scholarly journals Neuroendocrine Tumors in Pediatrics

2019 ◽  
Vol 6 ◽  
pp. 2333794X1986271
Author(s):  
Zainab Azam Farooqui ◽  
Aman Chauhan
Keyword(s):  
Neuroendocrine Tumors ◽  
Early Stage ◽  
Peptide Receptor Radionuclide Therapy ◽  
The Body ◽  
Neuroendocrine Cells ◽  
Presenting Symptoms ◽  
Rare Tumors ◽  
Pediatric Age Group ◽  
Organ Systems ◽  
Low Incidence

Neuroendocrine cells are dispersed diffusely throughout many organ systems in the body and hence neuroendocrine tumors (NETs) can arise from almost anywhere in the body. NETs are considered rare tumors, and the current incidence is reported to be about 6 cases in 100 000 in adults and about 2.8 cases per million in the pediatric age group. Despite the indolent nature of these tumors, they have the potential for metastasis and significant morbidity. NETs can be asymptomatic at the time of diagnosis or can present with flushing, diarrhea, wheezing, weight loss, and fatigue among other symptoms. Due to the ambiguity of presenting symptoms, it is not uncommon for NETs to be diagnosed late in the disease course. Despite low incidence, the prevalence of the disease is high since patients live for many years and sometimes decades. Early detection of well-differentiated NETs has excellent outcomes with the majority of early-stage diseases being cured with surgical resection alone. There have been recent advancements in the management of metastatic progressive NETs with approval of peptide receptor radionuclide therapy, telotristat, and everolimus. Awareness of these rare tumors and its management is crucial for optimal management. This article will focus on pediatric NETs and current advances in its management.

scholarly journals Congenital Lumbar Hernia in an 8-Month-Old Boy

2021 ◽  
Vol 15 (1) ◽  
pp. 431-435
Author(s):  
Mohamed Mansy ◽  
Mostafa Kotb ◽  
Mohamed Abouheba
Keyword(s):  
Congenital Anomalies ◽  
Lumbar Hernia ◽  
The Body ◽  
Age Group ◽  
Pediatric Age ◽  
Operative Repair ◽  
Pediatric Age Group ◽  
Organ Systems

Congenital lumbar hernias are uncommonly seen in the pediatric age group, with only about 60 cases reported in the literature. It is usually accompanied by a multitude of congenital anomalies involving different organ systems of the body. For instance, it may involve the ribs, spine, muscles, and the kidneys. Herein, we report a case of congenital lumbar hernia in an 8-month-old boy who underwent an operative repair using a mesh with an uneventful outcome.

scholarly journals Poorly Differentiated Small-Cell-Type Neuroendocrine Carcinoma of the Prostate: A Case Report and Literature Review

2018 ◽  
Vol 11 (3) ◽  
pp. 676-681 ◽  
Author(s):  
Kishore Kumar ◽  
Rafeeq Ahmed ◽  
Chime Chukwunonso ◽  
Hassan Tariq ◽  
Masooma Niazi ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Tumor Grade ◽  
Small Cell ◽  
The Body ◽  
Neuroendocrine Cells ◽  
Cell Type ◽  
Variable Response ◽  
Ki 67 ◽  
Platinum Based Chemotherapy ◽  
Poorly Differentiated

Neuroendocrine cells are widespread throughout the body and can give rise of neuroendocrine tumors due to abnormal growth of the chromaffin cells. Neuroendocrine tumors divide into many subtypes based on tumor grade (Ki-67 index and mitotic count) and differentiation. These tumors can be further divided into secretory and nonsecretory types based on the production of peptide hormone by tumor cells. Poorly differentiated small-cell-type neuroendocrine tumors are one of the subtypes of neuroendocrine tumors. These tumors are less common; however, they tend to be locally invasive and aggressive in behavior with poor overall median survival. Treatment of the nonsecretory small-cell type is modeled to small-cell lung cancer with a regimen consisting of platinum-based chemotherapy and etoposide with variable response. Here, we present a case of poorly differentiated small-cell neuroendocrine tumor originating from the prostate.

scholarly journals Ca2+ Channel Toolkit in Neuroendocrine Tumors

2019 ◽  
Vol 110 (1-2) ◽  
pp. 147-154
Author(s):  
Alessandra Fiorio Pla ◽  
Dimitra Gkika
Keyword(s):  
Neuroendocrine Tumors ◽  
Transient Receptor Potential ◽  
Treatment Options ◽  
Unmet Need ◽  
Receptor Potential ◽  
Transient Receptor Potential Channels ◽  
The Body ◽  
Neuroendocrine Cells ◽  
Neoplastic Progression ◽  
Ca2 Channels

Neuroendocrine tumors (NET) constitute a heterogeneous group of malignancies with various clinical presentations and growth rates but a common origin in neuroendocrine cells located all over the body. NET are a relatively low-frequency disease mostly represented by gastroenteropancreatic (GEP) and bronchopulmonary tumors (pNET); on the other hand, an increasing frequency and prevalence have been associated with NET. Despite great efforts in recent years, the management of NET is still a critical unmet need due to the lack of knowledge of the biology of the disease, the lack of adequate biomarkers, late presentation, the relative insensitivity of imaging modalities, and a paucity of predictably effective treatment options. In this context Ca2+ signals, being pivotal molecular devices in sensing and integrating signals from the microenvironment, are emerging to be particularly relevant in cancer, where they mediate interactions between tumor cells and the tumor microenvironment to drive different aspects of neoplastic progression (e.g., cell proliferation and survival, cell invasiveness, and proangiogenetic programs). Indeed, ion channels represent good potential pharmacological targets due to their location on the plasma membrane, where they can be easily accessed by drugs. The present review aims to provide a critical and up-to-date overview of NET development integrating Ca2+ signal involvement. In this perspective, we first give an introduction to NET and Ca2+ channels and then describe the different families of Ca2+ channels implicated in NET, i.e., ionotropic receptors, voltage-dependent Ca2+ channels, and transient receptor potential channels, as well as intracellular Ca2+ channels and their signaling molecules.

scholarly journals Incidentally Found Aorto-Pulmonary Middle Mediastinal Hypervascular Mass on Pulmonary CT Angiography in a Covid 19 patient

2021 ◽  
Vol 6 (4) ◽  
pp. 01-03
Author(s):  
Berrin Erok
Keyword(s):  
Differential Diagnosis ◽  
Ct Angiography ◽  
Neuroendocrine Tumors ◽  
Mediastinal Mass ◽  
The Body ◽  
Neuroendocrine Cells ◽  
Mediastinal Tumors ◽  
Radiological Features ◽  
Adjacent Structures ◽  
Pulmonary Ct

Paragangliomas (PGs) are rare neuroendocrine tumors arising from paraganglia, clusters of neuroendocrine cells scattered throughout the body. Mediastinal paragangliomas represent less than 2% of all paragangliomas and less than 0.3 % of the mediastinal tumors. These tumors may secrete catecholamines, however in up to 50% of cases they are nonfunctional and are diagnosed incidentaly or with symptoms of mass effcet to the adjacent structures. They should be considered in the differential diagnosis of hypervascular mediastinal masses. The typical radiological features are very guiding in the diagnosis and the management of the patient. We aimed to present a case of a hypervascular middle mediastinal mass incidentally found in a 69 year old woman and diagnosed with aortopulmonary nonfunctional PG radiologically.

scholarly journals GEP- NETS UPDATE: Genetics of neuroendocrine tumors

2016 ◽  
Vol 174 (6) ◽  
pp. R275-R290 ◽  
Author(s):  
Joakim Crona ◽  
Britt Skogseid
Keyword(s):  
Neuroendocrine Tumors ◽  
Neurofibromatosis Type ◽  
Genetic Alterations ◽  
The Body ◽  
Neuroendocrine Cells ◽  
Genetic Syndrome ◽  
Von Hippel Lindau ◽  
Actual Patient ◽  
Therapeutic Decisions ◽  
Near Future

Neuroendocrine tumors (NETs) are a heterogeneous group of neoplasms, arising from neuroendocrine cells that are dispersed throughout the body. Around 20% of NETs occur in the context of a genetic syndrome. Today there are at least ten recognized NET syndromes. This includes the classical syndromes: multiple endocrine neoplasias types 1 and 2, and von Hippel–Lindau and neurofibromatosis type 1. Additional susceptibility genes associated with a smaller fraction of NETs have also been identified. Recognizing genetic susceptibility has proved essential both to provide genetic counseling and to give the best preventive care. In this review we will also discuss the knowledge of somatic genetic alterations in NETs. At least 24 genes have been implicated as drivers of neuroendocrine tumorigenesis, and the overall rates of genomic instability are relatively low. Genetic intra-tumoral, as well as inter-tumoral heterogeneity in the same patient, have also been identified. Together these data point towards the common pathways in NET evolution, separating early from late disease drivers. Although knowledge of specific mutations in NETs has limited impact on actual patient management, we predict that in the near future genomic profiling of tumors will be included in the clinical arsenal for diagnostics, prognostics and therapeutic decisions.

scholarly journals Somatostatin receptor endoradiotherapy of neuroendocrine tumors: experience in Hungarian patients

2011 ◽  
Vol 152 (10) ◽  
pp. 392-397 ◽  
Author(s):  
Péter Reismann ◽  
Zoltán Kender ◽  
Gabriella Dabasi ◽  
Lídia Sréter ◽  
Károly Rácz ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Radionuclide Therapy ◽  
Somatostatin Receptor ◽  
Somatostatin Receptors ◽  
Peptide Receptor Radionuclide Therapy ◽  
Somatostatin Analogues ◽  
Theoretical Background ◽  
Neuroendocrine Cells ◽  
Peptide Receptor ◽  
The University

Beside conventional therapies for the treatment of neuroendocrine tumors, a new therapeutical approach, peptide receptor radionuclide therapy has been developed recently. There are two important features which make this therapy feasible: somatostatin receptors are strongly over-expressed in most neuroendocrine tumors resulting in a high tumor-to-background ratio and internalization of the somatostatin-receptor complex in neuroendocrine cells. Due to these features, neuroendocrine tumors can be treated with radiolabelled somatostatin analogues. For peptide receptor radionuclide therapy, somatostatin analogues are conjugated to a chelator that can bind a radionuclide. The most frequently used radionuclides for neuroendocrine tumor treatment are the β-emitter Yttrium-90 (90Y) and the β+γ emitter Lutetium-177 (177Lu). Candidates for somatostatin receptor endoradiotherapy are patients with progressive, metastatic, somatostatin-receptor positive neuroendocrine tumors. Many patients have been successively treated with this approach: according to international results major remission can be achieved in 25% of the cases. Although this therapy is still unavailable in Hungary, Hungarian patients can be treated with somatostatin receptor endoradiotherapy with financial support from the National Health Fund in a co-operation with the University of Basel since 2005. During the past 5 years, 51 Hungarian patients have been treated with this therapy. This review briefly summarizes the theoretical background, indications, effectiveness and side effects of somatostatin receptor endoradiotherapy and the authors present the first data obtained from Hungarian patients. Orv. Hetil., 2011, 152, 392–397.

scholarly journals Somatostatin Receptor-Based Molecular Imaging and Therapy for Neuroendocrine Tumors

2013 ◽  
Vol 2013 ◽  
pp. 1-11 ◽  
Author(s):  
Ling Wang ◽  
Kun Tang ◽  
Qi Zhang ◽  
Huanbin Li ◽  
Zhengwei Wen ◽  
...  
Keyword(s):  
Molecular Imaging ◽  
Neuroendocrine Tumors ◽  
Somatostatin Receptor ◽  
The Body ◽  
Neuroendocrine Cells ◽  
Whole Body ◽  
Receptor Imaging ◽  
Whole Body Imaging ◽  
Tumor Type ◽  
Beta Emitters

Neuroendocrine tumors (NETs) are tumors originated from neuroendocrine cells in the body. The localization and the detection of the extent of NETs are important for diagnosis and treatment, which should be individualized according to the tumor type, burden, and symptoms. Molecular imaging of NETs with high sensitivity and specificity is achieved by nuclear medicine method using single photon-emitting and positron-emitting radiopharmaceuticals. Somatostatin receptor imaging (SRI) using SPECT or PET as a whole-body imaging technique has become a crucial part of the management of NETs. The radiotherapy with somatostatin analogues labeled with therapeutic beta emitters, such as lutetium-177 or yttrium-90, has been proved to be an option of therapy for patients with unresectable and metastasized NETs. Molecular imaging can deliver an important message to improve the outcome for patients with NETs by earlier diagnosis, better choice of the therapeutic method, and evaluation of the therapeutic response.

Diagnostic discrepancies between second-opinion and referring pathology reports of neuroendocrine tumors.

2013 ◽  
Vol 31 (4_suppl) ◽  
pp. 154-154
Author(s):  
Hillary Lin ◽  
Teri A. Longacre ◽  
Vilay H. Khandelwal ◽  
Raymond R. Balise ◽  
Pamela L. Kunz
Keyword(s):  
Neuroendocrine Tumors ◽  
The Body ◽  
Neuroendocrine Cells ◽  
University Hospital ◽  
Second Opinion ◽  
Systematic Evaluation ◽  
Additional Time ◽  
Future Studies ◽  
Clinical Diagnoses ◽  
Pathology Reports

154 Background: Neuroendocrine tumors (NETs) are rare malignancies that can arise from neuroendocrine cells throughout the body, most commonly in the lungs, gastrointestinal tract, and pancreas. The nomenclature and histologic classification schemes for NETs have historically been heterogeneous and inconsistent. Klimstra, et al published a set of NET pathology guidelines in 2010. We undertook a systematic evaluation of discrepancies between referring versus second-opinion NET pathology reports using these guidelines as our reference. Methods: We developed a cohort ofall NET cases seen at Stanford University Hospital between April 1998 and February 2012 with available Stanford and referring pathology reports for the same specimen to identify the discrepancies between their clinical diagnoses. Results: Of 141 cases, primary sites were identified as 39 (28%) pancreas, 21 (15%) small intestine, 49 (35%) other NET sites, and 32 (22%) unknown or missing. Most reports agreed on a diagnosis of NET, although twelve different terms were used to describe the NET diagnosis. There were 24 cases (17%) with major discrepancies by histologic description (NET vs. not NET). Grade, mitotic index (MI), and Ki67 were among the variables examined; they were missing from the majority of cases in one or both reports, yet more likely to be included in the Stanford report. Grade was not reported (NR) in one or both reports in 124 cases (88%); of the 17 cases reporting grade in both reports, 3 had major discrepancies. MI was NR in one or both reports in 105 cases (74%); of the 36 cases reporting MI in both reports, 5 had major discrepancies. Ki67 was NR in one or both reports in 127 cases (90%); of the 14 cases reporting Ki67 in both reports, 3 had major discrepancies. Conclusions: Clinically relevant differences were frequently found between Stanford and referring pathology reports for NETs. Our results suggest that it is beneficial for NET cases to be reviewed by NET pathology experts given the potential to impact treatment. Future studies of NET pathology discrepancies are warranted to allow additional time for adoption of the 2010 guidelines.

scholarly journals Retrospective analysis of primary neuroendocrine tumors of the ovary: Management and outcomes

2021 ◽  
Author(s):  
li pang ◽  
zhiqiang guo
Keyword(s):  
Prognostic Factors ◽  
Neuroendocrine Tumors ◽  
Early Stage ◽  
Treatment Strategies ◽  
Histological Type ◽  
Comprehensive Treatment ◽  
Cancer Specific Survival ◽  
Ajcc Stage ◽  
Low Incidence ◽  
Patient Prognosis

Abstract Background Due to the low incidence of ovarian neuroendocrine tumors (NETs), clinicians may be unaware of appropriate treatments for the disease and factors influencing patient prognosis, which may cause them to miss the window of opportunity for treatment. Moreover, there is currently no recognized first-line treatment strategy, and no studies have reported prognostic statistics derived from large samples. This retrospective study aimed to investigate the clinical behavior of ovarian NETs. Methods The Surveillance, Epidemiology, and End Results database was used to identify women diagnosed with ovarian NETs from 2004 to 2015. Overall survival (OS), cancer-specific survival (CSS), and independent prognostic factors for ovarian NETs were evaluated. The effects of different treatments on prognosis were also compared, as were OS and CSS rates for histological subtypes. Results The 5-year OS rates were 83.3%, 30.0%, 20.3%, and 9.8% for patients in stages I (n = 159), II (n = 23), III (n = 101), and IV (n = 148), respectively. The 5-year CSS rates were 85.6%, 41.7%, 21.2%, and 9.8% for patients in stages I–IV, respectively. Age, American Joint Committee on Cancer (AJCC) stage, lymph node metastasis, treatment, and histological type were related to poor OS and CSS. In the early stage, the 5-year OS and CSS rates were 97.03% and 96.90%, respectively. For patients in the advanced stage receiving comprehensive treatment (surgery + chemotherapy + radiotherapy), 5-year OS and CSS rates were 72.9% and 70.00%, respectively. When comparing low- and high-grade neuroendocrine carcinoma, the 5-year OS rates were 93.96% vs. 7.01%, 5-year CSS rates were 97.44% vs. 7.31%, 10-year OS rates were 93.56% vs. 2.34%, and 10-year CSS rates were 97.44% vs. 4.88%, respectively. Conclusion Age, AJCC stage, treatment, and histological type are independent prognostic factors of ovarian NETs. Prognosis is relatively good for early-stage cases treated with surgery alone, whereas more comprehensive treatment is required to improve prognosis for advanced cases. Future studies should focus on the development of individualized treatment strategies for prolonging survival time in patients with ovarian NETs.

scholarly journals 99mTc-EDDA/HYNIC-TOC is a New Opportunity in Neuroendocrine Tumors of the Lung (and in other Malignant and Benign Pulmonary Diseases)

2020 ◽  
Vol 13 (3) ◽  
pp. 166-176
Author(s):  
Vittorio Briganti ◽  
Vincenzo Cuccurullo ◽  
Valentina Berti ◽  
Giuseppe D. Di Stasio ◽  
Flavia Linguanti ◽  
...  
Keyword(s):  
Lung Cancer ◽  
Neuroendocrine Tumors ◽  
Somatostatin Receptors ◽  
Lung Neoplasm ◽  
Peptide Receptor Radionuclide Therapy ◽  
Diagnostic Algorithm ◽  
Neuroendocrine Cells ◽  
Small Cells ◽  
Contrast Enhanced Ct

Neuroendocrine tumors (NETs) consist of a relatively rare spectrum of malignancies that can arise from neuroendocrine cells; lung NETs (L-NETs) represent about 25% of primary lung neoplasm and 10% of all carcinoid tumors. Diagnostic algorithm usually takes into consideration chest Xray, contrast-enhanced CT and MRI. Nuclear medicine plays a crucial role in the detection and correct assessment of neoplastic functional status as it provides in vivo metabolic data related to the overexpression of Somatostatin Receptors (SSTRs) and also predicting response to peptide receptor radionuclide therapy (PRRT). 111In-Pentreotide (Octreoscan®) is commercially available for imaging of neuroendocrine tumors, their metastases and the management of patients with NETs. More recently, 99mTc-EDDA/HYNIC-TOC(Tektrotyd®) was introduced into the market and its use has been approved for imaging of patients with L-NETs and other SSTR-positive tumors. 99mTc-EDDA/HYNIC-TOC could also represent a good alternative to 68Ga-DOTA-peptides (DOTA-TOC, DOTA-NOC, DOTATATE) in hospitals or centers where PET/CT or 68Ge/68Ga generators are not available. When compared to 111In-Pentetreotide, Tektrotyd® showed slightly higher sensitivity, in the presence of higher imaging quality and lower radiation exposure for patients. Interesting perspectives depending on the kinetic analysis allowed by Tektrotyd® may be obtained in differential diagnosis of non-small cells lung cancer (NSCLC) versus small cells lung cancer (SCLC) and NETs. An interesting perspective could be also associated with a surgery radio-guided by Tektrotyd® in operable lung tumors, including either NETs and NSCLC.

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