Preventive medicine of von Hippel-Lindau disease-associated pancreatic neuroendocrine tumors
| Autor: | Stefan Zschiedrich, Nicole Reisch, Ursula Ploeckinger, Joanne Ngeow, Raymond H. Kim, William F. Young, Dmitry Beltsevich, Francesca Schiavi, Umit Ugurlu, Madson Q. Almeida, Taweesak Wannachalee, Gabriela Sanso, Mònica Recasens, Angelica Malinoc, Roman Petrov, Luis Robles Diaz, Andrzej Januszewicz, Jochen Seufert, Holger Amthauer, Svetlana Yaremchuk, Karl-Heinrich Link, Ulrich F. Wellner, Timm Denecke, Jens Aberle, Nalini S. Shah, Xiao-Ping Qi, Marina Y. Yukina, Zheiwei Zhang, Ernst von Dobschuetz, Marta Barontini, Maria Candida Barisson Villares Fragoso, Andrey Kvachenyuk, Laura von Duecker, Giuseppe Opocher, Swati S Jadhav, Roland Därr, Birke Bausch, Merav Fraenkel, Viacheslav I. Egorov, Staffan Welin, Özer Makay, Sirinart Sirinvaravong, Rene Eduardo Diaz, Garrett Bullivant, Matthias Schott, Ana Rosa Pinto Quidute, Ekaterina Kuchinskaya, Camilla Schalin-Jäntti, Charis Eng, Martin K. Walz, Ana O. Hoff, Barbara Jarzab, Tobias B. Huber, Thera P. Links, Nikolaus Tiling, Kornelia Hasse-Lazar, Eric Jonasch, Gianmaria Pennelli, Per Hellman, Maria Adelaide Albergaria Pereira, Nelson Wohllk, Tada Kunavisarut, Attila Patócs, Dirk Bausch, Juri Ruf, Hartmut P. H. Neumann, Alice Helena Dutra Violante, Simona Grozinsky-Glasberg, Stefania Zovato, Oliver Gimm, Alfonso Massimiliano Ferrara, Delmar Munir Lourenço, Mariola Pęczkowska, Marija Pfeifer, Irina Bancos, Tobias Krauss, Karina Villar Gómez de las Heras |
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| Přispěvatelé: | Damage and Repair in Cancer Development and Cancer Treatment (DARE), Guided Treatment in Optimal Selected Cancer Patients (GUTS) |
| Jazyk: | angličtina |
| Rok vydání: | 2018 |
| Předmět: |
Oncology
INVOLVEMENT Cancer Research Endocrinology Diabetes and Metabolism Medizin Disease Neuroendocrine tumors PanNET 0302 clinical medicine Endocrinology management recommendations CRITERIA Registries Child Islet cell tumors Middle Aged GA-68-DOTATOC 3. Good health Tumor Burden Neuroendocrine Tumors 030220 oncology & carcinogenesis survival von Hippel–Lindau disease GROWTH Adult medicine.medical_specialty Adolescent PET/CT 030209 endocrinology & metabolism 03 medical and health sciences Young Adult Internal medicine SURVEILLANCE medicine Humans Von Hippel–Lindau disease Preventive healthcare Aged PET-CT business.industry JAPANESE PATIENTS CLINICAL-FEATURES von Hippel-Lindau disease medicine.disease Pancreatic Neoplasms ISLET-CELL TUMORS Mutation business |
| Zdroj: | Endocrine-Related cancer, 25(9), 783-793. BIOSCIENTIFICA LTD |
| ISSN: | 1351-0088 |
| Popis: | 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 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 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. |
| Databáze: | OpenAIRE |
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