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Ectopic GH-secreting pituitary adenoma of the clivus: systematic literature review of a challenging tumour.
Pituitary. 2020 Aug; 23(4):457-466.P

Abstract

PURPOSE

Acromegaly is usually due to growth hormone (GH)-secreting pituitary adenomas, but it may be exceptionally caused by GH-secreting ectopic pituitary adenomas (EPA). EPA are defined as extra-sellar pituitary tumours, extra- or intra-cranially sited, entirely separated from the pituitary stalk and gland. The aim of the study is to address the challenges in the management of clival GHEPA.

METHODS

We reported a case of a 53-year-old acromegalic patient with a primary clival GHEPA and reviewed systematically the relevant English literature between 1975 and 2019, in keeping with the PRISMA guidelines.

RESULTS

Four cases of primary clival GHEPA have been described in literature apart from ours. All patients presented with acromegalic features, elevated circulating GH and/or insulin-like growth factor-1 levels. Hyperprolactinemia and empty sella were described in two cases, respectively. These tumours show the typical imaging characteristics of pituitary adenomas, but their neuroradiological diagnosis may be challenging due to their sizes and the difficulty in defining the absence of connections with the pituitary fossa.

CONCLUSION

Although primary clival GHEPA are exceedingly rare, even if likely under-reported in literature, they should be considered in the differential diagnosis of clival tumours because of their specific management. Surgery represents the first-line treatment option, while medical and radiation therapies can be adopted as neo-adjuvant, adjuvant or primary treatments according to tumour and patient characteristics.

Authors+Show Affiliations

Department of Neurosurgery, Ospedale "Vito Fazzi", Piazza F. Muratore, 1, 73100, Lecce, Italy. dott.luciariccio@libero.it. Vita-Salute San Raffaele University, Milan, Italy. dott.luciariccio@libero.it.Department of Neurosurgery, Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust, Manchester Academic Health Sciences Centre, University of Manchester, Manchester, UK.ENT Department, Ospedale "Vito Fazzi", Lecce, Italy.Department of Radiology, Azienda Ospedaliera "Cardinale Giovanni Panico", Tricase, Italy.Department of Neurosurgery, Ospedale "Vito Fazzi", Piazza F. Muratore, 1, 73100, Lecce, Italy.

Pub Type(s)

Case Reports
Journal Article
Systematic Review

Language

eng

PubMed ID

32504302

Citation

Riccio, Lucia, et al. "Ectopic GH-secreting Pituitary Adenoma of the Clivus: Systematic Literature Review of a Challenging Tumour." Pituitary, vol. 23, no. 4, 2020, pp. 457-466.
Riccio L, Donofrio CA, Tomacelli G, et al. Ectopic GH-secreting pituitary adenoma of the clivus: systematic literature review of a challenging tumour. Pituitary. 2020;23(4):457-466.
Riccio, L., Donofrio, C. A., Tomacelli, G., De Blasi, R., & Melatini, A. (2020). Ectopic GH-secreting pituitary adenoma of the clivus: systematic literature review of a challenging tumour. Pituitary, 23(4), 457-466. https://doi.org/10.1007/s11102-020-01057-9
Riccio L, et al. Ectopic GH-secreting Pituitary Adenoma of the Clivus: Systematic Literature Review of a Challenging Tumour. Pituitary. 2020;23(4):457-466. PubMed PMID: 32504302.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Ectopic GH-secreting pituitary adenoma of the clivus: systematic literature review of a challenging tumour. AU - Riccio,Lucia, AU - Donofrio,Carmine Antonio, AU - Tomacelli,Giovanni, AU - De Blasi,Roberto, AU - Melatini,Alessandro, PY - 2020/6/7/pubmed PY - 2021/5/4/medline PY - 2020/6/7/entrez KW - Acromegaly KW - Clival tumours KW - Ectopic pituitary adenoma KW - Growth hormone KW - Growth hormone-secreting adenoma KW - Rare tumours SP - 457 EP - 466 JF - Pituitary JO - Pituitary VL - 23 IS - 4 N2 - PURPOSE: Acromegaly is usually due to growth hormone (GH)-secreting pituitary adenomas, but it may be exceptionally caused by GH-secreting ectopic pituitary adenomas (EPA). EPA are defined as extra-sellar pituitary tumours, extra- or intra-cranially sited, entirely separated from the pituitary stalk and gland. The aim of the study is to address the challenges in the management of clival GHEPA. METHODS: We reported a case of a 53-year-old acromegalic patient with a primary clival GHEPA and reviewed systematically the relevant English literature between 1975 and 2019, in keeping with the PRISMA guidelines. RESULTS: Four cases of primary clival GHEPA have been described in literature apart from ours. All patients presented with acromegalic features, elevated circulating GH and/or insulin-like growth factor-1 levels. Hyperprolactinemia and empty sella were described in two cases, respectively. These tumours show the typical imaging characteristics of pituitary adenomas, but their neuroradiological diagnosis may be challenging due to their sizes and the difficulty in defining the absence of connections with the pituitary fossa. CONCLUSION: Although primary clival GHEPA are exceedingly rare, even if likely under-reported in literature, they should be considered in the differential diagnosis of clival tumours because of their specific management. Surgery represents the first-line treatment option, while medical and radiation therapies can be adopted as neo-adjuvant, adjuvant or primary treatments according to tumour and patient characteristics. SN - 1573-7403 UR - https://www.unboundmedicine.com/medline/citation/32504302/Ectopic_GH_secreting_pituitary_adenoma_of_the_clivus:_systematic_literature_review_of_a_challenging_tumour_ L2 - https://doi.org/10.1007/s11102-020-01057-9 DB - PRIME DP - Unbound Medicine ER -