Rachel S van Leeuwaarde1, Carolina R C Pieterman2, Anne M May3, Olaf M Dekkers4, Anouk N van der Horst-Schrivers5, Ad R Hermus6, Wouter W de Herder7, Madeleine L Drent8, Peter H Bisschop9, Bas Havekes10, Menno R Vriens11, Gerlof D Valk12. 1. Department of Endocrine Oncology, University Medical Center Utrecht, University of Utrecht, Utrecht, The Netherlands, r.vanleeuwaarde@umcutrecht.nl. 2. Dutch MEN advocacy group, Department of Endocrine Oncology, University Medical Center Utrecht, University of Utrecht, Utrecht, The Netherlands. 3. Department of Clinical Epidemiology, Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, University of Utrecht, Utrecht, The Netherlands. 4. Departments of Endocrinology and Metabolism and Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands. 5. Department of Endocrinology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. 6. Department of Endocrinology, Radboud University Medical Center, Nijmegen, The Netherlands. 7. Department of Internal Medicine, Erasmus Medical Center, Rotterdam, The Netherlands. 8. Department of Internal Medicine, Section of Endocrinology, VU University Medical Center, Amsterdam, The Netherlands. 9. Department of Endocrinology and Metabolism, Academic Medical Center, Amsterdam, The Netherlands. 10. Department of Internal Medicine, Division of Endocrinology, Maastricht University Medical Center, Maastricht, The Netherlands. 11. Department of Endocrine Surgery, University Medical Center Utrecht, University of Utrecht, Utrecht, The Netherlands. 12. Department of Endocrine Oncology, University Medical Center Utrecht, University of Utrecht, Utrecht, The Netherlands.
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.
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 MEN1patients returned the questionnaires. Health-related QOL scores (HRQOL) in MEN1patients 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.
Authors: Jason B Liu; Jinman Cai; Mashaal Dhir; Alessandro Paniccia; Amer H Zureikat; Kimberly M Ramonell; Kelly L McCoy; Sally E Carty; Linwah Yip Journal: Ann Surg Oncol Date: 2022-08-13 Impact factor: 4.339
Authors: Weihua Kong; Max Benjamin Albers; Jerena Manoharan; Joachim Nils Goebel; Peter Herbert Kann; Moritz Jesinghaus; Detlef Klaus Bartsch Journal: Cancers (Basel) Date: 2022-04-11 Impact factor: 6.575
Authors: Xuefang Hu; Jian Guan; Yangdi Wang; Siya Shi; Chenyu Song; Zi-Ping Li; Shi-Ting Feng; Jie Chen; Yanji Luo Journal: Ann Transl Med Date: 2021-06
Authors: Andrea Crafa; Aldo E Calogero; Rossella Cannarella; Laura M Mongioi'; Rosita A Condorelli; Emanuela A Greco; Antonio Aversa; Sandro La Vignera Journal: Front Endocrinol (Lausanne) Date: 2021-06-16 Impact factor: 5.555