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Gregory A Kaltsas
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Jane Evanson Endocrine Unit, Department of Academic Radiology, Department of Endocrinology, Department of Endocrinology, Department of Pathophysiology, National University of Athens, Athens, Greece

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Alexandra Chrisoulidou Endocrine Unit, Department of Academic Radiology, Department of Endocrinology, Department of Endocrinology, Department of Pathophysiology, National University of Athens, Athens, Greece

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Ashley B Grossman Endocrine Unit, Department of Academic Radiology, Department of Endocrinology, Department of Endocrinology, Department of Pathophysiology, National University of Athens, Athens, Greece

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The sellar and parasellar region is an anatomically complex area where a number of neoplastic, inflammatory, infectious, developmental and vascular diseases can develop. Although most sellar lesions are due to pituitary adenomas, a number of other pathologies involving the parasellar region can present in a similar manner. The diagnosis of such lesions involves a multidisciplinary approach, and detailed endocrinological, ophthalmological, neuroimaging, neurological and finally histological studies are required. Correct diagnosis prior to any intervention is essential as the treatment of choice will be different for each disorder, particularly in the case of primary malignant parasellar tumours. The complexity of structures that define the parasellar region can produce a variety of neoplastic processes, the malignant potential of which relies on histological grading. In the majority of parasellar tumours, a multimodal therapeutic approach is frequently necessary including surgery, radiotherapy, primary or adjuvant medical treatment and replacement of apparent endocrine deficits. Disease-specific medical therapies are mandatory in order to prevent recurrence or further tumour growth. This is particularly important as neoplastic lesions of the parasellar region tend to recur after prolonged follow-up, even when optimally treated. Apart from the type of treatment, identification of clinical and radiological features that could predict patients with different prognosis seems necessary in order to identify high-risk patients. Due to their rarity, central registration of parasellar tumours is required in order to be able to provide evidence-based diagnostic and mainly therapeutic approaches.

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Paul Benjamin Loughrey Patrick G Johnston Centre for Cancer Research, Queen’s University Belfast, Belfast, UK
Regional Centre for Endocrinology and Diabetes, Belfast Health and Social Care Trust, Belfast, UK

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Brian Herron Department of Cellular Pathology, Belfast Health and Social Care Trust, Belfast, UK

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Stephen Cooke Department of Neurosurgery, Belfast Health and Social Care Trust, Belfast, UK

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Philip Weir Department of Neurosurgery, Belfast Health and Social Care Trust, Belfast, UK

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Jayna Elizabeth Smyth Regional Centre for Endocrinology and Diabetes, Belfast Health and Social Care Trust, Belfast, UK

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Karen R Mullan Regional Centre for Endocrinology and Diabetes, Belfast Health and Social Care Trust, Belfast, UK

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Estelle G Healy Department of Cellular Pathology, Belfast Health and Social Care Trust, Belfast, UK

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Jane Evanson Department of Radiology, Barts Health NHS Trust, London, UK

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Stephanie G Craig Patrick G Johnston Centre for Cancer Research, Queen’s University Belfast, Belfast, UK

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Jacqueline A James Patrick G Johnston Centre for Cancer Research, Queen’s University Belfast, Belfast, UK
Department of Cellular Pathology, Belfast Health and Social Care Trust, Belfast, UK
Northern Ireland Biobank, Belfast Health and Social Care Trust, Belfast, UK

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Márta Korbonits Department of Endocrinology, William Harvey Research Institute, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, London, UK

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Steven J Hunter Regional Centre for Endocrinology and Diabetes, Belfast Health and Social Care Trust, Belfast, UK

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Cushing’s disease is a rare condition that occurs due to an adrenocorticotrophin-producing corticotrophinoma arising from the pituitary gland. The consequent hypercortisolaemia results in multisystem morbidity and mortality. This study aims to report incidence, clinicopathological characteristics, remission outcomes and mortality in a regional pituitary neurosurgical cohort of patients diagnosed with Cushing’s disease in Northern Ireland (NI) from 2000 to 2019. Clinical, biochemical and radiological data from a cohort of patients operated for Cushing’s disease were retrospectively collected and analysed. Fifty-three patients were identified, resulting in an estimated annual incidence of Cushing’s disease of 1.39–1.57 per million population per year. Females accounted for 72% (38/53) of the cohort. The majority (74%, 39/53) of corticotrophinomas were microadenomas and in 44% (17/39) of these no tumour was identified on preoperative magnetic resonance imaging. Histopathological characterisation was similarly difficult, with no tumour being identified in the histopathological specimen in 40% (21/53) of cases. Immediate postoperative remission rates were 53% and 66% when considering serum morning cortisol cut-offs of ≤ 50 nmol/L (1.8 µg/dL) and ≤ 138 nmol/L (5 µg/dL), respectively, in the week following pituitary surgery. Approximately 70% (37/53) of patients achieved longer-term remission with a single pituitary surgery. Three patients had recurrent disease. Patients with Cushing’s disease had a significantly higher mortality rate compared to the NI general population (standardised mortality ratio 8.10, 95% CI 3.3–16.7, P < 0.001). Annual incidence of Cushing’s disease in NI is consistent with other Northern European cohorts. Functioning corticotrophinomas are a clinically, radiologically and histopathologically elusive disease with increased mortality compared to the general population.

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