Ectopic sphenoidal ACTH-secreting adenoma revealed by 11 C Methionine PET scan: case report.

Autor: Lurquin F; Endocrinology and Nutrition Department, Cliniques Universitaires Saint-Luc, n°10, Avenue Hippocrate, 1200, Brussels, Belgium., Constantinescu SM; Endocrinology and Nutrition Department, Cliniques Universitaires Saint-Luc, n°10, Avenue Hippocrate, 1200, Brussels, Belgium. stefan.m.constantinescu@uclouvain.be., Furnica RM; Endocrinology and Nutrition Department, Cliniques Universitaires Saint-Luc, n°10, Avenue Hippocrate, 1200, Brussels, Belgium., Duprez T; Department of Radiology, Cliniques Universitaires Saint Luc, UCLouvain, 1200, Brussels, Belgium., Raftopoulos C; Department of Neurosurgery, Cliniques Universitaires Saint Luc, UCLouvain, 1200, Brussels, Belgium., Daoud L; Department of Pathology, Cliniques Universitaires Saint Luc, UCLouvain, 1200, Brussels, Belgium., Lammens M; Department of Pathology, Cliniques Universitaires Saint Luc, UCLouvain, 1200, Brussels, Belgium.; Department of Pathology, Antwerp University Hospital, University of Antwerp, 2650, Antwerp, Belgium., Maiter D; Endocrinology and Nutrition Department, Cliniques Universitaires Saint-Luc, n°10, Avenue Hippocrate, 1200, Brussels, Belgium.
Jazyk: angličtina
Zdroj: BMC endocrine disorders [BMC Endocr Disord] 2023 Feb 16; Vol. 23 (1), pp. 43. Date of Electronic Publication: 2023 Feb 16.
DOI: 10.1186/s12902-023-01298-2
Abstrakt: Background: Ectopic ACTH pituitary adenomas (EAPA), located outside the sella turcica and deriving from cellular remnants of Rathke's pouch are a very rare cause of Cushing's syndrome (CS). The diagnosis is often difficult and delayed, even after comprehensive work-up. To our knowledge, we report for the first time an ectopic corticotroph tumor of the posterior wall of the sphenoid sinus, leading to false positive results of bilateral inferior petrosal sinus sampling (BIPPS) and which was finally localized by a co-registered 11 C Methionine PET/MR imaging.
Case Presentation: A 48-year-old woman was referred for a high clinical suspicion of ACTH-dependent CS. Biological testing comprising low dose dexamethasone suppression and CRH stimulation tests were indicative of pituitary Cushing's disease, but comprehensive pituitary MRI did not reveal any pituitary adenoma. BIPSS confirmed however a central origin of ACTH secretion (central-to-peripheral ACTH ratio > 100) and revealed a significant right-to-left gradient (6.2), leading to a first right-sided exploratory hypophysectomy, that did not cure the patient. BIPSS images were reviewed and revealed preferential drainage of the left pituitary to the right petrosal sinus, leading us to a left sided exploratory hypophysectomy, which was again unsuccessful. A 11 C Methionine PET/MRI was performed and revealed a hypermetabolic lesion adjacent to the posterior wall of the sphenoidal sinus. After surgical resection, this polypoid mass was identified as an ectopic ATCH-secreting pituitary adenoma expressing ACTH and T-Pit and complete remission of hypercortisolism was observed.
Conclusions: In conclusion, we report a case of ACTH-dependent Cushing's syndrome, caused by an ectopic corticotroph adenoma located in the sphenoidal sinus, which perfectly mimicked the biological features of a classical pituitary ACTH adenoma on a comprehensive hormonal evaluation including BIPPS, and the features of a benign naso-sinusal polyp at MRI. We report for the first time a key role of 11 C Methionine PET co-registered to high resolution MRI for localizing ectopic adenomas, efficiently guiding surgical removal and leading to complete remission of hypercortisolism.
(© 2023. The Author(s).)
Databáze: MEDLINE
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