scholarly journals Preventive medicine of von Hippel–Lindau disease-associated pancreatic neuroendocrine tumors

2018 ◽  
Vol 25 (9) ◽  
pp. 783-793 ◽  
Author(s):  
Tobias Krauss ◽  
Alfonso Massimiliano Ferrara ◽  
Thera P Links ◽  
Ulrich Wellner ◽  
Irina Bancos ◽  
...  

Pancreatic neuroendocrine tumors (PanNETs) are rare in von Hippel–Lindau disease (VHL) but cause serious morbidity and mortality. Management guidelines for VHL-PanNETs continue to be based on limited evidence, and survival data to guide surgical management are lacking. We established the European-American-Asian-VHL-PanNET-Registry to assess data for risks for metastases, survival and long-term outcomes to provide best management recommendations. Of 2330 VHL patients, 273 had a total of 484 PanNETs. Median age at diagnosis of PanNET was 35 years (range 10–75). Fifty-five (20%) patients had metastatic PanNETs. Metastatic PanNETs were significantly larger (median size 5 vs 2 cm; P < 0.001) and tumor volume doubling time (TVDT) was faster (22 vs 126 months; P = 0.001). All metastatic tumors were ≥2.8 cm. Codons 161 and 167 were hotspots for VHL germline mutations with enhanced risk for metastatic PanNETs. Multivariate prediction modeling disclosed maximum tumor diameter and TVDT as significant predictors for metastatic disease (positive and negative predictive values of 51% and 100% for diameter cut-off ≥2.8 cm, 44% and 91% for TVDT cut-off of ≤24 months). In 117 of 273 patients, PanNETs >1.5 cm in diameter were operated. Ten-year survival was significantly longer in operated vs non-operated patients, in particular for PanNETs <2.8 cm vs ≥2.8 cm (94% vs 85% by 10 years; P = 0.020; 80% vs 50% at 10 years; P = 0.030). This study demonstrates that patients with PanNET approaching the cut-off diameter of 2.8 cm should be operated. Mutations in exon 3, especially of codons 161/167 are at enhanced risk for metastatic PanNETs. Survival is significantly longer in operated non-metastatic VHL-PanNETs.

Surgery ◽  
1998 ◽  
Vol 124 (6) ◽  
pp. 1153-1159 ◽  
Author(s):  
Steven K. Libutti ◽  
Peter L. Choyke ◽  
David L. Bartlett ◽  
Hernan Vargas ◽  
McClellan Walther ◽  
...  

Surgery ◽  
2007 ◽  
Vol 142 (6) ◽  
pp. 814-818.e2 ◽  
Author(s):  
Joseph A. Blansfield ◽  
Lynda Choyke ◽  
Shane Y. Morita ◽  
Peter L. Choyke ◽  
James F. Pingpank ◽  
...  

Suizo ◽  
2016 ◽  
Vol 31 (2) ◽  
pp. 150-157
Author(s):  
Tatsunori MINAMIDE ◽  
Masaya WADA ◽  
Yohei TANIGUCHI ◽  
Masashi FUKUSHIMA ◽  
Shuko MORITA ◽  
...  

2015 ◽  
Vol 262 (2) ◽  
pp. 384-388 ◽  
Author(s):  
Louis de Mestier ◽  
Sébastien Gaujoux ◽  
Jérôme Cros ◽  
Olivia Hentic ◽  
Marie-Pierre Vullierme ◽  
...  

Endocrine ◽  
2021 ◽  
Author(s):  
F. Penitenti ◽  
L. Landoni ◽  
M. Scardoni ◽  
M. L. Piredda ◽  
S. Cingarlini ◽  
...  

Abstract Purpose Data regarding the clinical management and follow-up of pancreatic neuroendocrine tumors (PanNETs) associated with Von Hippel–Lindau (VHL) syndrome are limited. This study aimed to assess clinical presentation, genotype–phenotype correlations, treatment and prognosis of PanNETs in a series of VHL syndrome patients. Methods Retrospective analysis of data of patients observed between 2005 and 2020. Results Seventeen patients, including 12 probands and 5 relatives (mean age 30.8 ± 18.4; 7 males), were recruited. PanNETs were found in 13/17 patients (77.5%) at a median age of 37 years: 4/13 (30.7%) at the time of VHL diagnosis and 9 (69.3%) during follow up. Six (46.1%) PanNET patients underwent surgery, whereas seven were conservatively treated (mean tumor diameter: 40 ± 10.9 vs. 15 ± 5.3 mm respectively). Four patients (30.7%) had lymph node metastases and a mean tumor diameter significantly larger than the nonmetastatic PanNETs (44.2 ± 9.3 vs. 17.4 ± 7 mm, p = 0.00049, respectively). Five (83.3%) operated patients had stable disease after a median follow up of 3 years whereas one patient showed liver metastases. Six (85.7%) non-resected PanNETs were stable after a median follow-up of 2 years, whereas one patient developed a new small PanNET and a slight increase in diameter of a pre-existing PanNET. No correlation was found between the type of germline mutation and malignant behavior of PanNETs. Conclusions PanNETs are a common disease of the VHL syndrome and can be the presenting feature. Tumor size rather than genetic mutation is a prognostic factor of malignancy.


Surgery ◽  
2000 ◽  
Vol 128 (6) ◽  
pp. 1022-1028 ◽  
Author(s):  
Steven K. Libutti ◽  
Peter L. Choyke ◽  
H.Richard Alexander ◽  
Gladys Glenn ◽  
David L. Bartlett ◽  
...  

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