Active Surveillance versus Surgery of Nonfunctioning Pancreatic Neuroendocrine Neoplasms ≤2 cm in MEN1 Patients

2016 ◽  
Vol 103 (6) ◽  
pp. 779-786 ◽  
Author(s):  
Stefano Partelli ◽  
Domenico Tamburrino ◽  
Caroline Lopez ◽  
Max Albers ◽  
Anna Caterina Milanetto ◽  
...  
Keyword(s):  
Active Surveillance ◽  
Neuroendocrine Neoplasms ◽  
Pancreatic Neuroendocrine Neoplasms

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 <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 >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.

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.

Systematic review of current prognostication systems for pancreatic neuroendocrine neoplasms

Surgery ◽  
2019 ◽  
Vol 165 (4) ◽  
pp. 672-685 ◽  
Author(s):  
Roxanne Y.A. Teo ◽  
Timothy Z. Teo ◽  
David W.M. Tai ◽  
Damien M. Tan ◽  
Simon Ong ◽  
...  
Keyword(s):  
Systematic Review ◽  
Neuroendocrine Neoplasms ◽  
Pancreatic Neuroendocrine Neoplasms
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