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Ectopic pituitary adenomas: clinical features, diagnostic challenges and management

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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.

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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

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

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This work was supported by the CAMS Innovation Fund for Medical Science (CAMS-2016-I2M-1-002).

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  1. Jianyu Zhu and Zhicheng Wang contributed equally to this paper.

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  1. Department of Neurosurgery, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, 100730, China

    Jianyu Zhu, Zhicheng Wang, Yi Zhang, Xiaoxu Li, Jie Liu, Kan Deng, Renzhi Wang & Yong Yao

  2. Key Laboratory of Endocrinology of National Health Commission, Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, 100730, China

    Lin Lu, Hui Pan & Huijuan Zhu

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Zhu, J., Wang, Z., Zhang, Y. et al. Ectopic pituitary adenomas: clinical features, diagnostic challenges and management. Pituitary 23, 648–664 (2020). https://doi.org/10.1007/s11102-020-01071-x

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