Objective: The European Reference Network on Rare Endocrine Conditions (Endo-ERN) aims to organize high-quality healthcare throughout Europe, including care for pituitary adenoma patients. As surgery is the mainstay of treatment, we aimed to describe the current surgical practice and published surgical outcomes of pituitary adenoma within Endo-ERN. Design and Methods: Systematic review and meta-analysis of studies reporting surgical outcomes of pituitary adenoma patients within Endo-ERN MTG6 Pituitary Reference Centers between 2010 and 2019. A survey was completed by Reference Centers on their current surgical practice. Results: Eighteen out of 43 (42%) Reference Centers located in seven of the 20 (35%) MTG6 represented countries published 48 articles. Remission rates were 50% (95%CI: 42-59) for patients with acromegaly, 68% (95%CI: 60-75) for Cushing’s disease, and 53% (95%CI: 39-66%) for prolactinoma. Gross total resection was achieved in 49% (95%CI: 37-61%) of patients, visual improvement in 78% (95%CI: 68-87). Mortality, hemorrhage, and carotid injury occurred in less than 1% of patients. New onset hypopituitarism occurred in 16% (95%CI: 11-23), transient diabetes insipidus in 12% (95%CI: 6-21), permanent diabetes insipidus in 4% (95%CI: 3-6), SIADH in 9% (95%CI: 5-14), severe epistaxis in 2% (95%CI: 0-4), and CSF leak in 4% (95%CI: 2-6). Thirty-five (81%) centers completed the survey: 54% operate endoscopically, 57% together with an ENT-surgeon. Conclusion: The results of this study could be used as a first benchmark for outcomes of pituitary adenoma surgery within Endo-ERN. However, the heterogeneity between studies in the reporting of outcomes hampers comparability, and warrants outcome collection through registries.

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Patient journeys are instruments developed by EURORDIS, The Voice of Rare Disease Patients in Europe, to collect patients’ experiences; they may identify gaps and areas deserving improvement, as well as elements positively considered by affected persons. As with other patient reported experiences, they can complete the clinical evaluation and management of a specific disease, improving the often long diagnostic delay, therapy, patient education and access to knowledgeable multidisciplinary teams. This review discusses the utility of such patient-reported experience measures (PREMs) and summarizes experiences of patients with acromegaly, Addison’s disease and congenital adrenal hyperplasia from different European countries. Despite rare endocrine diseases being varied and presenting differently, feelings of not having been taken seriously by health professionals, family and friends was a common patient complaint. Empathy and a positive patient-centred environment tend to improve clinical practice, by creating a trustworthy and understanding atmosphere, where individual patient needs are considered. Offering access to adequate patient information on their disease, treatments and outcome, helps to adapt to living with a chronic disease and what to expect in the future, contemplating the impact of a disease on patients’ everyday life, not only clinical outcome but also social, financial, educational, family and leisure issues is desirable; this facilitates more realistic expectancies for patients, and can even lead to a reduction in health costs. Patient empowerment with patient-centred approaches to these complex or chronic diseases should be contemplated more and more, for the benefit of those affected, but also for the entire health system.

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