title: From benign adrenal incidentaloma to adrenocortical carcinoma: an exceptional random event.
abstract: New European guidelines for the management of adrenal incidentalomas were recently released. One of the most novel recommendations is to stop following patients when they present a typical, small and non-secreting adenoma. We report here the case of a 71-year-old man with such an adenoma, who developed an adrenocortical carcinoma (ACC) fourteen years later, with subsequent metastases and death. Clinically, he had a normal blood pressure and no sign of hormonal hypersecretion. The hormonal work-up showed no hormone excess: urinary free cortisol level was normal, the diurnal cortisol rhythm was respected and urinary catecholamine metabolites levels were normal. Computed tomography (CT) scan showed a homogeneous lesion, with a low density. The lesion remained unchanged during the five years of follow-up. Eight years after the last CT, a large right heterogeneous adrenal mass was incidentally discovered during an ultrasound examination. On CT scan, it was a 6 cm heterogeneous tumor. On hormonal work-up, there was no secretion. The patient was operated of an adrenalectomy, and the histology described an ACC with a Weiss score at 8, with no benign contingent. To our knowledge, this is the first case of an ACC occurring in a patient with prior adrenal imaging showing a typical benign adenoma.
authors.affiliation: Department of EndocrinologyCenter for Rare Adrenal Diseases, Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Paris, France.
authors.forename: I
authors.initials: I
authors.lastname: Belmihoub
authors.affiliation: Department of RadiologySaint Joseph Hospital, Paris, France.
authors.forename: S
authors.initials: S
authors.lastname: Silvera
authors.affiliation: Departments of Pathology.
authors.forename: M
authors.initials: M
authors.lastname: Sibony
authors.affiliation: Departments of Digestive and Endocrine Surgery.
authors.forename: B
authors.initials: B
authors.lastname: Dousset
authors.affiliation: Departments of Radiology AHôpital Cochin, Assistance Publique Hôpitaux de Paris, Paris, France.
authors.forename: P
authors.initials: P
authors.lastname: Legmann
authors.affiliation: Department of EndocrinologyCenter for Rare Adrenal Diseases, Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Paris, France.
authors.forename: X
authors.initials: X
authors.lastname: Bertagna
authors.affiliation: Department of EndocrinologyCenter for Rare Adrenal Diseases, Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Paris, France.|Institut CochinInstitut National de la Santé et de la Recherche Médicale U1016, Centre National de la Recherche Scientifique UMR8104, Université Paris Descartes, Sorbonne Paris Cité, Paris, France.
authors.forename: J
authors.initials: J
authors.lastname: Bertherat
authors.affiliation: Department of EndocrinologyCenter for Rare Adrenal Diseases, Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Paris, France.|Institut CochinInstitut National de la Santé et de la Recherche Médicale U1016, Centre National de la Recherche Scientifique UMR8104, Université Paris Descartes, Sorbonne Paris Cité, Paris, France.
authors.forename: G
authors.initials: G
authors.lastname: Assié
mesh_terms: D000236:Adenoma / Q000000981:diagnostic imaging* / Q000209:etiology / Q000473:pathology; D000310:Adrenal Gland Neoplasms / Q000000981:diagnostic imaging* / Q000209:etiology / Q000473:pathology; D000311:Adrenal Glands / Q000000981:diagnostic imaging* / Q000473:pathology / Q000601:surgery; D000315:Adrenalectomy; D018268:Adrenocortical Carcinoma / Q000000981:diagnostic imaging* / Q000209:etiology / Q000473:pathology / Q000601:surgery; D000368:Aged; D005060:Europe; D017809:Fatal Outcome; D005602:France; D006801:Humans; D033162:Incidental Findings; D008297:Male; D009190:Myelodysplastic Syndromes / Q000503:physiopathology*; D060787:Neoplasm Grading; D009367:Neoplasm Staging; D017410:Practice Guidelines as Topic; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography