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- Krauss, Tobias, et al.
(författare)
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Preventive medicine of von Hippel-Lindau disease-associated pancreatic neuroendocrine tumors
- 2018
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Ingår i: Endocrine-Related Cancer. - : BIOSCIENTIFICA LTD. - 1351-0088 .- 1479-6821. ; 25:9, s. 783-793
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Tidskriftsartikel (refereegranskat)abstract
- 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.
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- Hansson, Petra, et al.
(författare)
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Coherence among phrases
- 2003
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Ingår i: [Host publication title missing]. ; , s. 607-610
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Konferensbidrag (refereegranskat)
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- Molander, Sverker, 1957, et al.
(författare)
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OMNIITOX - operational life-cycle impact assessment models and information tools for practitioners
- 2004
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Ingår i: International Journal of Life Cycle Assessment. - 1614-7502 .- 0948-3349. ; 9:5, s. 282-288
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Tidskriftsartikel (refereegranskat)abstract
- This article is the preamble to a set of articles describing initial results from an on-going European Commission funded, 5th Framework project called OMNIITOX, Operational Models aNd Information tools for Industrial applications of eco/TOXicological impact assessments. The different parts of this case study-driven project are briefly presented and put in relation to the aims of contributing to an operational life cycle-impact assessment (LCIA) model for impacts of toxicants. The present situation has been characterised by methodological difficulties, both regarding choice of the characterisation model(s) and limited input data on chemical properties, which often has resulted in the omission of toxicants from the LCIA, or at best focus on well characterised chemicals. The project addresses both problems and integrates models, as well as data, in an information system- the OMNIITOX IS. There is also a need for clarification of the relations between the (environmental) risk assessments of toxicants and LCIA, in addition to investigating the feasibility of introducing LCA into European chemicals legislation, tasks that also were addressed in the project. Keywords: Case studies; characterisation factor; chemicals; environmental risk assessment; hazard assessment; information system; life cycle impact assessment (LCIA); potentially toxic substances; regulation; risk assessment; risk ranking
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