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1.
BMC Anesthesiol ; 24(1): 265, 2024 Jul 31.
Artigo em Inglês | MEDLINE | ID: mdl-39085758

RESUMO

BACKGROUND: Carcinoid tumors are rare neuroendocrine malignancies presenting in an increasing number in our center. The incidence of carcinoid tumors is approximatively between 2.5 and 5 cases per 100,000 people of whom about 50% develop carcinoid syndrome. Once the carcinoid syndrome has developed, a carcinoid cardiomyopathy can occur. Carcinoid heart disease (CaHD) remains a serious and rare complication associated with a significant increase in morbidity and mortality. Although carcinoid tumors have been known and studied for several years, there are still scarce data on the anesthetic management and the peri operative period. CASE PRESENTATION: We describe a case of a Caucasian 44-year-old woman with an unusual presentation of left CaHD with an ileal neuroendocrine tumor and liver metastases. Our preoperative somatostatin administration protocol, limit the cardiac damage. The maintenance of stable hemodynamics, the use of balanced anesthetic technique, all along with a good understanding of the pathology, played a major role in the successful management of anesthesia. This case report allows us to introduce our decision algorithm for the management of this type of pathology in our tertiary hospital, Cliniques Universitaires Saint-Luc. CONCLUSION: Despite the paucity of data, anesthetic management of patients with carcinoid tumor can be safely performed with effective hemodynamic monitoring and a good understanding of the pathophysiology. Knowledge and application of a clear institutional algorithm for octreotide administration and multidisciplinary consultation at a referral center are essential for the management of these patients.


Assuntos
Doença Cardíaca Carcinoide , Neoplasias do Íleo , Tumores Neuroendócrinos , Humanos , Feminino , Adulto , Doença Cardíaca Carcinoide/complicações , Neoplasias do Íleo/complicações , Tumores Neuroendócrinos/complicações , Anestesia/métodos , Tumor Carcinoide/complicações , Somatostatina/análogos & derivados , Somatostatina/administração & dosagem , Somatostatina/uso terapêutico , Neoplasias Hepáticas/secundário
2.
Cureus ; 16(5): e59885, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38854363

RESUMO

We present a rare and complex case of a 76-year-old male patient with a history of low-grade neuroendocrine tumor (NET) of the small intestine, status post resection, who presented with recurrence of the tumor in the liver and subsequent carcinoid heart syndrome (CHS). The recurrent liver tumor caused severe tricuspid regurgitation and CHS, highlighting the rare association between NETs and CHS, particularly in the elderly population. This case underscores the importance of multidisciplinary care and close monitoring for patients with recurrent NETs and potential cardiac complications.

3.
Cureus ; 16(2): e55163, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38558605

RESUMO

Carcinoid tumors are rare neuroendocrine tumors that can be found in the gastrointestinal tract as well as other areas throughout the body. The neurosecretory nature of these tumors can have implications for other chronic diseases that patients may have, such as diabetes. Certain treatments that may be implemented for patients who have carcinoid tumors, such as somatostatin analogs and Everolimus, can also alter blood glucose control. This highlights the importance of diagnosing and treating carcinoid tumors as early as possible to avoid complications associated with metastasis and more intense treatment. With more advanced diseases, clinicians should consider the possible effects of carcinoid tumors and their treatments on other chronic conditions as they manage the patient. For gastrointestinal carcinoid tumors, colonoscopy screening guidelines are incredibly important to counsel patients on, as resection can yield a complete cure for carcinoid tumors when they are found at an early stage. Here, we describe the case of an incidentally diagnosed rectal carcinoid tumor in a 46-year-old female patient with a history of type 2 diabetes mellitus and hypertension.

4.
Cureus ; 16(1): e51862, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38327919

RESUMO

Bronchial carcinoid tumors represent a relatively uncommon category within lung neoplasms, originating from neuroendocrine cells. The exact cause of these pulmonary tumors remains not fully understood. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is characterized by widespread hyperplasia of these neuroendocrine cells, essential for regulating air and blood flow in response to stimuli such as hypoxia, dyspnea, and chronic obstructive pulmonary disease (COPD). The prognosis for bronchial carcinoid tumors hinges on factors such as grade and stage, with lung resection being the preferred treatment. A chest computed tomography (CT) scan unveiled diffuse bilateral pulmonary nodules with ground-glass opacities, leading to a right video-assisted thoracoscopic surgery (VATS) wedge resection. Immunohistochemical examination confirmed neuroendocrine differentiation, describing a lung wedge measuring 9 × 4 × 1.5 cm with spongy parenchyma and scattered white nodules.

5.
Cancers (Basel) ; 15(23)2023 Nov 25.
Artigo em Inglês | MEDLINE | ID: mdl-38067279

RESUMO

Well-differentiated lung neuroendocrine tumors (LNETs) are heterogeneous cancers that are increasing in incidence. Treatment options for LNETs have expanded in recent years, and our knowledge of the molecular subtypes has also advanced. Multidisciplinary teams have an established role in personalizing the best treatment for individual patients. Other precision medicine approaches for the treatment of LNETs have lagged behind those for non-small-cell lung cancer, with only rare actionable molecular alterations identified and few established predictive factors to guide therapy selection. However, as summarized in this review, there is increasing potential for personalized treatment of patients with LNETs. In particular, advances in radiotheragnostics may allow us to tailor the treatment of individual patients with NETs in the coming years. These advances may soon deliver the promise of more effective, less toxic treatments and better outcomes for patients with these increasingly common cancers.

6.
Cureus ; 15(10): e46411, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37800163

RESUMO

The presentation of recurrent pneumonia, particularly in the same lobe, should raise suspicion for possible neuroendocrine tumors of the lung within that respective lobe. Commonly, these types of tumors will have a gastrointestinal origin with a larger incidence of carcinoid syndrome, but they may also originate in the pancreas or lungs. This case illustrates the potential for a masked lung tumor in an otherwise young and healthy 31-year-old patient, with a short history of tobacco dependence and unremarkable family history, who presents with recurrent pneumonia and dyspnea. Although rare in itself, this case was even more unique due to the partial calcification of the neuroendocrine tumor mass along with causing a collapse in the entire right middle lobe.

7.
Insights Imaging ; 14(1): 148, 2023 Sep 19.
Artigo em Inglês | MEDLINE | ID: mdl-37726504

RESUMO

OBJECTIVES: Lung carcinoids and atypical hamartomas may be difficult to differentiate but require different treatment. The aim was to differentiate these tumors using contrast-enhanced CT semantic and radiomics criteria. METHODS: Between November 2009 and June 2020, consecutives patient operated for hamartomas or carcinoids with contrast-enhanced chest-CT were retrospectively reviewed. Semantic criteria were recorded and radiomics features were extracted from 3D segmentations using Pyradiomics. Reproducible and non-redundant radiomics features were used to training a random forest algorithm with cross-validation. A validation-set from another institution was used to evaluate of the radiomics signature, the 3D 'median' attenuation feature (3D-median) alone and the mean value from 2D-ROIs. RESULTS: Seventy-three patients (median 58 years [43‒70]) were analyzed (16 hamartomas; 57 carcinoids). The radiomics signature predicted hamartomas vs carcinoids on the external dataset (22 hamartomas; 32 carcinoids) with an AUC = 0.76. The 3D-median was the most important in the model. Density thresholds < 10 HU to predict hamartoma and > 60 HU to predict carcinoids were chosen for their high specificity > 0.90. On the external dataset, sensitivity and specificity of the 3D-median and 2D-ROIs were, respectively, 0.23, 1.00 and 0.13, 1.00 < 10 HU; 0.63, 0.95 and 0.69, 0.91 > 60 HU. The 3D-median was more reproducible than 2D-ROIs (ICC = 0.97 95% CI [0.95‒0.99]; bias: 3 ± 7 HU limits of agreement (LoA) [- 10‒16] vs. ICC = 0.90 95% CI [0.85‒0.94]; bias: - 0.7 ± 21 HU LoA [- 4‒40], respectively). CONCLUSIONS: A radiomics signature can distinguish hamartomas from carcinoids with an AUC = 0.76. Median density < 10 HU and > 60 HU on 3D or 2D-ROIs may be useful in clinical practice to diagnose these tumors with confidence, but 3D is more reproducible. CRITICAL RELEVANCE STATEMENT: Radiomic features help to identify the most discriminating imaging signs using random forest. 'Median' attenuation value (Hounsfield units), extracted from 3D-segmentations on contrast-enhanced chest-CTs, could distinguish carcinoids from atypical hamartomas (AUC = 0.85), was reproducible (ICC = 0.97), and generalized to an external dataset. KEY POINTS: • 3D-'Median' was the best feature to differentiate carcinoids from atypical hamartomas (AUC = 0.85). • 3D-'Median' feature is reproducible (ICC = 0.97) and was generalized to an external dataset. • Radiomics signature from 3D-segmentations differentiated carcinoids from atypical hamartomas with an AUC = 0.76. • 2D-ROI value reached similar performance to 3D-'median' but was less reproducible (ICC = 0.90).

8.
Rev Mal Respir ; 40(8): 716-722, 2023 Oct.
Artigo em Francês | MEDLINE | ID: mdl-37633811

RESUMO

INTRODUCTION: Ectopic Cushing's syndrome (CS) is a rare condition nevertheless well-known to endocrinologists. The pneumologist may be called upon to treat CS not only because bronchial carcinoid tumors are the most frequent source of ectopic ACTH secretion, but also due to the fact that the immunosuppression induced by hypercorticism favors lower respiratory tract infections. CASE REPORT: We report the case of a female patient presenting with acute respiratory failure secondary to Enterobacter cloacae pneumonia exacerbated by SC. Further investigations confirmed ectopic ACTH secretion and revealed a right upper lobe pulmonary nodule. After appropriate antibiotic therapy, the patient received preoperative adrenolytic treatment. Management by right upper lobectomy resulted in the extraction of a 12mm tumor. Pathological analysis was consistent with the diagnosis of a typical carcinoid tumor. Immunohistochemistry confirmed ACTH secretion by the tumor. Even though the postoperative course showed CS regression, the patient developed adrenal insufficiency. CONCLUSION: Ectopic CS induces immunosuppression, which aggravates lower respiratory tract infections. Search for a pulmonary neuroendocrine tumor should be systematic. Following control of the secretory syndrome by adrenolytic treatment, and if the diagnosis of carcinoid tumor is confirmed, surgical treatment is the preferred option.


Assuntos
Síndrome de ACTH Ectópico , Neoplasias Brônquicas , Tumor Carcinoide , Síndrome de Cushing , Pneumonia , Infecções Respiratórias , Humanos , Feminino , Síndrome de Cushing/etiologia , Síndrome de Cushing/complicações , Síndrome de ACTH Ectópico/complicações , Síndrome de ACTH Ectópico/diagnóstico , Neoplasias Brônquicas/complicações , Neoplasias Brônquicas/diagnóstico , Neoplasias Brônquicas/cirurgia , Tumor Carcinoide/complicações , Tumor Carcinoide/diagnóstico , Tumor Carcinoide/cirurgia , Infecções Respiratórias/complicações , Hormônio Adrenocorticotrópico , Antagonistas Adrenérgicos
9.
Oncol Lett ; 25(6): 243, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-37153061

RESUMO

Neuroendocrine tumors (NETs) are tumors originating from neuroendocrine cells and peptidergic neurons. Primary renal well-differentiated NETs (WDNETs) are rare and only sporadic cases have been reported worldwide. In November 2021, a 45-year-old female patient was admitted to The Affiliated Hospital of Zunyi Medical University (Zunyi, China) with right-sided lumbago. Abdominal computed tomography revealed a 44×34×70-mm mass in the right kidney. Following a complete examination, a laparoscopic partial nephrectomy of the right kidney was performed under general anesthesia. The postoperative pathology indicated a well-differentiated NET of the right kidney. There was no tumor recurrence or metastasis during the 1-year follow-up period. WDNETs are rare, their clinical and imaging findings are not specific, and their diagnosis depends on immunohistochemical analysis. The degree of malignancy is low and the prognosis is positive. Surgical resection is often the first choice, and long-term follow-up is required.

10.
Thorac Cancer ; 14(14): 1311-1315, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36941083

RESUMO

Thymic neuroendocrine tumors associated with multiple endocrine neoplasia are only defined as carcinoid and are not associated with large-cell neuroendocrine carcinoma (LCNEC). We report the case of a multiple endocrine neoplasia type 1 patient with atypical carcinoid tumors with elevated mitotic counts (AC-h), an intermediate condition between carcinoid and LCNEC. A 27-year-old man underwent surgery for an anterior mediastinal mass and was diagnosed with thymic LCNEC. Fifteen years later, a mass appeared at the same site, which was determined to be a postoperative recurrence based on the pathological results of a needle biopsy and the clinical course. The patient's disease remained stable for 10 months on anti-programmed death-ligand 1 antibody and platinum-containing chemotherapy. The needle biopsy specimen was submitted for next-generation sequencing, which revealed a MEN1 gene mutation, and after further examination, a diagnosis of multiple endocrine neoplasia type 1 was made. A re-examination of the surgical specimen from 15 years prior showed that it corresponded to AC-h. Although thymic AC-h is classified as thymic LCNEC according to the current definition, our data suggests that a search for multiple endocrine neoplasia is warranted in such patients.


Assuntos
Tumor Carcinoide , Carcinoma Neuroendócrino , Neoplasia Endócrina Múltipla Tipo 1 , Neoplasia Endócrina Múltipla , Tumores Neuroendócrinos , Timoma , Neoplasias do Timo , Masculino , Humanos , Adulto , Neoplasia Endócrina Múltipla Tipo 1/complicações , Neoplasia Endócrina Múltipla Tipo 1/genética , Neoplasia Endócrina Múltipla Tipo 1/patologia , Tumor Carcinoide/genética , Tumor Carcinoide/patologia , Tumores Neuroendócrinos/patologia , Neoplasias do Timo/diagnóstico , Neoplasias do Timo/genética , Neoplasias do Timo/cirurgia , Timoma/complicações , Carcinoma Neuroendócrino/genética
11.
Expert Rev Endocrinol Metab ; 17(5): 389-403, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-35822906

RESUMO

INTRODUCTION: Neuroendocrine neoplasms (NENs) are a heterogeneous group of malignancies mainly arising in the gastroenteropancreatic (GEP) and bronchopulmonary systems, with steadily increasing incidence. The therapeutic landscape has widened and the therapeutic strategy should be based on new sequences and combinations, still debated. AREAS COVERED: Herein, we provide an overview of current approved pharmacological treatments in patients with NENs, with the aim to summarize evidence of efficacy of the main different options in GEP and pulmonary NENs, principally focusing on somatostatin analogs (SSAs), targeted therapy with everolimus and sunitinib, peptide receptor radionuclide therapy (PRRT) and chemotherapy. We discuss biological rationale and toxicities, including current indications according to differentiation and placement in the therapeutic algorithm, clinical trials, and combinations. Furthermore, we recommend areas for further research. EXPERT OPINION: Therapeutic management of patients with NENs represents a challenge for clinicians and the identification of effective sequences and combinations is of utmost importance. Major efforts should be directed to early identify and overcome resistance and to limit toxicity. The progress in the therapeutic management of NENs grows faster and the choice of the best approach should be based on randomized clinical trials, as well as on long-term, real-world data.


Assuntos
Tumores Neuroendócrinos , Neoplasias Pancreáticas , Everolimo/uso terapêutico , Humanos , Tumores Neuroendócrinos/tratamento farmacológico , Tumores Neuroendócrinos/patologia , Neoplasias Pancreáticas/tratamento farmacológico , Neoplasias Pancreáticas/patologia , Somatostatina/uso terapêutico
12.
Int J Cancer ; 150(12): 2058-2071, 2022 06 15.
Artigo em Inglês | MEDLINE | ID: mdl-35262195

RESUMO

Lung carcinoid tumors, also referred to as pulmonary neuroendocrine tumors or lung carcinoids, are rare neoplasms of the lung with a more favorable prognosis than other subtypes of lung cancer. Still, some patients suffer from relapsed disease and metastatic spread. Several recent single-cell studies have provided detailed insights into the cellular heterogeneity of more common lung cancers, such as adeno- and squamous cell carcinoma. However, the characteristics of lung carcinoids on the single-cell level are yet completely unknown. To study the cellular composition and single-cell gene expression profiles in lung carcinoids, we applied single-cell RNA sequencing to three lung carcinoid tumor samples and normal lung tissue. The single-cell transcriptomes of carcinoid tumor cells reflected intertumoral heterogeneity associated with clinicopathological features, such as tumor necrosis and proliferation index. The immune microenvironment was specifically enriched in noninflammatory monocyte-derived myeloid cells. Tumor-associated endothelial cells were characterized by distinct gene expression profiles. A spectrum of vascular smooth muscle cells and pericytes predominated the stromal microenvironment. We found a small proportion of myofibroblasts exhibiting features reminiscent of cancer-associated fibroblasts. Stromal and immune cells exhibited potential paracrine interactions which may shape the microenvironment via NOTCH, VEGF, TGFß and JAK/STAT signaling. Moreover, single-cell gene signatures of pericytes and myofibroblasts demonstrated prognostic value in bulk gene expression data. Here, we provide first comprehensive insights into the cellular composition and single-cell gene expression profiles in lung carcinoids, demonstrating the noninflammatory and vessel-rich nature of their tumor microenvironment, and outlining relevant intercellular interactions which could serve as future therapeutic targets.


Assuntos
Tumor Carcinoide , Carcinoma Neuroendócrino , Neoplasias Pulmonares , Tumores Neuroendócrinos , Tumor Carcinoide/genética , Tumor Carcinoide/metabolismo , Tumor Carcinoide/patologia , Carcinoma Neuroendócrino/patologia , Células Endoteliais/metabolismo , Humanos , Pulmão/patologia , Neoplasias Pulmonares/patologia , Tumores Neuroendócrinos/patologia , Prognóstico , Microambiente Tumoral/genética
13.
Cureus ; 13(9): e17629, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34646678

RESUMO

Carcinoid tumors are one of the most common types of small intestinal neuroendocrine tumors (SI-NETs). However, SI-NETs that manifest as subacute intestinal obstruction are extremely rare. The annual occurrence rate of jejuno-ileal NETs is 0.28-0.8 per 100,000 people. In this report, we describe a case of subacute intestinal obstruction due to a mid-ileal stricture. The patient underwent laparotomy after evaluation and investigation. Mid-ileal growth was noted, and small bowel resection was performed with primary end-to-end anastomosis. Postoperative histopathology revealed the growth to be a well-differentiated NET.

14.
Anal Chim Acta ; 1181: 338925, 2021 Oct 09.
Artigo em Inglês | MEDLINE | ID: mdl-34556226

RESUMO

An electrochemically synthetized nano-sensor based on molecularly imprinted polypyrrole (MIPPy) was successfully developed for the detection of 5-hydroxyindole-3-acetic acid (5-HIAA) in human biological fluids namely serum, urine, and plasma. The imprinted glassy carbon electrode was prepared by electropolymerisation of pyrrole via cyclic voltammetry (C.V). After completely leaching the imprinted molecules from the polymeric network, complementary cavities are created. The developed MIPPy sensor, under optimized conditions, shows a high sensitivity towards the target molecule (LOQ = 5 × 10-11 M). Moreover, it presents a wide linear response in the range of 5 × 10-11 - 5 × 10-5 M (R2 > 0.999) with a detection limit of 15 × 10-12 M. In order to evaluate the selectivity of the MIPPy film, several structural analogues and compounds forming the real matrices were tested. The obtained results show an excellent recovery rate (between 98.86 and 101.52%) proving the promising application of the proposed nano-sensor in the detection of 5-HIAA in human biological fluids without any significant interference recorded.


Assuntos
Tumor Carcinoide , Impressão Molecular , Acetatos , Biomarcadores Tumorais , Técnicas Eletroquímicas , Eletrodos , Humanos , Ácido Hidroxi-Indolacético , Indóis , Limite de Detecção , Polímeros , Pirróis
15.
Oncotarget ; 12(15): 1470-1489, 2021 Jul 20.
Artigo em Inglês | MEDLINE | ID: mdl-34316328

RESUMO

OBJECTIVE: Aberrations in the PI3K/AKT/mTOR survival pathway in many cancers are the most common genomic abnormalities. The phytochemical and bioactive agent sulforaphane (SFN) has nutrigenomic potential in activating the expression of several cellular protective genes via the transcription factor nuclear factor erythroid 2-related factor 2 (Nrf2). Nrf2 is primarily related to mechanisms of endogenous cellular defense and survival. The efficacy of SFN in combination with acetazolamide (AZ) was investigated in reducing typical H727 and atypical H720 BC survival, migration potential, and apoptosis in vitro and in vivo preclinical xenograft tissues. MATERIALS AND METHODS: Microscopic imaging, immunocytochemistry, wound healing assay, caspase-cleaved cytokeratin 18 (M30, CCK18) CytoDeath ELISA assay, immunofluorescence labeling assays for apoptosis, hypoxia, Western Blotting, Tunnel assay, measurement of 5-HT secretion by carbon fiber amperometry assay, quantitative methylation-specific PCR (qMSP), morphologic changes, cell viability, apoptosis activity and the expression levels of phospho-Akt1, Akt1, HIF-1α, PI3K, p21, CAIX, 5-HT, phospho-mTOR, and mTOR in xenografts derived from typical H727 and atypical H720 BC cell lines. RESULTS: Combining AZ+SFN reduced tumor cell survival compared to each agent alone, both in vitro and in vivo xenograft tissues. AZ+SFN targeted multiple pathways involved in cell cycle, serotonin secretion, survival, and growth pathways, highlighting its therapeutic approach. Both H727 and H720 cells were associated with induction of apoptosis, upregulation of the p21 cell cycle inhibitor, and downregulation of the PI3K/Akt/mTOR pathway, suggesting that the PI3K/Akt/mTOR pathway is a primary target of the AZ+SFN combination therapy. CONCLUSIONS: Combining SFN+AZ significantly inhibits the PI3K/Akt/mTOR pathway and significantly reducing 5-HT secretion in carcinoid syndrome.

16.
Clin Endosc ; 54(6): 818-824, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33794563

RESUMO

Small bowel neuroendocrine tumors (NETs) represent approximately one-third of NETs of the gastrointestinal tract, and their incidence is increasing. When determining if endoscopic resection is appropriate, endoscopic ultrasound is used to assess the lesion size and depth of invasion for duodenal NETs. A number of techniques, including endoscopic mucosal resection (EMR), band-assisted EMR (band-EMR), endoscopic submucosal dissection (ESD), and over-the-scope clip-assisted endoscopic full-thickness resection (EFTR), have been studied; however, the best technique for endoscopic resection remains unclear. The vast majority of currently available data are retrospective, and prospective studies with longer follow-up times are required. For jejunal and ileal NETs, endoscopic techniques such as video capsule endoscopy (VCE) and balloon enteroscopy (BE) assist in diagnosis. This includes localization of the primary NET in metastatic disease where initial workup has been negative, and the identification of multifocal disease, which may change management and prognostication.

17.
Pediatr Blood Cancer ; 68(4): e28880, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33522705

RESUMO

BACKGROUND: Extra-appendicular neuroendocrine tumors (NETs) are very rare tumors. While diagnostic and therapeutic guidelines are well established for adults, data on children and adolescents are lacking. PATIENTS AND METHODS: Patients with a diagnosis of extra-appendicular NET registered on the Tumori Rari in Età Pediatrica - Rare Tumors in Pediatric Age (TREP) from 2000 to 2020 were analyzed. Clinical characteristics including patients' presentation, tumor features, treatment, and outcome were reviewed. RESULTS: Twenty-seven patients with extra-appendicular NET registered on TREP with a median age of 173 months. The primary site was the pancreas (12) or bronchi (10) in the majority of cases. Other primary sites included the thymus, Meckel's diverticulum, and liver. Thirteen (48%) of tumors extended beyond the organ of origin: four invaded neighboring organs and/or regional nodes and nine involved distant metastases. The 3-year event-free survival (EFS) for those with localized disease was superior to those with metastatic disease (66.6% 95% CI 5-95% vs 33% 95% CI 5-68%, respectively; P = .005). A complete resection was feasible in 17 patients. The 3-year EFS in these patients was superior to those with no or incomplete resection (R0 vs R1/R2, respectively; P = .007). Overall, 16 children had no evidence of disease at follow-up, and one is alive with disease; five died, and five were lost to follow-up. CONCLUSIONS: Data from our experience demonstrated a wide heterogeneity of presentation and outcome of these tumors. Localized disease and complete surgical resection were the main prognostic factors of good outcome. Other therapies may have a role in prolonging survival in metastatic disease.


Assuntos
Neoplasias Brônquicas/diagnóstico , Tumores Neuroendócrinos/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Adolescente , Neoplasias Brônquicas/epidemiologia , Neoplasias Brônquicas/terapia , Criança , Gerenciamento Clínico , Feminino , Humanos , Neoplasias Intestinais/diagnóstico , Neoplasias Intestinais/epidemiologia , Neoplasias Intestinais/terapia , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/epidemiologia , Neoplasias Hepáticas/terapia , Masculino , Tumores Neuroendócrinos/epidemiologia , Tumores Neuroendócrinos/terapia , Neoplasias Pancreáticas/epidemiologia , Neoplasias Pancreáticas/terapia , Neoplasias do Timo/diagnóstico , Neoplasias do Timo/epidemiologia , Neoplasias do Timo/terapia
18.
BMC Cancer ; 21(1): 146, 2021 Feb 09.
Artigo em Inglês | MEDLINE | ID: mdl-33563241

RESUMO

BACKGROUND: Medical centers with varying levels of expertise treat gastroenteropancreatic neuroendocrine tumors (GEP-NETs), which are relatively rare tumors. This study assesses the impact of center volume on GEP-NET treatment outcomes. METHODS: We used the Surveillance, Epidemiology, and End Results (SEER) registry linked to Medicare claims data. The data includes patients diagnosed between 1995 and 2010 who had no health maintenance organization (HMO) coverage, participated in Medicare parts A and B, were older than 65 at diagnosis, had tumor differentiation information, and had no secondary cancer. We identified medical centers at which patients received GEP-NET treatment (surgery, chemotherapy, somatostatin analogues, or radiation therapy) using Medicare claims data. Center volume was divided into 3 tiers - low, medium, and high - based on the number of unique GEP-NET patients treated by a medical center over 2 years. We used Kaplan-Meier curves and Cox regression to assess the association between volume and disease-specific survival. RESULTS: We identified 899 GEP-NET patients, of whom 37, 45, and 18% received treatment at low, medium volume, and high-volume centers, respectively. Median disease-specific survival for patients at low and medium tiers were 1.4 years and 5.3 years, respectively, but was not reached for patients at high volume centers. Results showed that patients treated at high volume centers had better survival than those treated in low volume centers (HR: 0.63, 95% CI: 0.4-0.9), but showed no difference in outcomes between medium and high-volume centers. CONCLUSIONS: Our results suggest that for these increasingly common tumors, referral to a tertiary care center may be indicated. Physicians caring for GEP-NET patients should consider early referral to high volume centers.


Assuntos
Hospitais com Alto Volume de Atendimentos/estatística & dados numéricos , Hospitais com Baixo Volume de Atendimentos/estatística & dados numéricos , Neoplasias Intestinais/mortalidade , Tumores Neuroendócrinos/mortalidade , Neoplasias Pancreáticas/mortalidade , Programa de SEER/estatística & dados numéricos , Neoplasias Gástricas/mortalidade , Idoso , Terapia Combinada , Feminino , Seguimentos , Humanos , Neoplasias Intestinais/patologia , Neoplasias Intestinais/terapia , Masculino , Tumores Neuroendócrinos/patologia , Tumores Neuroendócrinos/terapia , Neoplasias Pancreáticas/patologia , Neoplasias Pancreáticas/terapia , Prognóstico , Estudos Retrospectivos , Neoplasias Gástricas/patologia , Neoplasias Gástricas/terapia , Taxa de Sobrevida
19.
BMC Cancer ; 21(1): 141, 2021 Feb 08.
Artigo em Inglês | MEDLINE | ID: mdl-33557782

RESUMO

BACKGROUND: Lung carcinoid is a rare malignant tumor with poor survival. The current study established a nomogram model for predicting cancer-specific survival (CSS) in patients with lung carcinoid tumors. METHODS: A total of 1956 patients diagnosed with primary lung carcinoid tumors were extracted from the Surveillance, Epidemiology, and End Results database. The specific predictors of CSS for lung carcinoid tumors were identified and integrated to build a nomogram. Validation of the nomogram was conducted using parameters concordance index (C-index), calibration plots, decision curve analyses (DCAs), and the receiver operating characteristic (ROC) curve. RESULTS: Age at diagnosis, grade, histological type, N stage, M stage, surgery of the primary site, radiation of the primary site, and tumor size were independent prognostic factors of CSS. High discriminative accuracy of the nomogram model was shown in the training cohort (C-index = 0.873), which was also testified in the internal validation cohort (C-index = 0.861). In both cohorts, the calibration plots showed good concordance between the predicted and observed CSS at 3, 5, and 10 years. The DCA showed great potential for clinical application. The ROC curve showed superior survival predictive ability of the nomogram model (area under the curve = 0.868). CONCLUSIONS: We developed a practical nomogram that provided independent predictions of CSS for patients with lung carcinoid tumors. This nomogram may have the potential to assist clinicians in prognostic evaluations or developing individualized therapies for patients with this neoplasm.


Assuntos
Tumor Carcinoide/mortalidade , Neoplasias Pulmonares/mortalidade , Nomogramas , Medição de Risco/métodos , Programa de SEER/estatística & dados numéricos , Adulto , Idoso , Idoso de 80 Anos ou mais , Tumor Carcinoide/patologia , Tumor Carcinoide/terapia , Terapia Combinada , Feminino , Seguimentos , Humanos , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/terapia , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida , Adulto Jovem
20.
J Gastrointest Cancer ; 52(2): 448-453, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33471257

RESUMO

INTRODUCTION: Although duodenal neuroendocrine tumors (dNETs) are rare, the incidence, particularly for lesions ≤ 10 mm, continues to rise with the advent of widely available, high-quality endoscopy. Given their rarity and controversy regarding prognostication factors, limited guidelines exist for resection of well-differentiated, ≤ 20-mm dNETs. Importantly, lesions ≤ 10 mm in a duodenal location are ideal for consideration of endoscopic management given both morbidity and technical challenges associated with surgery and their accessibility to a wide range of endoscopic techniques. OBJECTIVES: The primary objective of this study was to demonstrate the safety and efficacy of the endoscopic resection of dNETs <10 mm in a case series and literature review. METHODS: We performed a literature review and present a series of nine cases to demonstrate the viability of endoscopic resection for diminutive dNETs as an alternative to surgery. RESULTS: Our case series included nine well-differentiated diminutive dNETs in seven patients, the majority resected using endoscopic mucosal resection (EMR), 67%, and there was no residual disease at follow-up. The literature review of 178 patients demonstrated that EMR was the most used method of resection of diminutive dNETs, 81%, compared to endoscopic submucosal dissection, 19%. The most common complication was intraoperative bleeding in 9.55%, and only 2.25% of patients had recurrence. CONCLUSION: While complications may arise with endoscopic resection of diminutive dNETs, they are usually managed endoscopically and compare favorably with the literature on surgical complication rates and typically result in shorter hospitalizations.


Assuntos
Neoplasias Duodenais/cirurgia , Ressecção Endoscópica de Mucosa/métodos , Tumores Neuroendócrinos/cirurgia , Idoso , Canadá , Neoplasias Duodenais/patologia , Endoscopia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Tumores Neuroendócrinos/patologia , Estudos Retrospectivos
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