scholarly journals Multiple neuroendocrine tumors of the pancreas associated with pancreas divisum

2004 ◽  
pp. 837-840 ◽  
Author(s):  
A Raffel ◽  
R Engers ◽  
K Cupisti ◽  
M Krausch ◽  
H Kreuz ◽  
...  

Pancreas divisum is the most common congenital anomaly of the pancreas, characterized by missing fusion of the ventral and dorsal pancreatic duct. It may cause pancreatitis, but is rarely associated with malignancy.We report herein for the first time the rare association, in a symptomless patient, of multiple neuroendocrine tumors of the pancreas with pancreas divisum and a failure of the exocrine system. Diagnosis was made incidentally by routine abdominal ultrasound. Laboratory examinations and a fine-needle aspiration revealed the neuroendocrine nature of the tumor. Spleen-preserving left pancreas resection was performed, with evidence of multiple neuroendocrine tumors of the pancreas with the typical histological characteristics. Eighteen months later the patient is still free of tumor burden.

1996 ◽  
Vol 35 (2) ◽  
pp. 237
Author(s):  
Dong Sik Choi ◽  
Dong Ho Lee ◽  
Young Tae Ko ◽  
Tae Il Han ◽  
Youp Yoon ◽  
...  

2016 ◽  
Vol 25 (3) ◽  
pp. 317-321 ◽  
Author(s):  
Raffaele Manta ◽  
Elisabetta Nardi ◽  
Nico Pagano ◽  
Claudio Ricci ◽  
Mariano Sica ◽  
...  

Background & Aims: Diagnosis of pancreatic neuroendocrine tumors (p-NETs) is frequently challenging. We describe a large series of patients with p-NETs in whom both pre-operative Computed Tomography (CT) and Endoscopic Ultrasonography (EUS) were performed. Methods: This was a retrospective analysis of prospectively collected sporadic p-NET cases. All patients underwent both standard multidetector CT study and EUS with fine-needle aspiration (FNA). The final histological diagnosis was achieved on a post-surgical specimen. Chromogranin A (CgA) levels were measured. Results: A total of 80 patients (mean age: 58 ± 14.2 years; males: 42) were enrolled. The diameter of functioning was significantly lower than that of non-functioning p-NETs (11.2 ± 8.5 mm vs 19.8 ± 12.2 mm; P = 0.0004). The CgA levels were more frequently elevated in non-functioning than functioning pNET patients (71.4% vs 46.9%; P = 0.049). Overall, the CT study detected the lesion in 51 (63.7%) cases, being negative in 26 (68.4%) patients with a tumor ≤10 mm, and in a further 3 (15%) cases with a tumor diameter ≤20 mm. CT overlooked the pancreatic lesion more frequently in patients with functioning than non-functioning p-NETs (46.5% vs 24.3%; P = 0.002). EUS allowed a more precise pre-operative tumor measurement, with an overall incorrect dimension in only 9 (11.2%) patients. Of note, the EUS-guided FNA suspected the neuroendocrine nature of tumor in all cases. Conclusions: Data of this large case series would suggest that the EUS should be included in the diagnostic work-up in all patients with a suspected p-NET, even when the CT study was negative for a primary lesion in the pancreas.– . Abbrevations: CgA: chromogranin A; EUS: Endoscopic Ultrasonography; FNA: fine-needle aspiration; p-NETs: pancreatic neuroendocrine tumors.


2021 ◽  
Vol 09 (07) ◽  
pp. E1164-E1170
Author(s):  
David M. de Jong ◽  
Pauline M. Stassen ◽  
Jan Werner Poley ◽  
Paul Fockens ◽  
Robin Timmer ◽  
...  

Abstract Background and study aims Although the majority of patients with pancreas divisum (PDiv) are asymptomatic, a subgroup present with recurrent pancreatitis or pain for which endoscopic therapy may be indicated. The aim of this study was to evaluate success rates and long-term outcomes of endoscopic treatment in patients with symptomatic PDiv. Patients and methods A multicenter, retrospective cohort study was performed. Patients with symptomatic PDiv presenting with recurrent acute pancreatitis (RAP), chronic pancreatitis (CP), or chronic abdominal pancreatic-type pain (CAP) who underwent endoscopic retrograde cholangiopancreatography (ERCP) between January 2000 and December 2019 were included. The primary outcome was clinical success, defined as either no recurrent episode of acute pancreatitis (AP) for RAP patients, no flares for CP patients, or absence of abdominal pain for patients with CAP after technically successful ERCP. Results In 60 of 81 patients (74.1 %) a technically successful papilla minor intervention was performed. Adverse events were reported in 30 patients (37 %), with post-ERCP pancreatitis in 18 patients. The clinical success rate for patients with at least 3 months of follow-up was 42.6 %, with higher rates of success among patients presenting with RAP (44.4 %) as compared to those with CP (33.3 %) or CAP (33.3 %). Long-term sustained response was present in 40.9 % of patients with a technically successful intervention. In patients with RAP who did not completely respond to treatment, the mean number of AP episodes after treatment decreased significantly from 3.5 to 1.1 per year, and subsequently the interval between AP episodes increased from 278 to 690 days (P = 0.0006). A potential predictive factor of failure of clinical success after technically successful ERCP, at univariate analysis, was male sex (OR = 0.25, P = 0.02). Conclusions Endoscopic therapy in patients with symptomatic PDiv is moderately effective, with its highest yield in patients presenting with RAP. Future studies are needed to assess factors predictive for success of endoscopic therapy and potential risk factors for relapse after ERCP.


Diagnostics ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 504
Author(s):  
Fiona Ohlendorf ◽  
Rudolf Werner ◽  
Christoph Henkenberens ◽  
Tobias Ross ◽  
Hans Christiansen ◽  
...  

Tumor microenvironment inflammation contributes to the proliferation and survival of malignant cells, angiogenesis, metastasis, subversion of adaptive immunity, and reduced treatment response. We aimed to evaluate the early predictive and prognostic significance of markers of systemic inflammation in patients receiving somatostatin-receptor targeted peptide receptor radionuclide therapy (PRRT). This retrospective observational cohort study included 33 patients with advanced gastro-entero-pancreatic neuroendocrine tumors (GEP-NETs) treated with PRRT. Pretreatment blood-based inflammatory biomarkers, e.g., Creactive protein levels (CRP), white blood cell count (WBC), and absolute neutrophil count (ANC), were documented and inflammation indexes, e.g., neutrophil-lymphocyte ratio (NLR) and Platelet × CRP multiplier (PCM), were calculated. Tumor burden was determined using [68Ga]GaDOTATATE PET/CT before enrollment and every 2 cycles thereafter until progression. Therapy response was assessed using RECIST 1.1, including its volumetric modification. Inflammatory biomarkers and inflammatory indexes demonstrated marked heterogeneity among patients, and were significantly higher in non-responders (e.g., CRP (P < 0.001), ANC (P = 0.002), and PCM (P < 0.001)). Change in whole-body tumor burden after two cycles of PRRT was significantly associated with CRP (P = 0.0157) and NLR (P = 0.0040) in multivariate regression analysis. A cut-off of 2.5 mg/L for CRP (AUC = 0.84, P = 0.001) revealed a significant outcome difference between patients with adversely high vs. low CRP (median PFS 508 days vs. not yet reached (HR = 4.52; 95% CI, 1.27 to 16.18; P = 0.02)). Tumor-driven systemic inflammatory networks may be associated with treatment response, change in tumor burden, and prognosis in patients with GEPNETs receiving PRRT.


2021 ◽  
Author(s):  
Lauren M Raymond ◽  
Tetiana Korzun ◽  
Adel Kardosh ◽  
Kenneth J. Kolbeck ◽  
Rodney Pommier ◽  
...  

Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are the most common form of neuroendocrine neoplasia, but there is no current consensus for the sequencing of approved therapies, particularly with respect to peptide receptor radionuclide therapy (PRRT). This comprehensive review evaluates the data supporting approved therapies for GEP-NETs and recommendations for therapeutic sequencing with a focus on how PRRT currently fits within sequencing algorithms. The current recommendations for PRRT sequencing restrict its use to metastatic, inoperable, progressive midgut NETs, however, this may change with emerging data to suggest PRRT might be beneficial as neoadjuvant therapy for inoperable tumors, is more tolerable than other treatment modalities following first-line standard dose somatostatin analogues, and can be used as salvage therapy after disease relapse following prior successful cycles of PRRT. PRRT has also been shown to reduce tumor burden, improve quality of life, and prolong the time to disease progression in a broad spectrum of patients with GEP-NETs. As the various potential benefits of PRRT in GEP-NET therapy continues to expand, it is necessary to review and critically evaluate our treatment algorithms for GEP-NETs.


1989 ◽  
Vol 13 (1) ◽  
pp. 140-141 ◽  
Author(s):  
Paul M. Silverman ◽  
Leon McVay ◽  
Robert K. Zeman ◽  
Brian S. Garra ◽  
Edward G. Grant ◽  
...  

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