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19 results on '"De Krijger, R R"'

1. Deep Learning Approaches Applied to Image Classification of Renal Tumors: A Systematic Review

Author

Pathologie Pathologen staf, Cancer, Pathologie Groep Van Diest, Amador, Sandra, Beuschlein, Felix, Chauhan, Vedant, Favier, Judith, Gil, David, Greenwood, Phillip, de Krijger, R. R., Kroiss, Matthias, Ortuño-Miquel, Samanta, Patocs, Attila, Stell, Anthony, Walch, Axel, Pathologie Pathologen staf, Cancer, Pathologie Groep Van Diest, Amador, Sandra, Beuschlein, Felix, Chauhan, Vedant, Favier, Judith, Gil, David, Greenwood, Phillip, de Krijger, R. R., Kroiss, Matthias, Ortuño-Miquel, Samanta, Patocs, Attila, Stell, Anthony, and Walch, Axel

Published
2024

2. Primary extrarenal rhabdoid tumour of the liver: a case report and literature review

Author

Pathologie Pathologen staf, Cancer, Pathologie Groep Van Diest, Meyers, Michel, Demetter, P., De Roo, A. K., Pezzullo, M., Jungels, C., Brichard, B., De Magnee, C., De Krijger, R. R., Verset, G., Pathologie Pathologen staf, Cancer, Pathologie Groep Van Diest, Meyers, Michel, Demetter, P., De Roo, A. K., Pezzullo, M., Jungels, C., Brichard, B., De Magnee, C., De Krijger, R. R., and Verset, G.

Published
2023

3. Treatment-related neuroendocrine prostatic carcinoma

Author

WHO Classification of Tumours Editorial Board, WHO Classification of Tumours Editorial Board, ( ), Rubin, M A, de Krijger, R R, Meon, S, Moch, Holger; https://orcid.org/0000-0002-7986-2839, WHO Classification of Tumours Editorial Board, WHO Classification of Tumours Editorial Board, ( ), Rubin, M A, de Krijger, R R, Meon, S, and Moch, Holger; https://orcid.org/0000-0002-7986-2839

Published
2022

4. Infantile fibrosarcoma with an EGFR kinase domain duplication:Underlining a close relationship with congenital mesoblastic nephroma and highlighting a similar morphological spectrum

Author

van Spronsen, R., Kester, L. A., Knops, R. R.G., van de Sande, M. A.J., van Leenders, G. J.L.H., de Laat, P. C.J., Stortelder, E., Korpershoek, E., van Noesel, M. M., Meister, M. T., Koerkamp, M. J.A.Groot, de Graaf, N., Giovannoni, I., Tops, B. B.J., de Krijger, R. R., ter Horst, S. A.J., Flucke, U., Alaggio, R., Hiemcke-Jiwa, L. S., van Spronsen, R., Kester, L. A., Knops, R. R.G., van de Sande, M. A.J., van Leenders, G. J.L.H., de Laat, P. C.J., Stortelder, E., Korpershoek, E., van Noesel, M. M., Meister, M. T., Koerkamp, M. J.A.Groot, de Graaf, N., Giovannoni, I., Tops, B. B.J., de Krijger, R. R., ter Horst, S. A.J., Flucke, U., Alaggio, R., and Hiemcke-Jiwa, L. S.

Abstract

Infantile fibrosarcoma (IFS) and congenital mesoblastic nephroma (CMN) are locally aggressive tumors primarily occurring in infants. Both IFS and the cellular subtype of CMN show overlapping morphological features and an ETV6-NTRK3 fusion, suggesting a close relationship. An activating alteration of EGFR, based on an EGFR kinase domain duplication (KDD), occurs in a subset of CMNs lacking an NTRK3 rearrangement, especially in the classic and mixed type. So far no EGFR-KDDs have been detected in IFS. We describe four pediatric tumors at the extremities (leg, n = 2; foot and arm n = 1) with histological features of IFS/CMN. Two cases showed classic IFS morphology while two were similar to classic/mixed type CMN. In all cases, an EGFR-KDD was identified without detection of a fusion gene. There were no abnormalities of the kidneys in any of the patients. This is the first description of IFS with an EGFR-KDD as driver mutation, supporting that IFS and CMN are similar lesions with the same morphological and genetic spectrum. Pathologists should be aware of the more fibrous variant of IFS, similar to classic/mixed type CMN. Molecular analyses are crucial to treat these lesions adequately, especially with regard to the administration of tyrosine kinase inhibitors.

Published
2022

5. Pleuropulmonary blastoma (PPB) and other DICER1-associated high-grade malignancies are morphologically, genetically and epigenetically related - A comparative study of 4 PPBs and 6 sarcomas

Author

Pathologie Groep Van Diest, Cancer, PMC Medisch specialisten, Onderzoek Beeld, Pathologie Pathologen staf, Hiemcke-Jiwa, L S, van Belle, S, Eijkelenboom, A, Merks, J H M, van Noesel, M M, Kaal, Suzanne E.J., Pijnenborg, J M A, Bulten, J, Tops, B B J, van de Ven, C P, van Gorp, J M, de Krijger, R R, Cheesman, E, Kelsey, A M, Kester, L A, Nijmegen, Radboud, Pathologie Groep Van Diest, Cancer, PMC Medisch specialisten, Onderzoek Beeld, Pathologie Pathologen staf, Hiemcke-Jiwa, L S, van Belle, S, Eijkelenboom, A, Merks, J H M, van Noesel, M M, Kaal, Suzanne E.J., Pijnenborg, J M A, Bulten, J, Tops, B B J, van de Ven, C P, van Gorp, J M, de Krijger, R R, Cheesman, E, Kelsey, A M, Kester, L A, and Nijmegen, Radboud

Published
2022

6. Infantile fibrosarcoma with an EGFR kinase domain duplication: Underlining a close relationship with congenital mesoblastic nephroma and highlighting a similar morphological spectrum

Author

Cancer, PMC Medisch specialisten, Onderzoek Beeld, Pathologie Pathologen staf, MS Radiologie, van Spronsen, R, Kester, L A, Knops, R R G, van de Sande, M A J, van Leenders, G J L H, de Laat, P C J, Stortelder, E, Korpershoek, E, van Noesel, M M, Meister, M T, Koerkamp, M J A Groot, de Graaf, N, Giovannoni, I, Tops, B B J, de Krijger, R R, Ter Horst, S A J, Flucke, U, Alaggio, R, Hiemcke-Jiwa, L S, Cancer, PMC Medisch specialisten, Onderzoek Beeld, Pathologie Pathologen staf, MS Radiologie, van Spronsen, R, Kester, L A, Knops, R R G, van de Sande, M A J, van Leenders, G J L H, de Laat, P C J, Stortelder, E, Korpershoek, E, van Noesel, M M, Meister, M T, Koerkamp, M J A Groot, de Graaf, N, Giovannoni, I, Tops, B B J, de Krijger, R R, Ter Horst, S A J, Flucke, U, Alaggio, R, and Hiemcke-Jiwa, L S

Published
2022

7. Neuroblastoma between 1990 and 2014 in the Netherlands : Increased incidence and improved survival of high-risk neuroblastoma

Author

Tas, M. L., Reedijk, A. M.J., Karim-Kos, H. E., Kremer, L. C.M., van de Ven, C. P., Dierselhuis, M. P., van Eijkelenburg, N. K.A., van Grotel, M., Kraal, K. C.J.M., Peek, A. M.L., Coebergh, J. W.W., Janssens, G. O.R., de Keizer, B., de Krijger, R. R., Pieters, R., Tygtat, G. A.M., van Noesel, M. M., Tas, M. L., Reedijk, A. M.J., Karim-Kos, H. E., Kremer, L. C.M., van de Ven, C. P., Dierselhuis, M. P., van Eijkelenburg, N. K.A., van Grotel, M., Kraal, K. C.J.M., Peek, A. M.L., Coebergh, J. W.W., Janssens, G. O.R., de Keizer, B., de Krijger, R. R., Pieters, R., Tygtat, G. A.M., and van Noesel, M. M.

Published
2020

8. Neuroblastoma between 1990 and 2014 in the Netherlands : Increased incidence and improved survival of high-risk neuroblastoma

Author

Tas, M. L., Reedijk, A. M.J., Karim-Kos, H. E., Kremer, L. C.M., van de Ven, C. P., Dierselhuis, M. P., van Eijkelenburg, N. K.A., van Grotel, M., Kraal, K. C.J.M., Peek, A. M.L., Coebergh, J. W.W., Janssens, G. O.R., de Keizer, B., de Krijger, R. R., Pieters, R., Tygtat, G. A.M., van Noesel, M. M., Tas, M. L., Reedijk, A. M.J., Karim-Kos, H. E., Kremer, L. C.M., van de Ven, C. P., Dierselhuis, M. P., van Eijkelenburg, N. K.A., van Grotel, M., Kraal, K. C.J.M., Peek, A. M.L., Coebergh, J. W.W., Janssens, G. O.R., de Keizer, B., de Krijger, R. R., Pieters, R., Tygtat, G. A.M., and van Noesel, M. M.

Published
2020

9. Neuroblastoma between 1990 and 2014 in the Netherlands : Increased incidence and improved survival of high-risk neuroblastoma

Author

Tas, M. L., Reedijk, A. M.J., Karim-Kos, H. E., Kremer, L. C.M., van de Ven, C. P., Dierselhuis, M. P., van Eijkelenburg, N. K.A., van Grotel, M., Kraal, K. C.J.M., Peek, A. M.L., Coebergh, J. W.W., Janssens, G. O.R., de Keizer, B., de Krijger, R. R., Pieters, R., Tygtat, G. A.M., van Noesel, M. M., Tas, M. L., Reedijk, A. M.J., Karim-Kos, H. E., Kremer, L. C.M., van de Ven, C. P., Dierselhuis, M. P., van Eijkelenburg, N. K.A., van Grotel, M., Kraal, K. C.J.M., Peek, A. M.L., Coebergh, J. W.W., Janssens, G. O.R., de Keizer, B., de Krijger, R. R., Pieters, R., Tygtat, G. A.M., and van Noesel, M. M.

Published
2020

10. Neuroblastoma between 1990 and 2014 in the Netherlands: Increased incidence and improved survival of high-risk neuroblastoma

Author

MS Radiotherapie, Cancer, MS Radiologie, Pathologie patiënten zorg, Pathologie Pathologen staf, Tas, M. L., Reedijk, A. M.J., Karim-Kos, H. E., Kremer, L. C.M., van de Ven, C. P., Dierselhuis, M. P., van Eijkelenburg, N. K.A., van Grotel, M., Kraal, K. C.J.M., Peek, A. M.L., Coebergh, J. W.W., Janssens, G. O.R., de Keizer, B., de Krijger, R. R., Pieters, R., Tygtat, G. A.M., van Noesel, M. M., MS Radiotherapie, Cancer, MS Radiologie, Pathologie patiënten zorg, Pathologie Pathologen staf, Tas, M. L., Reedijk, A. M.J., Karim-Kos, H. E., Kremer, L. C.M., van de Ven, C. P., Dierselhuis, M. P., van Eijkelenburg, N. K.A., van Grotel, M., Kraal, K. C.J.M., Peek, A. M.L., Coebergh, J. W.W., Janssens, G. O.R., de Keizer, B., de Krijger, R. R., Pieters, R., Tygtat, G. A.M., and van Noesel, M. M.

Published
2020

11. Renal cell carcinoma in young FH mutation carriers: case series and review of the literature

Author

Genetica Klinische Genetica, MS Neonatologie, MS Radiologie, Cancer, Pathologie Pathologen staf, Child Health, PMC Medisch specialisten, Speerpunt, Zorg en O&O, Hol, J A, Jongmans, M C J, Littooij, A S, de Krijger, R R, Kuiper, R P, van Harssel, J J T, Mensenkamp, A, Simons, M, Tytgat, G A M, van den Heuvel-Eibrink, M M, van Grotel, M, Genetica Klinische Genetica, MS Neonatologie, MS Radiologie, Cancer, Pathologie Pathologen staf, Child Health, PMC Medisch specialisten, Speerpunt, Zorg en O&O, Hol, J A, Jongmans, M C J, Littooij, A S, de Krijger, R R, Kuiper, R P, van Harssel, J J T, Mensenkamp, A, Simons, M, Tytgat, G A M, van den Heuvel-Eibrink, M M, and van Grotel, M

Published
2020

12. TNM Staging of Neoplasms of the Endocrine Pancreas: Results From a Large International Cohort Study

Author

Rindi, G., Falconi, M., Klersy, C., Albarello, L., Boninsegna, L., Buchler, M. W., Capella, C., Caplin, M., Couvelard, A., Doglioni, C., Delle Fave, G., Fischer, L., Fusai, G., de Herder, W. W., Jann, H., Komminoth, P., de Krijger, R. R., La Rosa, S., Luong, T. V., Pape, U., Perren, A., Ruszniewski, P., Scarpa, A., Schmitt, A., Solcia, E., Wiedenmann, B., Rindi, G., Falconi, M., Klersy, C., Albarello, L., Boninsegna, L., Buchler, M. W., Capella, C., Caplin, M., Couvelard, A., Doglioni, C., Delle Fave, G., Fischer, L., Fusai, G., de Herder, W. W., Jann, H., Komminoth, P., de Krijger, R. R., La Rosa, S., Luong, T. V., Pape, U., Perren, A., Ruszniewski, P., Scarpa, A., Schmitt, A., Solcia, E., and Wiedenmann, B.

Abstract

Background Both the European Neuroendocrine Tumor Society (ENETS) and the International Union for Cancer Control/American Joint Cancer Committee/World Health Organization (UICC/AJCC/WHO) have proposed TNM staging systems for pancreatic neuroendocrine neoplasms. This study aims to identify the most accurate and useful TNM system for pancreatic neuroendocrine neoplasms. Methods The study included 1072 patients who had undergone previous surgery for their cancer and for which at least 2 years of follow-up from 1990 to 2007 was available. Data on 28 variables were collected, and the performance of the two TNM staging systems was compared by Cox regression analysis and multivariable analyses. All statistical tests were two-sided. Results Differences in distribution of sex and age were observed for the ENETS TNM staging system. At Cox regression analysis, only the ENETS TNM staging system perfectly allocated patients into four statistically significantly different and equally populated risk groups (with stage I as the reference; stage II hazard ratio [HR] of death = 16.23, 95% confidence interval [CI] = 2.14 to 123, P = .007; stage III HR of death = 51.81, 95% CI = 7.11 to 377, P < .001; and stage IV HR of death = 160, 95% CI = 22.30 to 1143, P < .001). However, the UICC/AJCC/WHO 2010 TNM staging system compressed the disease into three differently populated classes, with most patients in stage I, and with the patients being equally distributed into stages II-III (statistically similar) and IV (with stage I as the reference; stage II HR of death = 9.57, 95% CI = 4.62 to 19.88, P < .001; stage III HR of death = 9.32, 95% CI = 3.69 to 23.53, P = .94; and stage IV HR of death = 30.84, 95% CI = 15.62 to 60.87, P < .001). Multivariable modeling indicated curative surgery, TNM staging, and grading were effective predictors of death, and grading was the second most effective independent predictor of survival in the absence of staging information. Though both TNM staging systems

Published
2017

13. Immunohistochemical expression of stem cell markers in pheochromocytomas/paragangliomas is associated with SDHx mutations

Author

Oudijk, L., Neuhofer, C. M., Lichtenauer, U. D., Papathomas, T. G., Korpershoek, E., Stoop, H., Oosterhuis, J. W., Smid, M., Restuccia, D. F., Robledo, M., De Cubas, A. A., Mannelli, M., Gimenez-Roqueplo, A. P., Dinjens, W. N M, Beuschlein, F., De Krijger, R. R., Oudijk, L., Neuhofer, C. M., Lichtenauer, U. D., Papathomas, T. G., Korpershoek, E., Stoop, H., Oosterhuis, J. W., Smid, M., Restuccia, D. F., Robledo, M., De Cubas, A. A., Mannelli, M., Gimenez-Roqueplo, A. P., Dinjens, W. N M, Beuschlein, F., and De Krijger, R. R.

Published
2015

14. 9B.09: Identification of markers predictive for malignant behaviour of Pheochromocytomas and paragangliomas

Author

UCL - (SLuc) Service d'anatomie pathologique, UCL - SSS/DDUV - Institut de Duve, UCL - SSS/IREC/NEFR - Pôle de Néphrologie, UCL - SSS/IREC/CARD - Pôle de recherche cardiovasculaire, UCL - (SLuc) Centre de génétique médicale UCL, UCL - (SLuc) Service de pathologie cardiovasculaire, Evenepoel, Lucie, Van Nederveen, F H, Oudijk, L, Papathomas, T G, Restuccia, D F, Belt, E J T, Franssen, G J H, Feelders, R A, Van Eeden, S, Timmers, H, De Herder, W W, Aydin, Selda, Vikkula, Miikka, De Krijger, R R, Dinjens, W N M, Persu, Alexandre, Korpershoek, E, 25th European Meeting on Hypertension and Cardiovascular Protection, UCL - (SLuc) Service d'anatomie pathologique, UCL - SSS/DDUV - Institut de Duve, UCL - SSS/IREC/NEFR - Pôle de Néphrologie, UCL - SSS/IREC/CARD - Pôle de recherche cardiovasculaire, UCL - (SLuc) Centre de génétique médicale UCL, UCL - (SLuc) Service de pathologie cardiovasculaire, Evenepoel, Lucie, Van Nederveen, F H, Oudijk, L, Papathomas, T G, Restuccia, D F, Belt, E J T, Franssen, G J H, Feelders, R A, Van Eeden, S, Timmers, H, De Herder, W W, Aydin, Selda, Vikkula, Miikka, De Krijger, R R, Dinjens, W N M, Persu, Alexandre, Korpershoek, E, and 25th European Meeting on Hypertension and Cardiovascular Protection

Abstract

OBJECTIVE: Pheochromocytomas and paragangliomas (PPGL) are relatively rare and mostly benign tumours. Approximately 10% of PPGL are malignant, as defined by the presence of metastases, i.e chromaffin tissue at a location that usually does not contain chromaffin cells. However, up to 35% of tumours in patients carrying an SDHB mutation appears to be malignant. Nowadays, no reliable marker allows to predict whether a PPGL is, or will become malignant. In addition, there are no curative treatments if metastases occur. The aim of the present study was to dentify genetic markers allowing to distinguish benign from malignant tumours. DESIGN AND METHOD: An mRNA expression array was performed on benign and malignant PPGL. The genes showing a different expression between the benign and malignant tumours were selected to be confirmed and validated by qRT-PCR. Finally, the remaining genes were stained by immunohistochemistry on Tissue MicroArray including a large series of PPGL. RESULTS: Forty benign and 12 malignant PPGL were investigated for differences in mRNA expression with Affymetrix arrays. Expression data were normalized according to Affymetrix recommendations. Then, using Pomelo II (http://pomelo2.bioinfo.cnio.es/), a Limma t-test was performed, to assess which genes were differentially expressed between benign and malignant PPGL. First, a non-clustered analysis was performed and 10 genes with a False Discovery Rate (FDR) below 0.05 and a relative overexpression ratio of at least 4 were found, including Interleukin 13 Receptor alpha 2 (IL13RA2) and Monooxygenase DBH-like 1 (MOXD1). Secondly, a supervised cluster analysis was performed (based on HIF target genes), resulting in 2 groups, which were both investigated for differences in mRNA expression between benign and malignant tumours. Five genes showed an FDR below 0.01 and were overexpressed in malignant tumours with a ratio higher than 4, including Contactin 4 (CNTN4), Iroquois Homeobox 3 (IRX3), and Sulfatase 2 (S

Published
2015

15. Immunohistochemical expression of stem cell markers in pheochromocytomas/paragangliomas is associated with SDHx mutations

Author

Oudijk, L., Neuhofer, C. M., Lichtenauer, U. D., Papathomas, T. G., Korpershoek, E., Stoop, H., Oosterhuis, J. W., Smid, M., Restuccia, D. F., Robledo, M., De Cubas, A. A., Mannelli, M., Gimenez-Roqueplo, A. P., Dinjens, W. N M, Beuschlein, F., De Krijger, R. R., Oudijk, L., Neuhofer, C. M., Lichtenauer, U. D., Papathomas, T. G., Korpershoek, E., Stoop, H., Oosterhuis, J. W., Smid, M., Restuccia, D. F., Robledo, M., De Cubas, A. A., Mannelli, M., Gimenez-Roqueplo, A. P., Dinjens, W. N M, Beuschlein, F., and De Krijger, R. R.

Published
2015

16. Immunohistochemical expression of stem cell markers in pheochromocytomas/paragangliomas is associated with SDHx mutations

Author

Oudijk, L., Neuhofer, C. M., Lichtenauer, U. D., Papathomas, T. G., Korpershoek, E., Stoop, H., Oosterhuis, J. W., Smid, M., Restuccia, D. F., Robledo, M., De Cubas, A. A., Mannelli, M., Gimenez-Roqueplo, A. P., Dinjens, W. N M, Beuschlein, F., De Krijger, R. R., Oudijk, L., Neuhofer, C. M., Lichtenauer, U. D., Papathomas, T. G., Korpershoek, E., Stoop, H., Oosterhuis, J. W., Smid, M., Restuccia, D. F., Robledo, M., De Cubas, A. A., Mannelli, M., Gimenez-Roqueplo, A. P., Dinjens, W. N M, Beuschlein, F., and De Krijger, R. R.

Published
2015

17. Immunohistochemical expression of stem cell markers in pheochromocytomas/paragangliomas is associated with SDHx mutations

Author

Pathologie patiënten zorg, Oudijk, L., Neuhofer, C. M., Lichtenauer, U. D., Papathomas, T. G., Korpershoek, E., Stoop, H., Oosterhuis, J. W., Smid, M., Restuccia, D. F., Robledo, M., De Cubas, A. A., Mannelli, M., Gimenez-Roqueplo, A. P., Dinjens, W. N M, Beuschlein, F., De Krijger, R. R., Pathologie patiënten zorg, Oudijk, L., Neuhofer, C. M., Lichtenauer, U. D., Papathomas, T. G., Korpershoek, E., Stoop, H., Oosterhuis, J. W., Smid, M., Restuccia, D. F., Robledo, M., De Cubas, A. A., Mannelli, M., Gimenez-Roqueplo, A. P., Dinjens, W. N M, Beuschlein, F., and De Krijger, R. R.

Published
2015

18. DNA copy number status is a powerful predictor of poor survival in endocrine pancreatic tumor patients

Author

Jonkers, Y. M. H., Claessen, S. M. H., Perren, A., Schmitt, A. M., Hofland, L. J., de Herder, W., de Krijger, R. R., Verhofstad, A. A. J., Hermus, A .R., Kummer, J .A., Skogseid, Britt M., Volante, M., Voogd, A. C., Ramaekers, F. C. S., Speel, E. J. M., Jonkers, Y. M. H., Claessen, S. M. H., Perren, A., Schmitt, A. M., Hofland, L. J., de Herder, W., de Krijger, R. R., Verhofstad, A. A. J., Hermus, A .R., Kummer, J .A., Skogseid, Britt M., Volante, M., Voogd, A. C., Ramaekers, F. C. S., and Speel, E. J. M.

Abstract

The clinical behavior of endocrine pancreatic tumors (EPTs) is difficult to predict in the absence of metastases or invasion to adjacent organs. Several markers have been indicated as potential predictors of metastatic disease, such as tumor size 2 cm, Ki67 proliferative index 2%, cytokeratin (CK) 19 status, and recently in insulinomas, chromosomal instability (CIN). The goal of this study was to evaluate the value of these markers, and in particular of the CIN, to predict tumor recurrence or progression and tumor-specific death, using a series of 47 insulinomas and 24 non-insulinoma EPTs. From these EPT cases, a genomic profile has been generated and follow-up data have been obtained. The proliferative index has been determined in 68 tumors and a CK19 expression pattern in 50 tumors. Results are statistically analyzed using Kaplan–Meier plots and the log-rank statistic. General CIN, as well as specific chromosomal alterations such as 3p and 6q loss and 12q gain, turned out to be the most powerful indicators for poor tumor-free survival (P0.0004) and tumor-specific death (P0.0113) in insulinomas. The CIN, chromosome 7q gain, and a proliferative index 2% were reliable in predicting a poor tumor-free survival in non-insulinoma EPTs (P0.0181, whereas CK19 expression was the most optimal predictor of tumor-specific death in these tumors. In conclusion, DNA copy number status is the most sensitive and efficient marker of adverse clinical outcome in insulinomas and of potential interest in non-insulinoma EPTs. As a consequence, this marker should be considered as a prognosticator to improve clinical d

Published
2007
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19. TNM Staging of Neoplasms of the Endocrine Pancreas: Results From a Large International Cohort Study

Author

Rindi, G., Falconi, M., Klersy, C., Albarello, L., Boninsegna, L., Buchler, M. W., Capella, C., Caplin, M., Couvelard, A., Doglioni, C., Delle Fave, G., Fischer, L., Fusai, G., de Herder, W. W., Jann, H., Komminoth, P., de Krijger, R. R., La Rosa, S., Luong, T. V., Pape, U., Perren, A., Ruszniewski, P., Scarpa, A., Schmitt, A., Solcia, E., Wiedenmann, B., Rindi, G., Falconi, M., Klersy, C., Albarello, L., Boninsegna, L., Buchler, M. W., Capella, C., Caplin, M., Couvelard, A., Doglioni, C., Delle Fave, G., Fischer, L., Fusai, G., de Herder, W. W., Jann, H., Komminoth, P., de Krijger, R. R., La Rosa, S., Luong, T. V., Pape, U., Perren, A., Ruszniewski, P., Scarpa, A., Schmitt, A., Solcia, E., and Wiedenmann, B.

Abstract

Background Both the European Neuroendocrine Tumor Society (ENETS) and the International Union for Cancer Control/American Joint Cancer Committee/World Health Organization (UICC/AJCC/WHO) have proposed TNM staging systems for pancreatic neuroendocrine neoplasms. This study aims to identify the most accurate and useful TNM system for pancreatic neuroendocrine neoplasms. Methods The study included 1072 patients who had undergone previous surgery for their cancer and for which at least 2 years of follow-up from 1990 to 2007 was available. Data on 28 variables were collected, and the performance of the two TNM staging systems was compared by Cox regression analysis and multivariable analyses. All statistical tests were two-sided. Results Differences in distribution of sex and age were observed for the ENETS TNM staging system. At Cox regression analysis, only the ENETS TNM staging system perfectly allocated patients into four statistically significantly different and equally populated risk groups (with stage I as the reference; stage II hazard ratio [HR] of death = 16.23, 95% confidence interval [CI] = 2.14 to 123, P = .007; stage III HR of death = 51.81, 95% CI = 7.11 to 377, P < .001; and stage IV HR of death = 160, 95% CI = 22.30 to 1143, P < .001). However, the UICC/AJCC/WHO 2010 TNM staging system compressed the disease into three differently populated classes, with most patients in stage I, and with the patients being equally distributed into stages II-III (statistically similar) and IV (with stage I as the reference; stage II HR of death = 9.57, 95% CI = 4.62 to 19.88, P < .001; stage III HR of death = 9.32, 95% CI = 3.69 to 23.53, P = .94; and stage IV HR of death = 30.84, 95% CI = 15.62 to 60.87, P < .001). Multivariable modeling indicated curative surgery, TNM staging, and grading were effective predictors of death, and grading was the second most effective independent predictor of survival in the absence of staging information. Though both TNM staging systems

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