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

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

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-Am...

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Veröffentlicht in:Endocrine-related cancer 2018-09, Vol.25 (9), p.783-793
Hauptverfasser: Krauss, Tobias, Ferrara, Alfonso Massimiliano, Links, Thera P, Wellner, Ulrich, Bancos, Irina, Kvachenyuk, Andrey, Villar Gómez de las Heras, Karina, Yukina, Marina Y, Petrov, Roman, Bullivant, Garrett, von Duecker, Laura, Jadhav, Swati, Ploeckinger, Ursula, Welin, Staffan, Schalin-Jäntti, Camilla, Gimm, Oliver, Pfeifer, Marija, Ngeow, Joanne, Hasse-Lazar, Kornelia, Sansó, Gabriela, Qi, Xiaoping, Ugurlu, M Umit, Diaz, Rene E, Wohllk, Nelson, Peczkowska, Mariola, Aberle, Jens, Lourenço, Delmar M, Pereira, Maria A A, Fragoso, Maria C B V, Hoff, Ana O, Almeida, Madson Q, Violante, Alice H D, Quidute, Ana R P, Zhang, Zhewei, Recasens, Mònica, Díaz, Luis Robles, Kunavisarut, Tada, Wannachalee, Taweesak, Sirinvaravong, Sirinart, Jonasch, Eric, Grozinsky-Glasberg, Simona, Fraenkel, Merav, Beltsevich, Dmitry, Egorov, Viacheslav I, Bausch, Dirk, Schott, Matthias, Tiling, Nikolaus, Pennelli, Gianmaria, Zschiedrich, Stefan, Därr, Roland, Ruf, Juri, Denecke, Timm, Link, Karl-Heinrich, Zovato, Stefania, von Dobschuetz, Ernst, Yaremchuk, Svetlana, Amthauer, Holger, Makay, Özer, Patocs, Attila, Walz, Martin K, Huber, Tobias B, Seufert, Jochen, Hellman, Per, Kim, Raymond H, Kuchinskaya, Ekaterina, Schiavi, Francesca, Malinoc, Angelica, Reisch, Nicole, Jarzab, Barbara, Barontini, Marta, Januszewicz, Andrzej, Shah, Nalini, Young, William F, Opocher, Giuseppe, Eng, Charis, Neumann, Hartmut P H, Bausch, Birke
Format: Artikel
Sprache:eng
Schlagworte:
Codons
Disease management
management recommendations
Metastases
Metastasis
Morbidity
Mutation
Neuroendocrine tumors
Pancreas
PanNET
Preventive medicine
Survival
Tumors
VHL protein
von Hippel-Lindau disease
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Zusammenfassung: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 1.5 cm in diameter were operated. Ten-year survival was significantly longer in operated vs non-operated patients, in particular for PanNETs
ISSN:1351-0088
1479-6821
1479-6821
DOI:10.1530/ERC-18-0100