scholarly journals Imaging of Pancreatic Neuroendocrine Neoplasms

2021 ◽  
Vol 18 (17) ◽  
pp. 8895
Author(s):  
Giuditta Chiti ◽  
Giulia Grazzini ◽  
Diletta Cozzi ◽  
Ginevra Danti ◽  
Benedetta Matteuzzi ◽  
...  
Keyword(s):  
Functional Imaging ◽  
Imaging Techniques ◽  
Therapy Monitoring ◽  
Somatostatin Receptor ◽  
World Health ◽  
Biological Behavior ◽  
Pancreatic Tumors ◽  
Neuroendocrine Neoplasms ◽  
Imaging Features ◽  
Pancreatic Neuroendocrine Neoplasms

Pancreatic neuroendocrine neoplasms (panNENs) represent the second most common pancreatic tumors. They are a heterogeneous group of neoplasms with varying clinical expression and biological behavior, from indolent to aggressive ones. PanNENs can be functioning or non-functioning in accordance with their ability or not to produce metabolically active hormones. They are histopathologically classified according to the 2017 World Health Organization (WHO) classification system. Although the final diagnosis of neuroendocrine tumor relies on histologic examination of biopsy or surgical specimens, both morphologic and functional imaging are crucial for patient care. Morphologic imaging with ultrasonography (US), computed tomography (CT) and magnetic resonance imaging (MRI) is used for initial evaluation and staging of disease, as well as surveillance and therapy monitoring. Functional imaging techniques with somatostatin receptor scintigraphy (SRS) and positron emission tomography (PET) are used for functional and metabolic assessment that is helpful for therapy management and post-therapeutic re-staging. This article reviews the morphological and functional imaging modalities now available and the imaging features of panNENs. Finally, future imaging challenges, such as radiomics analysis, are illustrated.

Multifocal Pancreatic Neuroendocrine Neoplasms In Tuberous Sclerosis: A Rare Case

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S77-S77
Author(s):  
N C Jadhav ◽  
D L Gang
Keyword(s):  
Tuberous Sclerosis ◽  
Neurofibromatosis Type ◽  
Abdominal Ultrasound ◽  
Biologically Active ◽  
Pancreatic Tumors ◽  
Neuroendocrine Neoplasms ◽  
Ki 67 ◽  
Pancreatic Neuroendocrine Neoplasms ◽  
Von Hippel Lindau ◽  
Multifocal Disease

Abstract Casestudy: Pancreatic neuroendocrine neoplasms (PanNEN) are rare accounting for 2-5% of pancreatic tumors. Although mostly sporadic, 10-20% are associated with inherited syndromes, notably MEN-1, Von Hippel- Lindau disease, neurofibromatosis type 1, and tuberous sclerosis (TS). When compared to sporadic cases, PanNEN in hereditary syndromes occur at a younger age, are often multifocal, cystic, and may show characteristic microscopic patterns. TS is an autosomal dominant multi-system disorder with mutations involving TSC1 or TSC2 genes which function as tumor suppressors by inhibiting mTORC1 kinase. PanNEN is observed in 1.5-1.8% of patients with TS and no surveillance guidelines for the assessment of pancreatic lesions are established. Compared to other syndromes, PanNEN associated with TS are solitary. To our knowledge, only two cases of multifocal PanNEN in TS patients have been reported. We present a case of a 67-year-old gentleman with a history of TS also affecting two daughters. He presented to the emergency department with severe abdominal pain. Abdominal ultrasound suggested acute appendicitis and an incidental 2.0 cm solid lesion was noted in the head of the pancreas. Follow-up MRI revealed two additional non-cystic masses in the pancreatic tail. Endoscopic ultrasound-guided biopsy of a tail lesion revealed monomorphic tumor cells with stippled chromatin without cytologic atypia. Immunohistochemical staining was positive for synaptophysin and chromogranin. Ki-67 labelling index was under 1%. Diagnosis of a well-differentiated neuroendocrine tumor (G1) was made. The patient denied symptoms of the carcinoid syndrome and no biologically active hormones were detected. Gallium PET scan revealed multiple foci of radiotracer uptake throughout the pancreas in addition to those described on MRI. Although PanNEN are rare in TS, malignant behavior has been reported. This case reinforces the importance of early detection through active surveillance, especially as surgical options may be limited in multifocal disease.

scholarly journals CD56 Expression Is Associated with Biological Behavior of Pancreatic Neuroendocrine Neoplasms

2020 ◽  
Vol Volume 12 ◽  
pp. 4625-4631
Author(s):  
Xin Chen ◽  
Chuangen Guo ◽  
Wenjing Cui ◽  
Ke Sun ◽  
Zhongqiu Wang ◽  
...  
Keyword(s):  
Biological Behavior ◽  
Neuroendocrine Neoplasms ◽  
Pancreatic Neuroendocrine Neoplasms ◽  
Cd56 Expression

scholarly journals Diagnostic and Interventional Role of Endoscopic Ultrasonography for the Management of Pancreatic Neuroendocrine Neoplasms

2021 ◽  
Vol 10 (12) ◽  
pp. 2638
Author(s):  
Giuseppinella Melita ◽  
Socrate Pallio ◽  
Andrea Tortora ◽  
Stefano Francesco Crinò ◽  
Antonio Macrì ◽  
...  
Keyword(s):  
Tumor Burden ◽  
Pancreatic Tumors ◽  
Proliferation Index ◽  
Neuroendocrine Neoplasms ◽  
Ki 67 ◽  
Ethanol Injection ◽  
Pancreatic Neuroendocrine Neoplasms ◽  
Reduce Tumor Burden ◽  
Precise Diagnosis

Pancreatic neuroendocrine neoplasms (PanNENs) are relatively rare, but their incidence has increased significantly in the last decades. Precise diagnosis and prognostic stratification are crucial for proper patient management. Endoscopic ultrasound (EUS) is the modality of choice for diagnosis of solid pancreatic tumors, showing a higher tumor detection rate than other imaging modalities, especially for small size lesions. EUS also serves as a guide for preoperative sampling and other interventions. EUS-tissue acquisition is a safe and highly accurate technique for cyto/histological diagnosis of PanNENs with a well-demonstrated correlation between Ki-67 proliferation index values and tumor grading on EUS and surgical specimens according to the WHO 2017 classification. Furthermore, the possibility of a preoperative EUS-guided fine needle tattooing or fiducial markers placement may help the surgeon to locate small and deep tumors, thus avoiding formal pancreatic resections in favor of parenchymal-sparing surgery. Finally, locoregional ablative treatments using either ethanol injection or radiofrequency ablation have been proposed in recent studies with promising results in order to control symptoms or reduce tumor burden in selected patients unfit for surgery with functioning or non-functioning PanNENs. This article review highlights the current role of EUS in PanNENs management, focusing on the present and future applications of EUS-guided interventions.

scholarly journals 68Ga-DOTATATE PET in Pediatric Paraganglioma / Pheochromocytoma: A Case-series Highlighting the Role of Functional Imaging

2021 ◽  
Author(s):  
Aleksandra Augustynowicz ◽  
Neha Kwatra ◽  
Laura Drubach ◽  
Christopher Weldon ◽  
Katherine Janeway ◽  
...  
Keyword(s):  
Functional Imaging ◽  
Imaging Techniques ◽  
Somatostatin Receptor ◽  
Case Series ◽  
Response Assessment ◽  
Receptor Expression ◽  
Imaging Modalities ◽  
Nuclear Medicine Imaging ◽  
Decision Algorithm ◽  
Pet Ct

Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors in childhood. Cancer predisposition syndromes (CPS) are increasingly recognized as the underlying cause for a number of pediatric malignancies and up to 40% of PPGL are currently thought to be associated with a hereditary predisposition1,2. With the increasingly widespread availability of functional molecular imaging techniques, nuclear medicine imaging modalities such as 18F-FDG-PET/CT, 123I-MIBG SPECT/CT, and 68Ga-DOTATATE PET/CT now play an essential role in the staging, response assessment and determination of suitability for targeted radiotherapy in patients with PPGL. Each of these imaging modalities targets a different cellular characteristic, such as glucose metabolism (FDG), norepinephrine transporter expression (MIBG), or somatostatin receptor expression (DOTATATE), and therefore can be complementary to anatomic imaging and to each other. Given the recent FDA approval3 and increasing use of 68Ga-DOTATATE for imaging in children4, the purpose of this article is to use a case-based approach to highlight both the advantages and limitations of DOTATATE imaging as it compares to current radiologic imaging techniques in the staging and response assessment of pediatric PPGL, and to offer a decision algorithm for the use of functional imaging that can be applied to PPGL, as well as other neuroendocrine malignancies.

Competitive Testing of the WHO 2010 versus the WHO 2017 Grading of Pancreatic Neuroendocrine Neoplasms: Data from a Large International Cohort Study

2018 ◽  
Vol 107 (4) ◽  
pp. 375-386 ◽  
Author(s):  
Guido Rindi ◽  
Catherine Klersy ◽  
Luca Albarello ◽  
Eric Baudin ◽  
Antonio Bianchi ◽  
...  
Keyword(s):  
Regression Analysis ◽  
Cox Regression ◽  
World Health ◽  
Neuroendocrine Neoplasms ◽  
Grading System ◽  
Cox Regression Analysis ◽  
Pancreatic Neuroendocrine Neoplasms ◽  
Grading Systems ◽  
Significant Difference ◽  
Explained Variation

Background: The World Health Organization (WHO) and the American Joint Cancer Committee (AJCC) modified the grading of pancreatic neuroendocrine neoplasms from a three-tier (WHO-AJCC 2010) to a four-tier system by introducing the novel category of NET G3 (WHO-AJCC 2017). Objectives: This study aims at validating the WHO-AJCC 2017 and identifying the most effective grading system. Method: A total of 2,102 patients were enrolled; entry criteria were: (i) patient underwent surgery; (ii) at least 2 years of follow-up; (iii) observation time up to 2015. Data from 34 variables were collected; grading was assessed and compared for efficacy by statistical means including Kaplan-Meier method, Cox regression analysis, Harrell’s C statistics, and Royston’s explained variation in univariable and multivariable analyses. Results: In descriptive analysis, the two grading systems demonstrated statistically significant differences for the major category sex but not for age groups. In Cox regression analysis, both grading systems showed statistically significant differences between grades for OS and EFS; however, no statistically significant difference was observed between the two G3 classes of WHO-AJCC 2017. In multivariable analysis for the two models fitted to compare efficacy, the two grading systems performed equally well with substantially similar optimal discrimination and well-explained variation for both OS and EFS. The WHO-AJCC 2017 grading system retained statistically significant difference between the two G3 classes for OS but not for EFS. Conclusions: The WHO-AJCC 2017 grading system is at least equally performing as the WHO-AJCC 2010 but allows the successful identification of the most aggressive PanNET subgroup. Grading is confirmed as probably the most powerful tool for predicting patient survival.

scholarly journals World Health Organization Grading Classification for Pancreatic Neuroendocrine Neoplasms: A Comprehensive Analysis from a Large Chinese Institution

2020 ◽  
Author(s):  
Min Yang ◽  
Lin Zeng ◽  
Neng-wen Ke ◽  
Chun-lu Tan ◽  
Bo-le Tian ◽  
...  
Keyword(s):  
World Health Organization ◽  
World Health ◽  
Neuroendocrine Neoplasms ◽  
The Novel ◽  
Pancreatic Neuroendocrine Neoplasms ◽  
Significant Survival ◽  
Prognostic Stratification ◽  
Long Term Prognosis ◽  
Health Organization ◽  
Survival Differences

Abstract Background Pancreatic neuroendocrine neoplasms (p-NENs) are a group of highly heterogeneous tumors with distinct clinicopathological features and long-term prognosis. In 2017, in order to better stratify patients into prognostic groups and predicting their outcomes, World Health Organization (WHO) officially updated its grading system for p-NENs which distinguished these neoplasms among Grading 1 (G1) pancreatic neuroendocrine tumors (p-NETs), G2 p-NETs, G3 p-NETs and G3 pancreatic neuroendocrine carcinomas (p-NECs). However, this new grading classification for p-NENs has not yet been rigorously validated.Methods Data of patients who were surgically treated and histopathologically diagnosed as p-NENs at West China Hospital of Sichuan University from January 2002 to December 2018 were retrospectively collected and analyzed according the novel WHO 2017 grading classification.Results We eventually enrolled 480 eligible patients with p-NENs in our present study, in which 150 patients with WHO 2017 G1 p-NETs, 158 with G2 p-NETs, 64 with G3 p-NETs and 108 with G3 p-NECs were identified. The estimated 5-year overall survival for patients with G1 p-NETs, G2 p-NETs, G3 p-NETs and G3 p-NECs was 75.8%, 58.4%, 35.1% and 11.1%, with a median survival time of 85.3mons, 67.4mons, 51.3mons and 26.8mons, respectively. Patients with G2 p-NETs present notably worse survival than those with G1 p-NETs (P = 0.03). Survival of G3 p-NETs were significantly worse than that of G1 p-NETs or G2 p-NETs (P < 0.001, P = 0.023, respectively), as well as that when comparing G3 p-NECs with G1 p-NETs or G2 p-NETs (P < 0.001, P < 0.001, respectively). Patients with G3 p-NECs showed statistically shorter survival than those with G3 p-NETs (P < 0.001). Both WHO 2017 and 2010 grading criteria could be independent predictor for the OS of p-NENs (P = 0.016, P = 0.022; respectively). The 95% confidence intervals of WHO 2017 grading classification (0.983–9.454) was slightly smaller than that of WHO 2010 criteria (0.201–13.374), indicating a relatively more accurate predicting ability for the prognosis of p-NENs.Conclusion The WHO 2017 grading classification for p-NENs could successfully allocate patients into four groups with distinct clinical features and significant survival differences, which might be superior to the WHO 2010 criteria for its better prognostic stratification and more accurate predicting ability.

scholarly journals Well-differentiated gastroenteropancreatic G3 NET: findings from a large single centre cohort

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
K. Lithgow ◽  
H. Venkataraman ◽  
S. Hughes ◽  
H. Shah ◽  
J. Kemp-Blake ◽  
...  
Keyword(s):  
Primary Tumour ◽  
Somatostatin Receptor ◽  
Somatostatin Analogs ◽  
Treatment Strategies ◽  
Biological Behavior ◽  
Queen Elizabeth Hospital ◽  
Neuroendocrine Neoplasms ◽  
Single Centre ◽  
Net G3 ◽  
Well Differentiated

AbstractNeuroendocrine neoplasms are known to have heterogeneous biological behavior. G3 neuroendocrine tumours (NET G3) are characterized by well-differentiated morphology and Ki67 > 20%. The prognosis of this disease is understood to be intermediate between NET G2 and neuroendocrine carcinoma (NEC). Clinical management of NET G3 is challenging due to limited data to inform treatment strategies. We describe clinical characteristics, treatment, and outcomes in a large single centre cohort of patients with gastroenteropancreatic NET G3. Data was reviewed from 26 cases managed at Queen Elizabeth Hospital, Birmingham, UK, from 2012 to 2019. Most commonly the site of the primary tumour was unknown and majority of cases with identifiable primaries originated in the GI tract. Majority of cases demonstrated somatostatin receptor avidity. Median Ki67 was 30%, and most cases had stage IV disease at diagnosis. Treatment options included surgery, somatostatin analogs (SSA), and chemotherapy with either platinum-based or temozolomide-based regimens. Estimated progression free survival was 4 months following initiation of SSA and 3 months following initiation of chemotherapy. Disease control was observed following treatment in 5/11 patients treated with chemotherapy. Estimated median survival was 19 months; estimated 1 year survival was 60% and estimated 2 year survival was 13%. NET G3 is a heterogeneous group of tumours and patients which commonly have advanced disease at presentation. Prognosis is typically poor, though select cases may respond to treatment with SSA and/or chemotherapy. Further study is needed to compare efficacy of different treatment strategies for this disease.

scholarly journals Management of Asymptomatic Sporadic Nonfunctioning Pancreatic Neuroendocrine Neoplasms (ASPEN) ≤2 cm: Study Protocol for a Prospective Observational Study

2020 ◽  
Vol 7 ◽  
Author(s):  
Stefano Partelli ◽  
John K. Ramage ◽  
Sara Massironi ◽  
Alessandro Zerbi ◽  
Hong Beom Kim ◽  
...  
Keyword(s):  
Active Surveillance ◽  
Imaging Techniques ◽  
Progression Free Survival ◽  
Operative Management ◽  
Needle Aspiration ◽  
Neuroendocrine Neoplasms ◽  
Free Survival ◽  
Pancreatic Neuroendocrine Neoplasms ◽  
Non Operative Management ◽  
Quality Imaging

Introduction: The optimal treatment for small, asymptomatic, nonfunctioning pancreatic neuroendocrine neoplasms (NF-PanNEN) is still controversial. European Neuroendocrine Tumor Society (ENETS) guidelines recommend a watchful strategy for asymptomatic NF-PanNEN &lt;2 cm of diameter. Several retrospective series demonstrated that a non-operative management is safe and feasible, but no prospective studies are available. Aim of the ASPEN study is to evaluate the optimal management of asymptomatic NF-PanNEN ≤2 cm comparing active surveillance and surgery.Methods: ASPEN is a prospective international observational multicentric cohort study supported by ENETS. The study is registered in ClinicalTrials.gov with the identification code NCT03084770. Based on the incidence of NF-PanNEN the number of expected patients to be enrolled in the ASPEN study is 1,000 during the study period (2017–2022). Primary endpoint is disease/progression-free survival, defined as the time from study enrolment to the first evidence of progression (active surveillance group) or recurrence of disease (surgery group) or death from disease. Inclusion criteria are: age &gt;18 years, the presence of asymptomatic sporadic NF-PanNEN ≤2 cm proven by a positive fine-needle aspiration (FNA) or by the presence of a measurable nodule on high-quality imaging techniques that is positive at 68Gallium DOTATOC-PET scan.Conclusion: The ASPEN study is designed to investigate if an active surveillance of asymptomatic NF-PanNEN ≤2 cm is safe as compared to surgical approach.

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