Ectopic pituitary adenomas: clinical features, diagnostic challenges and management

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Ectopic pituitary adenomas: clinical features, diagnostic challenges and management Jianyu Zhu1   · Zhicheng Wang1   · Yi Zhang1 · Xiaoxu Li1   · Jie Liu1   · Kan Deng1 · Lin Lu2 · Hui Pan2 · Renzhi Wang1 · Yong Yao1   · Huijuan Zhu2

© Springer Science+Business Media, LLC, part of Springer Nature 2020

Abstract Purpose  Ectopic pituitary adenomas (EPAs) are extremely rare pituitary adenomas located outside the sella turcica without any connection with intrasellar components. This study aims to review all the reported cases to date and describe the clinical characteristics of EPAs. Methods  In a retrospective chart review, 14 patients were identified with EPAs in our hospital. A literature review was performed, and 166 cases in the literature met the criteria. Clinical data were analyzed. Results  Of 180 patients with EPAs, the mean age at diagnosis was 45.4 years, and 66.5% of the patients were females. EPAs were mainly located in the sphenoid sinus (34.4%) and suprasellar region (25.6%), followed by the clivus (15.6%), cavernous sinus (13.3%) and nasopharynx (5.6%). Adrenocorticotropic hormone (ACTH)-secreting (38.9%) and nonfunctioning (27.2%) adenomas were predominant. Patients with suprasellar EPAs were more likely to present menstrual disorders and visual changes, while patients with clival EPAs were more likely to suffer from headaches. EPAs in the cavernous sinus and suprasellar space were more likely to be initially misdiagnosed as a suspicious intrasellar mass on imaging examination. The complete tumor resection rates for EPAs in the sphenoid sinus, suprasellar region, clivus, cavernous sinus and nasopharynx were 72.3%, 88.6%, 45.0%, 73.3% and 88.9%, respectively. Conclusions  EPA clinical characteristics varied across different tumor locations and hormone-secreting types. In addition to comprehensive hormone evaluation and careful review of imaging data, nuclear medicine and surgical biopsy should also be considered when facing differential difficulty. EPA management should be individualized. Keywords  Ectopic pituitary adenoma · Tumor locations · Clinical characteristics · Diagnosis · Treatment Abbreviations EPA Ectopic pituitary adenoma ACTH Adrenocorticotrophic hormone DST Dexamethasone suppression test IGF1 Insulin-like growth factor GH Growth hormone OGTT​ Oral glucose tolerance test Jianyu Zhu and Zhicheng Wang contributed equally to this paper. Electronic supplementary material  The online version of this article (https​://doi.org/10.1007/s1110​2-020-01071​-x) contains supplementary material, which is available to authorized users. * Yong Yao [email protected] * Huijuan Zhu [email protected] Extended author information available on the last page of the article

TSH Thyroid stimulating hormone MRI Magnetic resonance imaging TCS Transcranial surgery TSS Transsphenoidal surgery CDI Central diabetes insipidus PRL Prolactin CT Computed tomography T1WI T1-weighted imaging T2WI T2-weighted imaging PET Positron emission tomography

Introduction Pituitary adenomas are common intracran