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Clinical and radiological presentation of parasellar ectopic pituitary adenomas: case series and systematic review of the literature

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Abstract

Purpose

Parasellar ectopic pituitary adenomas (pEPAs) are extremely rare tumors located out of the sella turcica. PEPAs are heterogeneous entities in terms of anatomical localization and secretion of anterior pituitary hormones.

Methods

Multicenter retrospective study. Clinical charts’ consultation of patients diagnosed with parasellar lesions, to identify all subjects fulfilling the diagnostic criteria of parasellar EPAs. Systematic review of the literature focused on the medical management of prolactin-secreting pEPAs and on the prevalence of radiological bone invasion in pEPAs.

Results

We identified four cases of pEPAs: (1) 54-year-old female with a prolactin-secreting suprasellar EPA successfully treated with cabergoline; (2) 74-year-old male with a non-functioning EPA of the sphenoidal sinus treated with endoscopic transsphenoidal surgery; (3) 75-year-old female with a giant lesion of the skull base (maximum diameter 7.2 cm) diagnosed as a non-functioning EPA after biopsy; (4) 49-year-old male with a silent corticotroph EPA of the sphenoidal sinus and clivus. Three out of four cases had radiological evidence of invasion of the surrounding bone structures. A systematic review of the literature highlighted that medical therapy can be effective in prolactin-secreting pEPAs. Overall, we found mention of local invasiveness in 65/147 cases (44.2%), confirmed by radiological signs of bone invasion/erosion.

Conclusion

Our experience confirms the heterogeneity of pEPAs in terms of clinical and radiological presentation, as well as hormone secretion. PEPAs show a high frequency of radiological bone invasion, though similar to that of sellar pituitary adenomas. Although extremely rare, pEPAs need to be considered in the differential diagnosis of parasellar lesions.

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  1. Claudia Campana and Federica Nista equally contributed to the manuscript

Authors and Affiliations

  1. Endocrinology Unit, Department of Internal Medicine and Center of Excellence for Biomedical Research, University of Genoa, Genoa, Italy

    C. Campana, F. Nista & D. Ferone

  2. Department of Radiology, Ospedale di Lavagna, Lavagna, Italy

    L. Castelletti

  3. Endocrinology, Department of Translational Medicine, Università del Piemonte Orientale, Novara, Italy

    M. Caputo, P. Marzullo & A. Ferrero

  4. Department of Health Sciences, Università del Piemonte Orientale, Novara, Italy

    M. Caputo

  5. Endocrinology Unit, IRCCS Humanitas Research Hospital and Humanitas University, Rozzano, Italy

    E. Lavezzi & A. Lania

  6. Laboratorio di Ricerche Metaboliche, IRCCS Istituto Auxologico Italiano, Piancavallo, Verbania, Italy

    P. Marzullo

  7. Department of Clinical Pathology, IRCCS Ospedale Policlinico San Martino, Genoa, Italy

    G. Gaggero

  8. Unit of Otorhinolaryngology-Head and Neck Surgery, University of Genoa, Genoa, Italy

    F. R. Canevari

  9. Division of Neurosurgery, Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, IRCCS Ospedale Policlinico San Martino, Genoa, Italy

    D. C. Rossi & G. Zona

  10. Endocrinology Unit, IRCCS Ospedale Policlinico San Martino, 16132, Genoa, Italy

    D. Ferone & F. Gatto

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  1. C. Campana
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Contributions

Study conception: FG, DCR. Material preparation, data collection and analysis were performed by CC, FN, LC, MC and EL. The first draft of the manuscript was written by CC, FN and supervised by FG. All authors revised and contributed to the following versions of the manuscript. All authors read and approved the final manuscript.

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Correspondence to F. Gatto.

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Conflicts of interest

DF has been a speaker for and participated on advisory boards and received research grants from Novartis, Ipsen and Pfizer. FG has been a speaker for Novartis and has participated on advisory boards of Novartis, AMCo Ltd, and IONIS Pharmaceuticals. The other authors have no conflicts of interest to declare.

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Clinical data were collected in accordance with local ethics requirements for case reports. The study was performed in line with the principles of the Declaration of Helsinki.

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Campana, C., Nista, F., Castelletti, L. et al. Clinical and radiological presentation of parasellar ectopic pituitary adenomas: case series and systematic review of the literature. J Endocrinol Invest 45, 1465–1481 (2022). https://doi.org/10.1007/s40618-022-01758-x

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