Search

Your search keyword '"Pieterman, Carolina R C"' showing total 129 results
Start Over Author "Pieterman, Carolina R C"
129 results on '"Pieterman, Carolina R C"'

1. Beyond the three P's: adrenal involvement in MEN.

Author

Clemente-Gutierrez, Uriel, Pieterman, Carolina R. C., Lui, Michael S., Yamashita, Thomas Szabo, Tame-Elorduy, Andrés, Huang, Bernice L., Shirali, Aditya S., Erstad, Derek J., Lee, Jeffrey E., Fisher, Sarah B., Graham, Paul H., Grubbs, Elizabeth G., Waguespack, Steven G., Ng, Chaan S., and Perrier, Nancy

Subjects
*WATCHFUL waiting, *ADRENAL tumors, *DISEASE progression, *HYPERALDOSTERONISM, *CUSHING'S syndrome, *PHEOCHROMOCYTOMA
Abstract

Adrenal lesions (ALs) are often detected in patients with multiple endocrine neoplasia type 1 (MEN1). However, they are not well described in MEN1, making their clinical management unclear. This study examined the prevalence and outcomes of ALs found in MEN1. We performed a retrospective chart review of patients diagnosed with MEN1 from 1990 to 2021. ALs were diagnosed using abdominal or thoracic imaging and classified as being unilateral or bilateral, having single or multiple nodules, and as having diffuse enlargement or not. Measurable nodular lesions were analyzed for their size and growth over time. Patients' clinical and radiographic characteristics were collected. We identified 382 patients with MEN1, 89 (23.3%) of whom had ALs. The mean age at detection was 47 ± 11.9 years. We documented 101 measurable nodular lesions (mean size, 17.5 mm; range, 3-123 mm). Twenty-seven nodules (26.7%) were smaller than 1 cm. Watchful waiting was indicated in 79 (78.2%) patients, of whom 28 (35.4%) had growing lesions. Functional lesions were diagnosed in 6 (15.8%) of 38 that had functional work-up (diagnoses: pheochromocytoma (n = 2), adrenocorticotropic hormone-dependent hypercortisolism (n = 2), hyperandrogenism (n = 1), hyperaldosteronism (n = 1)); surgery was indicated for 5 (83.3%; n = 12 nodules), 2 of whom had bilateral, diffuse adrenal enlargement. Two patients were diagnosed with adrenocortical carcinoma and two with neoplasms of uncertain malignant potential. Radiographic or clinical progression of ALs is uncommon. Malignancy should be suspected on the basis of a lesion's growth rate and size. A baseline hormonal work-up is recommended, and no further biochemical work-up is suggested when the initial assessment shows nonfunctioning lesions. [ABSTRACT FROM AUTHOR]

Published
2024
Full Text
View/download PDF

2. Blood-based Proteomic Signatures Associated With MENI-related Duodenopancreatic Neuroendocrine Tumor Progression.

Author

Fahrmann, Johannes F., Wasylishen, Amanda R., Pieterman, Carolina R. C., Irajizad, Ehsan, Vykoukal, Jody, Wu, Ranran, Dennison, Jennifer B., Peterson, Christine B., Hua Zhao, Kim-Anh Do, Halperin, Daniel M., Agarwal, Sunita K., Blau, Jenny E., Jha, Smita, Del Rivero, Jaydira, Nilubol, Naris, Walter, Mary F., Welch, James M., Weinstein, Lee S., and Vriens, Menno R.

Subjects
NEUROENDOCRINE tumors, MASS spectrometry, DISEASE progression
Abstract

Purpose: Patients with multiple endocrine neoplasia type 1 (MEN1) are predisposed to develop duodenopancreatic neuroendocrine tumors (dpNETs), and metastatic dpNET is the primary cause of disease-related mortality. Presently, there is a paucity of prognostic factors that can reliably identify patients with MEN1-related dpNETS who are at high risk of distant metastasis. In the current study, we aimed to establish novel circulating molecular protein signatures associated with disease progression. Experimental Design: Mass spectrometry-based proteomic profiling was conducted on plasmas procured through an international collaboration between MD Anderson Cancer Center, the National Institutes of Health, and the University Medical Center Utrecht from a cohort of 56 patients with MEN1 [14 with distant metastasis dpNETs (cases) and 42 with either indolent dpNETs or no dpNETs (controls)]. Findings were compared to proteomic profiles generated from serially collected plasmas from a mouse model of Men1-pancreatic neuroendocrine tumors (Men1fl/flPdx1-CreTg) and control mice (Men1fl/fl). Results: A total of 187 proteins were found to be elevated in MEN1 patients with distant metastasis compared to controls, including 9 proteins previously associated with pancreatic cancer and other neuronal proteins. Analyses of mouse plasmas revealed 196 proteins enriched for transcriptional targets of oncogenic MYCN, YAP1, POU5F1, and SMAD that were associated with disease progression in Men1fl/flPdx1-CreTg mice. Cross-species intersection revealed 19 proteins positively associated with disease progression in both human patients and in Men1fl/flPdx1-CreTg mice. Conclusions: Our integrated analyses identified novel circulating protein markers associated with disease progression in MEN1-related dpNET. [ABSTRACT FROM AUTHOR]

Published
2023
Full Text
View/download PDF

3. Blood-based proteomic signatures associated with MEN1-related Duodenopancreatic Neuroendocrine Tumor Progression

Author

Cancer, MS Endocriene Oncologie, Heelkunde Opleiding, MS CGO, Fahrmann, Johannes F, Wasylishen, Amanda R, Pieterman, Carolina R C, Irajizad, Ehsan, Vykoukal, Jody, Wu, Ranran, Dennison, Jennifer B, Peterson, Christine B, Zhao, Hua, Do, Kim-Anh, Halperin, Daniel M, Agarwal, Sunita K, Blau, Jenny E, Jha, Smita, Rivero, Jaydira Del, Nilubol, Naris, Walter, Mary F, Welch, James M, Weinstein, Lee S, Vriens, Menno R, van Leeuwaarde, Rachel S, van Treijen, Mark J C, Valk, Gerlof D, Perrier, Nancy D, Hanash, Samir M, Katayama, Hiroyuki, Cancer, MS Endocriene Oncologie, Heelkunde Opleiding, MS CGO, Fahrmann, Johannes F, Wasylishen, Amanda R, Pieterman, Carolina R C, Irajizad, Ehsan, Vykoukal, Jody, Wu, Ranran, Dennison, Jennifer B, Peterson, Christine B, Zhao, Hua, Do, Kim-Anh, Halperin, Daniel M, Agarwal, Sunita K, Blau, Jenny E, Jha, Smita, Rivero, Jaydira Del, Nilubol, Naris, Walter, Mary F, Welch, James M, Weinstein, Lee S, Vriens, Menno R, van Leeuwaarde, Rachel S, van Treijen, Mark J C, Valk, Gerlof D, Perrier, Nancy D, Hanash, Samir M, and Katayama, Hiroyuki

Published
2023

4. Status of Surveillance and Nonsurgical Therapy for Small Nonfunctioning Pancreatic Neuroendocrine Tumors

Author

Heelkunde Opleiding, Cancer, Pathologie Groep Brosens, Pathologie Pathologen staf, MS Endocriene Oncologie, MS CGO, van Beek, Dirk-Jan, Verschuur, Anna Vera D, Brosens, Lodewijk A A, Valk, Gerlof D, Pieterman, Carolina R C, Vriens, Menno R, Heelkunde Opleiding, Cancer, Pathologie Groep Brosens, Pathologie Pathologen staf, MS Endocriene Oncologie, MS CGO, van Beek, Dirk-Jan, Verschuur, Anna Vera D, Brosens, Lodewijk A A, Valk, Gerlof D, Pieterman, Carolina R C, and Vriens, Menno R

Published
2023

5. Diagnosing pancreatic neuroendocrine tumors in patients with multiple endocrine neoplasia type 1 in daily practice

Author

van Beek, Dirk-Jan, primary, Pieterman, Carolina R. C., additional, Wessels, Frank J., additional, van de Ven, Annenienke C., additional, de Herder, Wouter W., additional, Dekkers, Olaf M., additional, Zandee, Wouter T., additional, Drent, Madeleine L., additional, Bisschop, Peter H., additional, Havekes, Bas, additional, Borel Rinkes, Inne H. M., additional, Vriens, Menno R., additional, and Valk, Gerlof D., additional

Published
2022
Full Text
View/download PDF

6. Diagnosing pancreatic neuroendocrine tumors in patients with multiple endocrine neoplasia type 1 in daily practice

Author

Heelkunde Opleiding, Cancer, MS CGO, MS Endocriene Oncologie, MS Radiologie, Regenerative Medicine and Stem Cells, van Beek, Dirk-Jan, Pieterman, Carolina R C, Wessels, Frank J, van de Ven, Annenienke C, de Herder, Wouter W, Dekkers, Olaf M, Zandee, Wouter T, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Borel Rinkes, Inne H M, Vriens, Menno R, Valk, Gerlof D, Heelkunde Opleiding, Cancer, MS CGO, MS Endocriene Oncologie, MS Radiologie, Regenerative Medicine and Stem Cells, van Beek, Dirk-Jan, Pieterman, Carolina R C, Wessels, Frank J, van de Ven, Annenienke C, de Herder, Wouter W, Dekkers, Olaf M, Zandee, Wouter T, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Borel Rinkes, Inne H M, Vriens, Menno R, and Valk, Gerlof D

Published
2022

10. Initiating Pancreatic Neuroendocrine Tumor (pNET) Screening in Young MEN1 Patients: Results From the DutchMEN Study Group

Author

Klein Haneveld, Mirthe J, primary, van Treijen, Mark J C, additional, Pieterman, Carolina R C, additional, Dekkers, Olaf M, additional, van de Ven, Annenienke, additional, de Herder, Wouter W, additional, Zandee, Wouter T, additional, Drent, Madeleine L, additional, Bisschop, Peter H, additional, Havekes, Bas, additional, Vriens, Menno R, additional, Verrijn Stuart, Annemarie A, additional, Valk, Gerlof D, additional, and van Leeuwaarde, Rachel S, additional

Published
2021
Full Text
View/download PDF

13. A Blood-based Polyamine Signature Associated With MEN1 Duodenopancreatic Neuroendocrine Tumor Progression

Author

Fahrmann, Johannes F, primary, Wasylishen, Amanda R, additional, Pieterman, Carolina R C, additional, Irajizad, Ehsan, additional, Vykoukal, Jody, additional, Murage, Eunice, additional, Wu, Ranran, additional, Dennison, Jennifer B, additional, Krishna, Hansini, additional, Peterson, Christine B, additional, Lozano, Guillermina, additional, Zhao, Hua, additional, Do, Kim-Anh, additional, Halperin, Daniel M, additional, Agarwal, Sunita K, additional, Blau, Jenny E, additional, Del Rivero, Jaydira, additional, Nilubol, Naris, additional, Walter, Mary F, additional, Welch, James M, additional, Weinstein, Lee S, additional, Vriens, Menno R, additional, van Leeuwaarde, Rachel S, additional, van Treijen, Mark J C, additional, Valk, Gerlof D, additional, Perrier, Nancy D, additional, and Hanash, Samir M, additional

Published
2021
Full Text
View/download PDF

14. It's not a mystery, it's in the history: Multidisciplinary management of multiple endocrine neoplasia type 1

Author

Shirali, Aditya S., primary, Pieterman, Carolina R. C., additional, Lewis, Mark A., additional, Hyde, Samuel M., additional, Makawita, Shalini, additional, Dasari, Arvind, additional, Thosani, Nirav, additional, Ikoma, Naruhiko, additional, McCutcheon, Ian E., additional, Waguespack, Steven G., additional, and Perrier, Nancy D., additional

Published
2021
Full Text
View/download PDF

15. A blood-based polyamine signature associated with MEN1 duodenopancreatic neuroendocrine tumor progression

Author

Heelkunde Opleiding, MS CGO, Cancer, MS Endocriene Oncologie, Fahrmann, Johannes F, Wasylishen, Amanda R, Pieterman, Carolina R C, Irajizad, Ehsan, Vykoukal, Jody, Murage, Eunice, Wu, Ranran, Dennison, Jennifer B, Krishna, Hansini, Peterson, Christine B, Lozano, Guillermina, Zhao, Hua, Do, Kim-Anh, Halperin, Daniel M, Agarwal, Sunita K, Blau, Jenny E, Del Rivero, Jaydira, Nilubol, Naris, Walter, Mary F, Welch, James M, Weinstein, Lee S, Vriens, Menno R, van Leeuwaarde, Rachel S, van Treijen, Mark J C, Valk, Gerlof D, Perrier, Nancy D, Hanash, Samir M, Heelkunde Opleiding, MS CGO, Cancer, MS Endocriene Oncologie, Fahrmann, Johannes F, Wasylishen, Amanda R, Pieterman, Carolina R C, Irajizad, Ehsan, Vykoukal, Jody, Murage, Eunice, Wu, Ranran, Dennison, Jennifer B, Krishna, Hansini, Peterson, Christine B, Lozano, Guillermina, Zhao, Hua, Do, Kim-Anh, Halperin, Daniel M, Agarwal, Sunita K, Blau, Jenny E, Del Rivero, Jaydira, Nilubol, Naris, Walter, Mary F, Welch, James M, Weinstein, Lee S, Vriens, Menno R, van Leeuwaarde, Rachel S, van Treijen, Mark J C, Valk, Gerlof D, Perrier, Nancy D, and Hanash, Samir M

Published
2021

16. Initiating Pancreatic Neuroendocrine Tumor (pNET) Screening in Young MEN1 Patients: results from the DutchMEN Study Group

Author

MS Endocriene Oncologie, Heelkunde Opleiding, MS CGO, Cancer, Cluster C, Endocrinologie, Child Health, Klein Haneveld, Mirthe J, van Treijen, Mark J C, Pieterman, Carolina R C, Dekkers, Olaf M, van de Ven, Annenienke, de Herder, Wouter W, Zandee, Wouter T, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Vriens, Menno R, Verrijn Stuart, Annemarie A, Valk, Gerlof D, van Leeuwaarde, Rachel S, MS Endocriene Oncologie, Heelkunde Opleiding, MS CGO, Cancer, Cluster C, Endocrinologie, Child Health, Klein Haneveld, Mirthe J, van Treijen, Mark J C, Pieterman, Carolina R C, Dekkers, Olaf M, van de Ven, Annenienke, de Herder, Wouter W, Zandee, Wouter T, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Vriens, Menno R, Verrijn Stuart, Annemarie A, Valk, Gerlof D, and van Leeuwaarde, Rachel S

Published
2021

17. Health-related quality of life in patients with Multiple Endocrine Neoplasia type 1

Author

MS Endocriene Oncologie, Cancer, Epidemiology & Health Economics, JC onderzoeksprogramma Kanker, Heelkunde Opleiding, MS CGO, van Leeuwaarde, Rachel S, Pieterman, Carolina R C, May, Anne M, Dekkers, Olaf M, van der Horst-Schrivers, Anouk N A, Hermus, Ad R, de Herder, Wouter, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Vriens, Menno R, Valk, Gerlof D, MS Endocriene Oncologie, Cancer, Epidemiology & Health Economics, JC onderzoeksprogramma Kanker, Heelkunde Opleiding, MS CGO, van Leeuwaarde, Rachel S, Pieterman, Carolina R C, May, Anne M, Dekkers, Olaf M, van der Horst-Schrivers, Anouk N A, Hermus, Ad R, de Herder, Wouter, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Vriens, Menno R, and Valk, Gerlof D

Published
2021

20. Clues For Genetic Anticipation In Multiple Endocrine Neoplasia Type 1

Author

van den Broek, Medard F M, primary, van Nesselrooij, Bernadette P M, additional, Pieterman, Carolina R C, additional, Verrijn Stuart, Annemarie A, additional, van de Ven, Annenienke C, additional, de Herder, Wouter W, additional, Dekkers, Olaf M, additional, Drent, Madeleine L, additional, Havekes, Bas, additional, Kerstens, Michiel N, additional, Bisschop, Peter H, additional, and Valk, Gerlof D, additional

Published
2020
Full Text
View/download PDF

21. Initiating Pancreatic Neuroendocrine Tumor (pNET) Screening in Young MEN1 Patients: Results From the DutchMEN Study Group.

Author

Haneveld, Mirthe J. Klein, van Treijen, Mark J. C., Pieterman, Carolina R. C., Dekkers, Olaf M., van de Ven, Annenienke, de Herder, Wouter W., Zandee, Wouter T., Drent, Madeleine L., Bisschop, Peter H., Havekes, Bas, Vriens, Menno R., Stuart, Annemarie A. Verrijn, Valk, Gerlof D., van Leeuwaarde, Rachel S., Klein Haneveld, Mirthe J, and Verrijn Stuart, Annemarie A

Subjects
NEUROENDOCRINE tumors, MORTALITY, AGE groups, PANCREATIC tumors, DATABASES, ANTHROPOMETRY, EARLY detection of cancer, RETROSPECTIVE studies, PROGNOSIS, DIAGNOSTIC imaging, WERMER syndrome, AGE factors in disease, LONGITUDINAL method
Abstract

Context: Nonfunctioning pancreatic neuroendocrine tumors (NF-pNETs) are highly prevalent and constitute an important cause of mortality in patients with multiple endocrine neoplasia type 1 (MEN1). Still, the optimal age to initiate screening for pNETs is under debate.Objective: The aim of this work is to assess the age of occurrence of clinically relevant NF-pNETs in young MEN1 patients.Methods: Pancreatic imaging data of MEN1 patients were retrieved from the DutchMEN Study Group database. Interval-censored survival methods were used to describe age-related penetrance, compare survival curves, and develop a parametric model for estimating the risk of having clinically relevant NF-pNET at various ages. The primary objective was to assess age at occurrence of clinically relevant NF-pNET (size ≥ 20 mm or rapid growth); secondary objectives were the age at occurrence of NF-pNET of any size and pNET-associated metastasized disease.Results: Five of 350 patients developed clinically relevant NF-pNETs before age 18 years, 2 of whom subsequently developed lymph node metastases. No differences in clinically relevant NF-pNET-free survival were found for sex, time frame, and type of MEN1 diagnosis or genotype. The estimated ages (median, 95% CI) at a 1%, 2.5%, and 5% risk of having developed a clinically relevant tumor are 9.5 (6.5-12.7), 13.5 (10.2-16.9), and 17.8 years (14.3-21.4), respectively.Conclusion: Analyses from this population-based cohort indicate that start of surveillance for NF-pNETs with pancreatic imaging at age 13 to 14 years is justified. The psychological and medical burden of screening at a young age should be considered. [ABSTRACT FROM AUTHOR]

Published
2021
Full Text
View/download PDF

22. Prognostic factors and survival in MEN1 patients with gastrinomas : Results from the DutchMEN study group (DMSG)

Author

van Beek, Dirk-Jan, Nell, Sjoerd, Pieterman, Carolina R C, de Herder, Wouter W, van de Ven, Annenienke C, Dekkers, Olaf M, van der Horst-Schrivers, Anouk N, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Borel Rinkes, Inne H M, Vriens, Menno R, Valk, Gerlof D, van Beek, Dirk-Jan, Nell, Sjoerd, Pieterman, Carolina R C, de Herder, Wouter W, van de Ven, Annenienke C, Dekkers, Olaf M, van der Horst-Schrivers, Anouk N, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Borel Rinkes, Inne H M, Vriens, Menno R, and Valk, Gerlof D

Published
2019

23. Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG)

Author

MS CGO, Other research (not in main researchprogram), MS Endocriene Oncologie, Regenerative Medicine and Stem Cells, Cancer, Heelkunde Opleiding, van Beek, Dirk-Jan, Nell, Sjoerd, Pieterman, Carolina R C, de Herder, Wouter W, van de Ven, Annenienke C, Dekkers, Olaf M, van der Horst-Schrivers, Anouk N, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Borel Rinkes, Inne H M, Vriens, Menno R, Valk, Gerlof D, MS CGO, Other research (not in main researchprogram), MS Endocriene Oncologie, Regenerative Medicine and Stem Cells, Cancer, Heelkunde Opleiding, van Beek, Dirk-Jan, Nell, Sjoerd, Pieterman, Carolina R C, de Herder, Wouter W, van de Ven, Annenienke C, Dekkers, Olaf M, van der Horst-Schrivers, Anouk N, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Borel Rinkes, Inne H M, Vriens, Menno R, and Valk, Gerlof D

Published
2019

24. ‘Quality in, quality out’, a stepwise approach to evidence-based medicine for rare diseases promoted by multiple endocrine neoplasia type 1

Author

van Beek, Dirk-Jan, primary, van Leeuwaarde, Rachel S, additional, Pieterman, Carolina R C, additional, Vriens, Menno R, additional, Valk, Gerlof D, additional, _, _, additional, P H, Bisschop, additional, I H M, Borel Rinkes, additional, O M, Dekkers, additional, M L, Drent, additional, B, Havekes, additional, W W, de Herder, additional, A R M M, Hermus, additional, A N A, van der Horst-Schrivers, additional, J, de Jong, additional, H F A, Vasen, additional, and B A, Zonnenberg, additional

Published
2018
Full Text
View/download PDF

25. DNA methylation profiling in MEN1-related pancreatic neuroendocrine tumors reveals a potential epigenetic target for treatment

Author

Conemans, Elfi B, Lodewijk, Lutske, Moelans, Cathy M, Offerhaus, G Johan A, Pieterman, Carolina R C, Morsink, Folkert H, Dekkers, Olaf M, de Herder, Wouter W, Hermus, Ad R, van der Horst-Schrivers, Anouk N A, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Brosens, Lodewijk A A, Dreijerink, Koen M A, Borel Rinkes, Inne H M, Timmers, H Th Marc, Valk, Gerlof D, Vriens, Menno R, Conemans, Elfi B, Lodewijk, Lutske, Moelans, Cathy M, Offerhaus, G Johan A, Pieterman, Carolina R C, Morsink, Folkert H, Dekkers, Olaf M, de Herder, Wouter W, Hermus, Ad R, van der Horst-Schrivers, Anouk N A, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Brosens, Lodewijk A A, Dreijerink, Koen M A, Borel Rinkes, Inne H M, Timmers, H Th Marc, Valk, Gerlof D, and Vriens, Menno R

Published
2018

26. Management of MEN1 Related Nonfunctioning Pancreatic NETs : A Shifting Paradigm: Results From the DutchMEN1 Study Group

Author

Nell, Sjoerd, Verkooijen, Helena M., Pieterman, Carolina R C, de Herder, Wouter W., Hermus, Ad R., Dekkers, Olaf M., van der Horst-Schrivers, Anouk N., Drent, Madeleine L., Bisschop, Peter H., Havekes, Bas, Borel Rinkes, Inne H M, Vriens, Menno R., Valk, Gerlof D., Nell, Sjoerd, Verkooijen, Helena M., Pieterman, Carolina R C, de Herder, Wouter W., Hermus, Ad R., Dekkers, Olaf M., van der Horst-Schrivers, Anouk N., Drent, Madeleine L., Bisschop, Peter H., Havekes, Bas, Borel Rinkes, Inne H M, Vriens, Menno R., and Valk, Gerlof D.

Published
2018

27. DNA methylation profiling in MEN1-related pancreatic neuroendocrine tumors reveals a potential epigenetic target for treatment

Author

Unit Opleiding Aios, Cancer, MS CGO, Pathologie Groep Van Diest, Pathologie Pathologen staf, Pathologie, Pathologie Groep Brosens, Regenerative Medicine and Stem Cells, MS Endocriene Oncologie, Heelkunde Opleiding, Conemans, Elfi B, Lodewijk, Lutske, Moelans, Cathy M, Offerhaus, G Johan A, Pieterman, Carolina R C, Morsink, Folkert H, Dekkers, Olaf M, de Herder, Wouter W, Hermus, Ad R, van der Horst-Schrivers, Anouk N A, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Brosens, Lodewijk A A, Dreijerink, Koen M A, Borel Rinkes, Inne H M, Timmers, H Th Marc, Valk, Gerlof D, Vriens, Menno R, Unit Opleiding Aios, Cancer, MS CGO, Pathologie Groep Van Diest, Pathologie Pathologen staf, Pathologie, Pathologie Groep Brosens, Regenerative Medicine and Stem Cells, MS Endocriene Oncologie, Heelkunde Opleiding, Conemans, Elfi B, Lodewijk, Lutske, Moelans, Cathy M, Offerhaus, G Johan A, Pieterman, Carolina R C, Morsink, Folkert H, Dekkers, Olaf M, de Herder, Wouter W, Hermus, Ad R, van der Horst-Schrivers, Anouk N A, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Brosens, Lodewijk A A, Dreijerink, Koen M A, Borel Rinkes, Inne H M, Timmers, H Th Marc, Valk, Gerlof D, and Vriens, Menno R

Published
2018

28. Management of MEN1 Related Nonfunctioning Pancreatic NETs: A Shifting Paradigm: Results From the DutchMEN1 Study Group

Author

MS Medische Oncologie, Trialbureau Beeld, Cancer, Circulatory Health, Epi Kanker Team A, JC onderzoeksprogramma Kanker, MS CGO, Regenerative Medicine and Stem Cells, Heelkunde Opleiding, MS Endocriene Oncologie, Nell, Sjoerd, Verkooijen, Helena M., Pieterman, Carolina R C, de Herder, Wouter W., Hermus, Ad R., Dekkers, Olaf M., van der Horst-Schrivers, Anouk N., Drent, Madeleine L., Bisschop, Peter H., Havekes, Bas, Borel Rinkes, Inne H M, Vriens, Menno R., Valk, Gerlof D., MS Medische Oncologie, Trialbureau Beeld, Cancer, Circulatory Health, Epi Kanker Team A, JC onderzoeksprogramma Kanker, MS CGO, Regenerative Medicine and Stem Cells, Heelkunde Opleiding, MS Endocriene Oncologie, Nell, Sjoerd, Verkooijen, Helena M., Pieterman, Carolina R C, de Herder, Wouter W., Hermus, Ad R., Dekkers, Olaf M., van der Horst-Schrivers, Anouk N., Drent, Madeleine L., Bisschop, Peter H., Havekes, Bas, Borel Rinkes, Inne H M, Vriens, Menno R., and Valk, Gerlof D.

Published
2018

29. High Fear of Disease Occurrence Is Associated With Low Quality of Life in Patients With Multiple Endocrine Neoplasia Type 1: Results From the Dutch MEN1 Study Group

Author

van Leeuwaarde, Rachel S, Pieterman, Carolina R C, Bleiker, Eveline M A, Dekkers, Olaf M, van der Horst-Schrivers, Anouk N, Hermus, Ad R, de Herder, Wouter W, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Vriens, Menno R, Valk, Gerlof D, van Leeuwaarde, Rachel S, Pieterman, Carolina R C, Bleiker, Eveline M A, Dekkers, Olaf M, van der Horst-Schrivers, Anouk N, Hermus, Ad R, de Herder, Wouter W, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Vriens, Menno R, and Valk, Gerlof D

Abstract

Objective: Multiple endocrine neoplasia type 1 (MEN1) is a hereditary disease characterized by a high risk of developing primary hyperparathyroidism, duodenopancreatic neuroendocrine tumors, and pituitary tumors (PITs). It is unclear if having MEN1 leads to psychological distress because of fear of disease occurrence (FDO), thereby potentially affecting quality of life.Design: A cross-sectional study was performed using the Dutch MEN1 cohort. All patients received the Cancer Worry Scale (a score ≥14 reflects high FDO), the Medical Outcomes Study 36-item Short-Form Health Survey (SF-36), and questions on sociodemographic and medical history.Results: A total of 227 of 285 (80%) eligible patients with MEN1 completed the questionnaire. The mean (± standard deviation) age was 47 ± 15 years. Overall, patients experienced an FDO of 15.1 ± 4.7, with 58% of patients having a score ≥14. This is higher than reported in previous studies assessing fear of cancer recurrence in different cancer populations (31% to 52%). Adjusted for age and sex, the FDO score was negatively associated with almost all SF-36 subscales. In multivariable analysis, the diagnosis of a PIT, a pancreatic neuroendocrine tumor, and not being employed were associated with FDO (P < 0.05). Patients had higher FDO scores for their family members than for themselves.Conclusion: The majority of patients with MEN1 have FDO for themselves and even more for their relatives. This psychological distress is associated with a lower health-related quality of life. Therefore, in the medical care for MEN1, emphasis should also be placed on FDO and quality of life.

Published
2018
Full Text
View/download PDF

30. Management of MEN1 Related Nonfunctioning Pancreatic NETs: A Shifting Paradigm

Author

Nell, Sjoerd, primary, Verkooijen, Helena M., additional, Pieterman, Carolina R. C., additional, de Herder, Wouter W., additional, Hermus, Ad R., additional, Dekkers, Olaf M., additional, van der Horst-Schrivers, Anouk N., additional, Drent, Madeleine L., additional, Bisschop, Peter H., additional, Havekes, Bas, additional, Borel Rinkes, Inne H. M., additional, Vriens, Menno R., additional, and Valk, Gerlof D., additional

Published
2018
Full Text
View/download PDF

31. High Fear of Disease Occurrence Is Associated With Low Quality of Life in Patients With Multiple Endocrine Neoplasia Type 1: Results From the Dutch MEN1 Study Group

Author

van Leeuwaarde, Rachel S, primary, Pieterman, Carolina R C, additional, Bleiker, Eveline M A, additional, Dekkers, Olaf M, additional, van der Horst-Schrivers, Anouk N, additional, Hermus, Ad R, additional, de Herder, Wouter W, additional, Drent, Madeleine L, additional, Bisschop, Peter H, additional, Havekes, Bas, additional, Vriens, Menno R, additional, and Valk, Gerlof D, additional

Published
2018
Full Text
View/download PDF

33. Prognostic value of WHO grade in pancreatic neuro-endocrine tumors in Multiple Endocrine Neoplasia type 1: Results from the DutchMEN1 Study Group

Author

Conemans, Elfi B, Brosens, Lodewijk A A, Raicu-Ionita, Gabriela M, Pieterman, Carolina R C, de Herder, Wouter W, Dekkers, Olaf M, Hermus, Ad R, van der Horst-Schrivers, Anouk N, Bisschop, Peter H, Havekes, Bas, Drent, Madeleine L, Timmers, H Th Marc, Offerhaus, G Johan, Valk, Gerlof D, Vriens, Menno R, Conemans, Elfi B, Brosens, Lodewijk A A, Raicu-Ionita, Gabriela M, Pieterman, Carolina R C, de Herder, Wouter W, Dekkers, Olaf M, Hermus, Ad R, van der Horst-Schrivers, Anouk N, Bisschop, Peter H, Havekes, Bas, Drent, Madeleine L, Timmers, H Th Marc, Offerhaus, G Johan, Valk, Gerlof D, and Vriens, Menno R

Abstract

BACKGROUND: The prognostic value of WHO grade in pancreatic neuroendocrine tumors (PanNETs) in patients with Multiple Endocrine Neoplasia Type 1 (MEN1) is unknown.METHODS: We performed a cohort study using the Dutch National MEN1 database, which includes >90% of the Dutch MEN1 population with data collected between 1990 and 2014. Formalin-fixed paraffin embedded tissue blocks from the largest resected PanNET per patient were collected. MIB1 staining was performed and KI67 labeling index (LI) was determined by manual eye-counting under a microscope and by digital image analysis. Mitotic count was evaluated from hematoxylin & eosin stains. Association between WHO grade and (time until) development of liver metastases was calculated.RESULTS: Sixty-nine MEN1 patients who underwent pancreatic surgery were included. Ten patients (14%) developed liver metastases and all had PanNETs ≥3 cm. WHO G1, G2 and G3 PanNETs were seen in 83% (n = 57), 16% (n = 11) and 1% (n = 1) respectively. In non-functioning PanNETs >2 cm, liver metastases occurred in 80% of WHO G2 PanNETs (4/5) compared to 23% (5/22) in WHO G1 PanNETs (p = 0.03) when WHO grade was based on mitotic count only. This significant association was not seen for WHO grade based on Ki67 LI. After five years, liver metastases in non-functioning PanNETs were not seen in tumors ≤2 cm, in 10% of the large WHO G1 (according to mitotic count only) tumors and in 60% of large WHO G2 tumors (p-value 0.000).CONCLUSION: High mitotic count is correlated with poor prognosis in MEN1 patients with large non-functioning PanNETs.

Published
2017
Full Text
View/download PDF

34. The future: medical advances in MEN1 therapeutic approaches and management strategies

Author

Unit Opleiding Aios, Cancer, MS Endocriene Oncologie, MS CGO, Heelkunde Opleiding, MS Interne Geneeskunde, van Leeuwaarde, Rachel S, de Laat, Joanne M, Pieterman, Carolina R C, Dreijerink, Koen, Vriens, Menno R, Valk, Gerlof D, Unit Opleiding Aios, Cancer, MS Endocriene Oncologie, MS CGO, Heelkunde Opleiding, MS Interne Geneeskunde, van Leeuwaarde, Rachel S, de Laat, Joanne M, Pieterman, Carolina R C, Dreijerink, Koen, Vriens, Menno R, and Valk, Gerlof D

Published
2017

35. Long-Term Natural Course of Small Nonfunctional Pancreatic Neuroendocrine Tumors in MEN1-Results From the Dutch MEN1 Study Group

Author

Unit Opleiding Aios, Cancer, MS Endocriene Oncologie, MS CGO, Regenerative Medicine and Stem Cells, Heelkunde Opleiding, Pieterman, Carolina R C, de Laat, Joanne M, Twisk, Jos W. R., van Leeuwaarde, Rachel S, de Herder, Wouter W, Dreijerink, Koen M A, Hermus, Ad R M M, Dekkers, Olaf M, van der Horst-Schrivers, Anouk N, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bastiaan, Borel Rinkes, Inne H M, Vriens, Menno R, Valk, Gerlof D, Unit Opleiding Aios, Cancer, MS Endocriene Oncologie, MS CGO, Regenerative Medicine and Stem Cells, Heelkunde Opleiding, Pieterman, Carolina R C, de Laat, Joanne M, Twisk, Jos W. R., van Leeuwaarde, Rachel S, de Herder, Wouter W, Dreijerink, Koen M A, Hermus, Ad R M M, Dekkers, Olaf M, van der Horst-Schrivers, Anouk N, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bastiaan, Borel Rinkes, Inne H M, Vriens, Menno R, and Valk, Gerlof D

Published
2017

37. MEN1 redefined, a clinical comparison of mutation-positive and mutation-negative patients

Author

de Laat, Joanne M., primary, van der Luijt, Rob B., additional, Pieterman, Carolina R. C., additional, Oostveen, Maria P., additional, Hermus, Ad R., additional, Dekkers, Olaf M., additional, de Herder, Wouter W., additional, van der Horst-Schrivers, Anouk N., additional, Drent, Madeleine L., additional, Bisschop, Peter H., additional, Havekes, Bas, additional, Vriens, Menno R., additional, and Valk, Gerlof D., additional

Published
2016
Full Text
View/download PDF

38. MEN1 redefined, a clinical comparison of mutation-positive and mutation-negative patients

Author

de Laat, Joanne M, van der Luijt, Rob B, Pieterman, Carolina R C, Oostveen, Maria P, Hermus, Ad R, Dekkers, Olaf M, de Herder, Wouter W, van der Horst-Schrivers, Anouk N, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Vriens, Menno R, Valk, Gerlof D, de Laat, Joanne M, van der Luijt, Rob B, Pieterman, Carolina R C, Oostveen, Maria P, Hermus, Ad R, Dekkers, Olaf M, de Herder, Wouter W, van der Horst-Schrivers, Anouk N, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Vriens, Menno R, and Valk, Gerlof D

Published
2016

39. Impact of delay in diagnosis in outcomes in MEN1 : results from the Dutch MEN1 study group

Author

van Leeuwaarde, Rachel S, van Nesselrooij, Bernadette P M, Hermus, Ad R, Dekkers, Olaf M, de Herder, Wouter W, van der Horst-Schrivers, Anouk N, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Vriens, Menno R, de Laat, Joanne M, Pieterman, Carolina R C, Valk, Gerlof D, van Leeuwaarde, Rachel S, van Nesselrooij, Bernadette P M, Hermus, Ad R, Dekkers, Olaf M, de Herder, Wouter W, van der Horst-Schrivers, Anouk N, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Vriens, Menno R, de Laat, Joanne M, Pieterman, Carolina R C, and Valk, Gerlof D

Published
2016

40. Impact of delay in diagnosis in outcomes in MEN1: results from the Dutch MEN1 study group

Author

MS Endocriene Oncologie, Genetica Sectie Oncogenetica, Cancer, Heelkunde Opleiding, MS CGO, MS Infectieziekten, MS Interne Geneeskunde, van Leeuwaarde, Rachel S, van Nesselrooij, Bernadette P M, Hermus, Ad R, Dekkers, Olaf M, de Herder, Wouter W, van der Horst-Schrivers, Anouk N, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Vriens, Menno R, de Laat, Joanne M, Pieterman, Carolina R C, Valk, Gerlof D, MS Endocriene Oncologie, Genetica Sectie Oncogenetica, Cancer, Heelkunde Opleiding, MS CGO, MS Infectieziekten, MS Interne Geneeskunde, van Leeuwaarde, Rachel S, van Nesselrooij, Bernadette P M, Hermus, Ad R, Dekkers, Olaf M, de Herder, Wouter W, van der Horst-Schrivers, Anouk N, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Vriens, Menno R, de Laat, Joanne M, Pieterman, Carolina R C, and Valk, Gerlof D

Published
2016

41. MEN1 redefined, a clinical comparison of mutation-positive and mutation-negative patients

Author

Genetica Sectie Genoomdiagnostiek, Cancer, Heelkunde Opleiding, MS CGO, MS Endocriene Oncologie, de Laat, Joanne M, van der Luijt, Rob B, Pieterman, Carolina R C, Oostveen, Maria P, Hermus, Ad R, Dekkers, Olaf M, de Herder, Wouter W, van der Horst-Schrivers, Anouk N, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Vriens, Menno R, Valk, Gerlof D, Genetica Sectie Genoomdiagnostiek, Cancer, Heelkunde Opleiding, MS CGO, MS Endocriene Oncologie, de Laat, Joanne M, van der Luijt, Rob B, Pieterman, Carolina R C, Oostveen, Maria P, Hermus, Ad R, Dekkers, Olaf M, de Herder, Wouter W, van der Horst-Schrivers, Anouk N, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Vriens, Menno R, and Valk, Gerlof D

Published
2016

42. Long-Term Natural Course of Pituitary Tumors in Patients With MEN1 : Results From the DutchMEN1 Study Group (DMSG)

Author

de Laat, Joanne M, Dekkers, Olaf M, Pieterman, Carolina R C, Kluijfhout, Wouter P, Hermus, Ad R, Pereira, Alberto M, van der Horst-Schrivers, Anouk N, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, de Herder, Wouter W, Valk, Gerlof D., de Laat, Joanne M, Dekkers, Olaf M, Pieterman, Carolina R C, Kluijfhout, Wouter P, Hermus, Ad R, Pereira, Alberto M, van der Horst-Schrivers, Anouk N, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, de Herder, Wouter W, and Valk, Gerlof D.

Published
2015

43. Long-Term Natural Course of Pituitary Tumors in Patients With MEN1: Results From the DutchMEN1 Study Group (DMSG)

Author

MS Infectieziekten, MS CGO, MS Endocriene Oncologie, Cancer, Other research (not in main researchprogram), de Laat, Joanne M, Dekkers, Olaf M, Pieterman, Carolina R C, Kluijfhout, Wouter P, Hermus, Ad R, Pereira, Alberto M, van der Horst-Schrivers, Anouk N, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, de Herder, Wouter W, Valk, Gerlof D., MS Infectieziekten, MS CGO, MS Endocriene Oncologie, Cancer, Other research (not in main researchprogram), de Laat, Joanne M, Dekkers, Olaf M, Pieterman, Carolina R C, Kluijfhout, Wouter P, Hermus, Ad R, Pereira, Alberto M, van der Horst-Schrivers, Anouk N, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, de Herder, Wouter W, and Valk, Gerlof D.

Published
2015

44. No Association of Blood Type O With Neuroendocrine Tumors in Multiple Endocrine Neoplasia Type 1

Author

MS Medische Oncologie, MS Endocriene Oncologie, MS Infectieziekten, Divisie Beeld & Oncologie, Cancer, Heelkunde Opleiding, Other research (not in main researchprogram), Nell, Sjoerd, van Leeuwaarde, Rachel S, Pieterman, Carolina R C, de Laat, Joanne M, Hermus, Ad R, Dekkers, Olaf M, de Herder, Wouter W, van der Horst-Schrivers, Anouk N, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Borel Rinkes, Inne H M, Vriens, Menno R, Valk, Gerlof D., MS Medische Oncologie, MS Endocriene Oncologie, MS Infectieziekten, Divisie Beeld & Oncologie, Cancer, Heelkunde Opleiding, Other research (not in main researchprogram), Nell, Sjoerd, van Leeuwaarde, Rachel S, Pieterman, Carolina R C, de Laat, Joanne M, Hermus, Ad R, Dekkers, Olaf M, de Herder, Wouter W, van der Horst-Schrivers, Anouk N, Drent, Madeleine L, Bisschop, Peter H, Havekes, Bas, Borel Rinkes, Inne H M, Vriens, Menno R, and Valk, Gerlof D.

Published
2015

46. Primary Hyperparathyroidism in MEN1 Patients

Author

Pieterman, Carolina R. C., primary, van Hulsteijn, Leonie T., additional, den Heijer, Martin, additional, van der Luijt, Rob B., additional, Bonenkamp, J. J., additional, Hermus, Ad R. M. M., additional, Borel Rinkes, Inne H. M., additional, Vriens, Menno R., additional, and Valk, Gerlof D., additional

Published
2012
Full Text
View/download PDF

47. Status of Surveillance and Nonsurgical Therapy for Small Nonfunctioning Pancreatic Neuroendocrine Tumors.

Author

van Beek DJ, Verschuur AVD, Brosens LAA, Valk GD, Pieterman CRC, and Vriens MR

Subjects
Humans, Neuroendocrine Tumors pathology, Pancreatic Neoplasms pathology, Multiple Endocrine Neoplasia Type 1 pathology, Multiple Endocrine Neoplasia Type 1 surgery, Neuroectodermal Tumors, Primitive
Abstract

Pancreatic neuroendocrine tumors (PNETs) occur in < 1/100,000 patients and most are nonfunctioning (NF). Approximately 5% occur as part of multiple endocrine neoplasia type 1. Anatomic and molecular imaging have a pivotal role in the diagnosis, staging and active surveillance. Surgery is generally recommended for nonfunctional pancreatic neuroendocrine tumors (NF-PNETs) >2 cm to prevent metastases. For tumors ≤2 cm, active surveillance is a viable alternative. Tumor size and grade are important factors to guide management. Assessment of death domain-associated protein 6/alpha-thalassemia/mental retardation X-linked and alternative lengthening of telomeres are promising novel prognostic markers. This review summarizes the status of surveillance and nonsurgical management for small NF-PNETs, including factors that can guide management., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Published
2023
Full Text
View/download PDF

48. Management of adrenocorticotropic hormone-secreting neuroendocrine tumors and the role of bilateral adrenalectomy in ectopic Cushing syndrome.

Author

Landry JP, Clemente-Gutierrez U, Pieterman CRC, Chiang YJ, Waguespack SG, Jimenez C, Habra MA, Halperin DM, Fisher SB, Graham PH, and Perrier ND

Subjects
Adrenalectomy adverse effects, Adrenocorticotropic Hormone, Humans, Retrospective Studies, ACTH Syndrome, Ectopic complications, ACTH Syndrome, Ectopic diagnosis, Cushing Syndrome diagnosis, Cushing Syndrome etiology, Cushing Syndrome surgery, Hyperglycemia complications, Neuroendocrine Tumors complications, Neuroendocrine Tumors surgery
Abstract

Background: Neuroendocrine tumors can cause ectopic Cushing syndrome, and most patients have metastatic disease at diagnosis. We identified risk factors for outcome, evaluated ectopic Cushing syndrome management, and explored the role of bilateral adrenalectomy in this population., Methods: This was a retrospective study including patients with diagnosis of ectopic Cushing Syndrome secondary to neuroendocrine tumors with adrenocorticotropic hormone secretion treated at our quaternary referral center over a 40-year period (1980-2020)., Results: Seventy-six patients were included. Mean age at diagnosis was 46.3 ± 15.8 years. Most patients (N = 61, 80%) had metastases at ectopic Cushing syndrome diagnosis. Average follow-up was 2.9 ± 3.7 years (range, 4 months-17.2 years). Patients with neuroendocrine tumors before ectopic Cushing syndrome had more frequent metastatic disease and resistant ectopic Cushing syndrome. Patients with de novo hyperglycemia, poor neuroendocrine tumor differentiation, and metastatic disease had worse survival. Of those with nonmetastatic disease, 8 (53%) had ectopic Cushing syndrome resolution after neuroendocrine tumor resection, 3 (20%) were medically controlled, and 4 (27%) underwent bilateral adrenalectomy. In patients with metastatic neuroendocrine tumors, hypercortisolism was initially medically managed in 92%, 3% underwent immediate bilateral adrenalectomy, 2% had control after primary neuroendocrine tumor debulking, and 2% were lost to follow-up. Medical treatment resulted in hormonal control in 7 (13%) patients. Of the 49 patients with metastatic disease and medically resistant ectopic Cushing syndrome, 23 ultimately had bilateral adrenalectomy with ectopic Cushing syndrome cure in all., Conclusion: Patients with neuroendocrine tumors before ectopic Cushing syndrome development were more likely metastatic and had worse survival. De novo hyperglycemia and poor neuroendocrine tumor differentiation were predictive of worse prognosis. Medical control of hypercortisolism is difficult to achieve in patients with neuroendocrine tumors-ectopic Cushing syndrome. Well-selected patients may benefit from bilateral adrenalectomy early in the treatment algorithm, and multidisciplinary management is essential in this complex disease., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Published
2022
Full Text
View/download PDF

49. Evaluation of risk factors, long-term outcomes, and immediate and delayed autotransplantation to minimize postsurgical hypoparathyroidism in multiple endocrine neoplasia type 1 (MEN1): A retrospective cohort study.

Author

Landry JP, Pieterman CRC, Clemente-Gutierrez U, Grubbs EG, Fisher SB, Graham PH, Waguespack SG, and Perrier ND

Subjects
Humans, Neoplasm Recurrence, Local surgery, Parathyroid Glands transplantation, Parathyroid Hormone, Parathyroidectomy adverse effects, Retrospective Studies, Risk Factors, Transplantation, Autologous adverse effects, Hypoparathyroidism etiology, Hypoparathyroidism prevention & control, Multiple Endocrine Neoplasia Type 1 complications, Multiple Endocrine Neoplasia Type 1 surgery
Abstract

Background: Postoperative hypoparathyroidism from inadequate parathyroid hormone is of concern after multigland resections in multiple endocrine neoplasia type 1-related primary hyperparathyroidism. We evaluated risk factors, long-term outcomes, and roles of autotransplantation and cryopreservation in postoperative hypoparathyroidism in multiple endocrine neoplasia type 1., Methods: Retrospective cohort study of patients with multiple endocrine neoplasia type 1 and parathyroidectomy who were evaluated at MD Anderson from 1990 to 2020., Results: We included 206 patients. Median follow-up after the last operation (index 65%, reoperation 35%) was 8 years. Index parathyroidectomy was subtotal in 47%, less than subtotal in 42%, and total in 12%; hypoparathyroidism was more frequent after total parathyroidectomy. Forty-seven patients (23%) had hypoparathyroidism ≥6 months; odds were significantly higher when cumulative ≥4 glands were resected (odds ratio 6 [2.96-12.24]) or when immediate postoperative parathyroid hormone was <15 pg/mL (odds ratio 13.10 [3.61-47.47]). After median 26 months postoperatively, 30% recovered parathyroid function spontaneously; this was less likely when ≥4 glands were resected (odds ratio 0.19 [0.05-0.72]). None of the 4 patients who were aparathyroid (parathyroid hormone undetectable or ≤3 pg/mL) at 6 months postoperatively recovered parathyroid function. Immediate autotransplantation success rate was 72%. Cryopreservation was performed in 96 operations with delayed autotransplantation in 10 patients (10% utilization), of whom 5 recovered parathyroid function (time to recovery 12-93 months)., Conclusion: Odds of prolonged hypoparathyroidism are higher when cumulative ≥4 glands are resected or postoperative parathyroid hormone is <15 pg/mL. Spontaneous recovery occurred but was less likely when ≥4 glands were resected or patients were aparathyroid at 6 months postoperatively. Cryopreservation should be sparingly used, but there is value in select high-risk patients such as reoperative parathyroidectomy/cervical surgery., (Copyright © 2021. Published by Elsevier Inc.)

Published
2022
Full Text
View/download PDF

50. Health-Related Quality of Life in Patients with Multiple Endocrine Neoplasia Type 1.

Author

van Leeuwaarde RS, Pieterman CRC, May AM, Dekkers OM, van der Horst-Schrivers AN, Hermus AR, de Herder WW, Drent ML, Bisschop PH, Havekes B, Vriens MR, and Valk GD

Subjects
Adult, Cross-Sectional Studies, Female, Humans, Male, Middle Aged, Netherlands, Unemployment psychology, Cost of Illness, Multiple Endocrine Neoplasia Type 1 psychology, Quality of Life psychology
Abstract

Introduction: Multiple endocrine neoplasia type 1 (MEN1) is a hereditary endocrine tumor syndrome characterized by the triad of primary hyperparathyroidism, duodenopancreatic neuroendocrine tumors (pNETs), and pituitary tumors. Patients are confronted with substantial morbidity and are consequently at risk for an impaired quality of life (QOL). Meticulous assessment of QOL and associated factors in a representative population is needed to understand the full spectrum of the burden of the disease., Patients and Methods: A cross-sectional study was performed using the national Dutch MEN1 cohort. Patients with a confirmed MEN1 mutation received the SF-36 Health Related Quality of Life questionnaire and questions regarding sociodemographic and medical history., Results: A total of 227 of 285 (80%) eligible MEN1 patients returned the questionnaires. Health-related QOL scores (HRQOL) in MEN1 patients were significantly lower for the majority of subscales of the SF-36 in comparison with the general Dutch population. The most consistent predictor for HRQOL was employment status, followed by the presence of a pituitary tumor. 16% of patients harboring a pNET and 29% of patients with a pituitary tumor according to the medical records, reported that they were unaware of such a tumor. These subgroups of patients had several significant better QOL scores than patients who were aware of their pNET or pituitary tumors., Conclusion: Patients with MEN1 have an impaired QOL in comparison with the general Dutch population warranting special attention within routine care. For daily practice, physicians should be aware of their patients' impaired QOL and of the impact of unemployment on QOL., (© 2020 The Author(s) Published by S. Karger AG, Basel.)

Published
2021
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results