotrópico , Adulto , Feminino , Humanos , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/epidemiologia , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/epidemiologia , Prognóstico
8.
Medicine (Baltimore) ; 100(2): e24054, 2021 Jan 15.
Artigo em Inglês | MEDLINE | ID: mdl-33466160

RESUMO

RATIONALE: Primary neuroendocrine tumors (NETs) of the retroperitoneum are extremely rare. The purpose of this case report is to highlight the unusual growth pattern and radiologic features of primary retroperitoneal NETs. PATIENT CONCERNS: A 46-year-old woman was found to have a retroperitoneal cystic and solid mass during a physical checkup. DIAGNOSES: The mass was mainly multiseptated in the cystic portion and had a bead-like, lobulated appearance. The solid portion showed restricted diffusion on diffusion-weighted imaging and obvious homogeneous enhancement. The cystic portion showed ring-like and septal enhancement. The patient was diagnosed with a grade 2 (G2) NET of the retroperitoneum after surgery. INTERVENTIONS: The patient underwent resection of the large retroperitoneal tumor. OUTCOMES: The patient returned 20 months later with tumor recurrence in the retroperitoneum. She was enrolled in a clinical trial for sulfatinib, and the mass was considerably reduced in size after 4 months. During a nearly 1.5-year follow-up, the mass gradually became slightly enlarged. The expression of somatostatin receptor 2 (SSTR2) was detected, and somatuline was administered as the current treatment. LESSONS SUBSECTIONS: When a retroperitoneal mass presents as a well-defined cystic or solid hypervascular mass with a fibrous capsule, a primary retroperitoneal NET should be considered in the differential diagnosis.


Assuntos
Neoplasias Císticas, Mucinosas e Serosas/diagnóstico , Tumores Neuroendócrinos/diagnóstico , Neoplasias Retroperitoneais/diagnóstico , Diagnóstico Diferencial , Feminino , Humanos , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/patologia , Neoplasias Císticas, Mucinosas e Serosas/patologia , Tumores Neuroendócrinos/patologia , Neoplasias Retroperitoneais/patologia , Espaço Retroperitoneal/patologia
9.
Medicine (Baltimore) ; 100(2): e24223, 2021 Jan 15.
Artigo em Inglês | MEDLINE | ID: mdl-33466202

RESUMO

ABSTRACT: Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are increasing in incidence. Clinicians urgently need a method that can effectively predict the prognosis of GEP-NENs.A total of 14770 GEP-NENs patients with pathologically confirmed between 1975 and 2016 were obtained from the surveillance, epidemiology, and end results database. All the patients were divided into primary (n = 10377) and validation (n = 4393) cohorts based on the principle of random grouping. Multivariate Cox proportional hazards proportional hazards regression analysis was performed to evaluate predictors associated with overall survival, and a nomogram was constructed based on the primary cohort. An independent external validation cohort and comparison with the eighth edition American Joint Committee on Cancer TNM staging system were subsequently used to assess the predictive performance of the nomogram.The multivariate Cox model indicated that age, tumour differentiation, and distant metastases were independent predictors associated with overall survival. With respect to the primary cohort, the nomogram exhibited better discriminatory power than the TNM classification (C-index: 0.821 vs 0.738). Discrimination was also superior to that of TNM classification for the validation cohort (C-index: 0.823 vs 0.738). The calibrated nomogram predicted 3- and 5-years survival rate that closely corresponded to the actual survival rate.This study developed and validated a prognostic nomogram applied to patients with GEP-NENs, which may help clinicians make reasonable prognostic judgments and treatment plans to a certain extent.


Assuntos
Neoplasias Intestinais/diagnóstico , Neoplasias Intestinais/mortalidade , Estadiamento de Neoplasias/métodos , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/mortalidade , Nomogramas , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/mortalidade , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/mortalidade , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Valor Preditivo dos Testes , Prognóstico , Modelos de Riscos Proporcionais , Reprodutibilidade dos Testes , Taxa de Sobrevida
10.
APMIS ; 129(4): 186-194, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33417719

RESUMO

Syntaxin-1 (STX1) is a recently described highly sensitive and specific neuroendocrine marker. We evaluated the applicability of STX1 as an immunohistochemical marker in pulmonary neuroendocrine neoplasms (NENs). We compared STX1 with established neuroendocrine markers, including insulinoma-associated protein 1 (INSM1). Typical carcinoids (n = 33), atypical carcinoids (n = 7), small cell lung carcinomas ([SCLCs] n = 30), and large cell neuroendocrine lung carcinomas (n = 17) were immunostained using tissue microarray for STX1, chromogranin A, synaptophysin, CD56, and INSM1. Eighty-four of eighty-seven (96.5%) NENs showed STX1 positivity. Carcinoids and LCNECs typically presented a combined strong membranous and weak cytoplasmic staining pattern; cytoplasmic expression was predominately observed in SCLCs. The sensitivity of STX1 was 90% in SCLCs and 100% in typical carcinoids, atypical carcinoids, and large cell neuroendocrine lung carcinomas. The overall sensitivity of STX1 in pulmonary NENs was 96.6%, and the sensitivity of the other markers was as follows: chromogranin A (85.2%), synaptophysin (85.2%), CD56 (92.9%), and INSM1 (97.7%). STX1 was found to be an excellent neuroendocrine marker of pulmonary NENs, with sensitivity and specificity surpassing that of classic markers. We propose a panel of STX1 and INSM1 for the routine immunohistochemical workup of pulmonary NENs.


Assuntos
Biomarcadores Tumorais/análise , Neoplasias Pulmonares/diagnóstico , Tumores Neuroendócrinos/diagnóstico , Proteínas Repressoras/biossíntese , Sintaxina 1/biossíntese , Feminino , Humanos , Masculino , Proteínas Repressoras/análise , Sensibilidade e Especificidade , Sintaxina 1/análise
13.
Medicine (Baltimore) ; 99(50): e23271, 2020 Dec 11.
Artigo em Inglês | MEDLINE | ID: mdl-33327249

RESUMO

BACKGROUND: Mixed neuroendocrine nonneuroendocrine neoplasms (MiNENs) originating from the biliary system (gallbladder, biliary tract, or ampulla of Vater) are extremely rare and have not been discussed in detail or systematically. We aimed to present the demographics, clinicopathological characteristics, management, and prognostic factors of biliary MiNENs. METHODS: A systematic search of electronic biomedical databases (Web of Science, PUBMED, and Embase) was performed to identify eligible studies. Survival was analyzed with the Kaplan-Meier method. Log-rank tests were used to evaluate the differences between groups, and the effects of various clinical and histopathological features on prognosis were analyzed by univariate and multivariate Cox regression. RESULTS: Fifty-three publications (patients, n = 67) were included. The median overall survival time was 21.0 months. Fifty-one patients (76.1%) underwent radical surgery and median survival for 41 months (P < .001). Twenty-two patients who received adjuvant radiochemotherapy treatment after radical surgery had a median survival for 43 months (P = .076). Radical resection (P < .001), Ki-67 index (P = .011), tumor stage (P < .001), neuroendocrine (NEC) grade (P = .011), and non-NEC grade (P = .017) were independent statistically significant prognostic factors according to univariate analysis; radical resection (P = .010) and small morphological subtype (P = .036) were independent statistically significant prognostic factors associated with higher overall survival according to multivariate analysis, and radical resection (P = .005) and age < 65 years (P = .026) were associated with higher recurrence free survival time. CONCLUSION: Radical resection is essential for long-term survival. Aggressive multimodality therapy with adjuvant radiochemotherapy and biotherapy may improve survival of biliary MiNENs. Further randomized controlled trials are needed to determine the standard treatment.


Assuntos
Neoplasias do Sistema Biliar/patologia , Carcinoma Neuroendócrino/patologia , Tumor Misto Maligno/patologia , Tumores Neuroendócrinos/patologia , Sistema Biliar/patologia , Neoplasias do Sistema Biliar/diagnóstico , Neoplasias do Sistema Biliar/terapia , Carcinoma Neuroendócrino/diagnóstico , Carcinoma Neuroendócrino/terapia , Humanos , Tumor Misto Maligno/diagnóstico , Tumor Misto Maligno/terapia , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/terapia , Prognóstico , Análise de Sobrevida
14.
PLoS One ; 15(12): e0244504, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33382770

RESUMO

BACKGROUND AND AIMS: MicroRNAs (miRNAs) are profoundly involved into the pathophysiology of manifold cancers. Recent data suggested a pivotal role of miRNAs as biomarkers in different biological processes including carcinogenesis. However, their role in neuroendocrine tumors (NETs) is only poorly understood. METHODS: We determined circulating levels of miR-21 and miR-223 in 45 samples from patients with NET treated between 2010 and 2019 at our department and compared them to healthy controls. Results were correlated with clinical records. RESULTS: In the total cohort of Patients with NET, miR-223 presented significantly lower levels compared to healthy control samples. In contrast, levels of miR-21 indicated no significant changes between the two groups. Interestingly, despite being significantly downregulated in all NET patients, concentrations of miR-223 were independent of clinical or histopathological factors such as proliferation activity according to Ki-67 index, tumor grading, TNM stage, somatostatin receptor expression, presence of functional/ non-functional disease or tumor relapse. Moreover, in contrast to data from recent publications analyzing other tumor entities, levels of miR-223 serum levels did not reflect prognosis of patients with NET. CONCLUSION: Lower concentrations of circulating miR-223 rather reflect the presence of NET itself than certain tumor characteristics. The value of miR-223 as a biomarker in NET might be limited to diagnostic, but not prognostic purposes.


Assuntos
Biomarcadores Tumorais/sangue , MicroRNAs/sangue , Tumores Neuroendócrinos/diagnóstico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Casos e Controles , Feminino , Seguimentos , Voluntários Saudáveis , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Tumores Neuroendócrinos/sangue , Tumores Neuroendócrinos/mortalidade , Tumores Neuroendócrinos/terapia , Prognóstico , Adulto Jovem
15.
Acta Gastroenterol Belg ; 83(4): 643-653, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33321023

RESUMO

Background and study aims: Neuroendocrine neoplasms (NENs) are relatively rare, with marked clinical and biological heterogeneity. Consequently, many controversial areas remain in diagnosis and optimal treatment stratification for NEN patients. We wanted to describe current clinical practice regarding controversial NEN topics and stimulate critical thinking and mutual learning among a Belgian multidisciplinary expert panel. Patients and methods: A 3-round, Delphi method based project, coordinated by a steering committee (SC), was applied to a predefined multidisciplinary NEN expert panel studying the following controversial topics : factors guiding therapeutic decision making, the use of somatostatin analogues (SSA) in adjuvant setting, the interference between non-radioactive and radioactive SSAs, challenging small intestine neuroendocrine tumor (NET) cases, the approach of the carcinoid syndrome, the role of chemotherapy in well differentiated NET, the relevance of NET G3 and neuroendocrine carcinoma subclassification and the role of imaging techniques in NEN management. Results: A high level of consensus exists regarding the necessary diagnostic work-up, use of imaging techniques and interference between non-radioactive and radioactive SSAs. However, the prognostic impact of tumor functionality might be overrated and adequate diarrhea differential diagnostic work-up in these patients is underused. Significant differences are seen between individual experts and centers regarding treatment preferences both on the treatment modality level, as well as the choice of specific drugs (e.g. chemotherapy regimen). Conclusions: A Delphi-like multi-round expert discussion proves useful to boost critical thinking and discussion among experts of different background, as well as to describe current clinical practice and stimulate mutual learning in the absence of high-level scientific guidance.


Assuntos
Carcinoma Neuroendócrino , Neoplasias Intestinais , Tumores Neuroendócrinos , Bélgica , Humanos , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/terapia , Somatostatina
16.
Ther Umsch ; 77(9): 449-455, 2020 Nov.
Artigo em Alemão | MEDLINE | ID: mdl-33146094

RESUMO

Neuroendocrine Neoplasia of the digestive tract Abstract. Neuroendocrine neoplasia are a rare and heterogenous tumor entity with long median survival even in metastatic situation. Surgery is the key therapeutic option although a wide range of other therapies are available in metastatic situation. Milestones in classification and grading were achieved by the European Neuroendocrine Tumor Society (ENETS) leading to practical guidelines and WHO- and TNM-classification comparable to the colorectal carcinoma. These new guidelines enable the handling of a rare and complex tumor entity.


Assuntos
Tumores Neuroendócrinos , Neoplasias Pancreáticas , Trato Gastrointestinal/patologia , Humanos , Estadiamento de Neoplasias , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/patologia , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/patologia , Prognóstico
18.
Arch. esp. urol. (Ed. impr.) ; 73(7): 611-623, sept. 2020. tab, graf
Artigo em Espanhol | IBECS | ID: ibc-195959

RESUMO

OBJETIVO: Realizar una revisión en el diagnóstico y el tratamiento de los feocromocitomas y los paragangliomas malignos. MATERIAL Y MÉTODO: Se realizó una búsqueda en PubMed y Google Académico de artículos o guías clínicas que se referían al diagnóstico y el tratamiento de estos tumores. RESULTADOS: Para el diagnóstico de malignidad se debe contar con una confirmación histologica de un feocromocitoma o paraganglioma más la presencia de metástasis confirmada por imágenes. Las metanefrinas se recomiendan sobre otras determinaciones bioquímicas. Para la estadificación se prefiere al PET-TC con 18F-FDG o 18F-DOPA por su mayor sensibilidad que las imágenes convencionales. La gammagrafía con 123I-MIBG se debería solicitar cuando se planea la radioterapia con 131I- MIBG. Para el tratamiento se recomienda el control de los síntomas adrenérgicos mediante el uso de α-bloqueantes. La vigilancia activa fue una opción en pacientes seleccionados con tumores lentamente progresivos. El tratamiento quirúrgico mejoró la SG (148 meses vs 36 meses p= <0,01). La terapia con 131I-MIBG fue indicada en pacientes con gammagrafía positiva, informando una sobrevida global de 50% a 5 años con respuestas tumorales variables. La quimioterapia se propuso en enfermedad rápidamente progresiva, informando una mediana de sobrevida global de 6 años. Las terapias ablativas se deben considerar cuando hay un número limitado de lesiones, para lograr el control local del tumor y reducir los síntomas del exceso de catecolaminas. La radioterapia externa a dosis altas sería eficaz para pacientes con síntomas locales por su carga tumoral. Se necesitan ensayos clínicos multiinstitucionales prospectivos para determinar los verdaderos beneficios de las terapias moleculares en estos pacientes. CONCLUSIONES: Aconsejamos un enfoque multidisciplinario en centros de alta complejidad para poder ofrecer todo el arsenal diagnóstico - terapéutico disponible hasta el momento que mejoren la sobrevida y la calidad de vida de estos pacientes


OBJECTIVE: Perform a review on the diagnosis and treatment of pheochromocytomas and malignant paragangliomas. MATERIAL AND METHOD: A search was conducted in PubMed and Google Scholar of articles or clinical guides that referred to the diagnosis and treatment of these tumors. RESULTS: For the diagnosis of malignancy, a histological confirmation of a pheochromocytoma or paraganglioma should be provided, plus the presence of metastasis confirmed by images. Methanephrines are recommended over other biochemical determinations. For staging, PET-CT with 18F-FDG or 18F-DOPA is preferred because of its greater sensitivity than conventional images. The 123I-MIBG scan should be requested when radiotherapy with 131I-MIBG is planned. For treatment, control of adrenergic symptoms through the use of α-blockers is recommended. Active surveillance was an option in selected patients with slowly progressive tumors. Surgical treatment improved OS (148 months vs 36 months p=<0.01). Therapy with 131I-MIBG was indicated in patients with positive scintigraphy, reporting a global survival of 50% at 5 years with variable tumor responses. Chemotherapy was proposed in rapidly progressive disease, reporting a median overall survival of 6 years. Ablative therapies should be considered when there is a limited number of lesions, to achieve local tumor control and reduce the symptoms of excess catecholamines. External radiation therapy at high doses would be effective for patients with local symptoms due to their tumor burden. Prospective multi-institutional clinical trials are needed to determine the true benefits of molecular therapies in these patients. CONCLUSIONS: We recommend a multidisciplinary approach in centers of high complexity to be able to offer the entire diagnostic - therapeutic arsenal available so far that they improve the survival and quality of life of these patients


Assuntos
Humanos , Feocromocitoma/diagnóstico , Feocromocitoma/terapia , Paraganglioma/diagnóstico , Paraganglioma/terapia , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/terapia , 3-Iodobenzilguanidina/uso terapêutico , Antineoplásicos/uso terapêutico , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Metástase Neoplásica , Medicina Baseada em Evidências
19.
APMIS ; 128(11): 563-572, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32794589

RESUMO

Neuroendocrine tumors (NETs) are often diagnosed from the metastases of an unknown primary tumor. Specific immunohistochemical (IHC) markers indicating the location of a primary tumor are needed. The proprotein convertase subtilisin/kexin type 2 (PCSK2) is found in normal neural and neuroendocrine cells, and known to express in NETs. We investigated the tissue microarray (TMA) of 86 primary tumors from 13 different organs and 9 metastatic NETs, including primary tumor-metastasis pairs, for PCSK2 expression with polymer-based IHC. PCSK2 was strongly positive in all small intestine and appendiceal NETs, the so-called midgut NETs, in most pheochromocytomas and paragangliomas, and in some of the typical and atypical pulmonary carcinoid tumors. NETs showing strong positivity were re-evaluated in larger tumor cohorts confirming the primary observation. In the metastases, the expression of PCSK2 mirrored that of the corresponding primary tumors. We found negative or weak staining in NETs from the thymus, gastric mucosa, pancreas, rectum, thyroid, and parathyroid. PCSK2 expression did not correlate with Ki-67 in well-differentiated NETs. Our data suggest that PCSK2 positivity can indicate the location of the primary tumor. Thus, PCSK2 could function in the IHC panel determined from screening metastatic NET biopsies of unknown primary origins.


Assuntos
Neoplasias das Glândulas Suprarrenais/genética , Carcinoma Neuroendócrino/genética , Neoplasias Gastrointestinais/genética , Neoplasias Pulmonares/genética , Tumores Neuroendócrinos/genética , Paraganglioma/genética , Feocromocitoma/genética , Pró-Proteína Convertase 2/genética , Adenocarcinoma/diagnóstico , Adenocarcinoma/genética , Adenocarcinoma/patologia , Adenocarcinoma/cirurgia , Neoplasias das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/patologia , Neoplasias das Glândulas Suprarrenais/cirurgia , Biomarcadores Tumorais/genética , Carcinoma Neuroendócrino/diagnóstico , Carcinoma Neuroendócrino/patologia , Carcinoma Neuroendócrino/cirurgia , Cromogranina A/genética , Feminino , Neoplasias Gastrointestinais/diagnóstico , Neoplasias Gastrointestinais/patologia , Neoplasias Gastrointestinais/cirurgia , Expressão Gênica , Humanos , Imuno-Histoquímica , Antígeno Ki-67/genética , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/cirurgia , Metástase Linfática , Masculino , Estadiamento de Neoplasias , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/patologia , Tumores Neuroendócrinos/cirurgia , Paraganglioma/diagnóstico , Paraganglioma/patologia , Paraganglioma/cirurgia , Feocromocitoma/diagnóstico , Feocromocitoma/patologia , Feocromocitoma/cirurgia , Neoplasias da Glândula Tireoide/diagnóstico , Neoplasias da Glândula Tireoide/genética , Neoplasias da Glândula Tireoide/patologia , Neoplasias da Glândula Tireoide/cirurgia
20.
Nat Rev Endocrinol ; 16(10): 590-607, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32839579

RESUMO

Over the past 5 years, a number of notable research advances have been made in the field of neuroendocrine cancer, specifically with regard to neuroendocrine cancer of the gastrointestinal tract. The aim of this Review is to provide an update on current knowledge that has proven effective for the clinical management of patients with these tumours. For example, for the first time in the tubular gastrointestinal tract, well-differentiated high-grade (grade 3) tumours and mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs) are defined in the WHO classification. This novel classification enables efficient identification of the most aggressive well-differentiated neuroendocrine tumours and helps in defining the degree of aggressiveness of MiNENs. The Review also discusses updates to epidemiology, cell biology (including vesicle-specific components) and the as-yet-unresolved complex genetic background that varies according to site and differentiation status. The Review summarizes novel diagnostic instruments, including molecules associated with the secretory machinery, novel radiological approaches (including pattern recognition techniques), novel PET tracers and liquid biopsy combined with DNA or RNA assays. Surgery remains the treatment mainstay; however, peptide receptor radionuclide therapy with novel radioligands and new emerging medical therapies (including vaccination and immunotherapy) are evolving and being tested in clinical trials, which are summarized and critically reviewed here.


Assuntos
Neoplasias Gastrointestinais , Tumores Neuroendócrinos , Medicina de Precisão , Animais , Antineoplásicos/uso terapêutico , Neoplasias Gastrointestinais/diagnóstico , Neoplasias Gastrointestinais/fisiopatologia , Neoplasias Gastrointestinais/secundário , Neoplasias Gastrointestinais/terapia , Humanos , Tumores Neuroendócrinos/complicações , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/fisiopatologia , Tumores Neuroendócrinos/terapia , Organização Mundial da Saúde
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