Gastro‐entero‐pancreatic neuroendocrine neoplasms ‐ prognostic features in primary and metastatic sites: grade, mesenteric tumor deposits and emerging novelties

Author(s):  
K Kankava ◽  
P Maisonneuve ◽  
A Mangogna ◽  
G Centonze ◽  
L Cattaneo ◽  
...  
2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S77-S77
Author(s):  
N C Jadhav ◽  
D L Gang

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.


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 ◽  
...  

Surgery ◽  
2017 ◽  
Vol 161 (5) ◽  
pp. 1235-1245 ◽  
Author(s):  
Roxanne Teo ◽  
Brian K.P. Goh ◽  
David W.M. Tai ◽  
John C. Allen ◽  
Tony K.H. Lim ◽  
...  

Pancreatology ◽  
2017 ◽  
Vol 17 (3) ◽  
pp. S43-S44
Author(s):  
Florian Sperling ◽  
Lars Nietfeld ◽  
Katharina Theuerkorn ◽  
Sebastian Krug ◽  
Heidi Griesmann ◽  
...  

2020 ◽  
Vol 9 (4) ◽  
pp. 289-298 ◽  
Author(s):  
Wentao Zhou ◽  
Tiantao Kuang ◽  
Xu Han ◽  
Wenqi Chen ◽  
Xuefeng Xu ◽  
...  

Objectives Systemic inflammation markers have been demonstrated to be associated with prognosis in various tumors. In this study, we aimed to assess the value of neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio, lymphocyte-to-monocyte ratio (LMR), systemic immune-inflammation index and the counts of lymphocyte, monocyte and neutrophil in predicting prognosis among patients with resected pancreatic neuroendocrine neoplasms (pNENs). Methods A total of 174 patients were included in the study. Univariate and multivariate analyses were performed to evaluate the predictive roles of inflammation markers for relapse-free survival (RFS) and overall survival (OS) in pNEN patients. Results The optimal cut-off values of NLR, LMR and lymphocyte count were 1.9, 5.0 and 1.4 × 109/L, respectively, determined by the X-tile software. RFS was found to be significantly longer in patients with NLR ≤1.9 (P = 0.041), LMR >5.0 (P < 0.001) and lymphocyte count >1.4 × 109/L (P = 0.002) in comparison to those with NLR >1.9, LMR ≤5.0 and lymphocyte count ≤1.4 × 109/L, respectively. Multivariate analysis revealed that LMR (hazard ratio 0.30, 95% CI 0.11–0.85, P = 0.023) was an independent predictor for RFS, but not NLR or lymphocyte count. For long-term survival analysis, patients with NLR ≤1.9 (P = 0.016) were found to be associated with favorable OS, but NLR was not an independent factor validated by multivariate analysis. Conclusions Preoperative LMR is an independent systemic inflammation marker to predict relapses in pNEN patients who underwent curative resections, whose clinical value needs to be verified in further large sample-based prospective studies.


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