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Cushing's syndrome associated with a nested stromal epithelial tumor of the liver: hormonal, immunohistochemical, and molecular studies

¹ Unité Fonctionnelle d'Endocrinologie et Maladies Métaboliques, Department of Medicine, CHU Côte de Nacre, 14033 Caen Cedex, France² Laboratoire de Génétique Moléculaire, CHR Clémenceau, 14033 Caen Cedex, France³ Département de Chirurgie Digestive et Transplantation Hépatique, ⁴ Département d'Anatomopathologie, CHU Côte de Nacre, 14033 Caen Cedex, France⁵ EA 2608-USC INRA, Université de Caen, 14032 Caen Cedex, France⁶ Laboratoire de Neuroendocrinologie Moléculaire et Cellulaire, INSERM, U-413, Unité affilieé au Centre National de la Recherche Scientifique, Université de Rouen, 76821 Mont St Aignan, France

(Correspondence should be addressed to Y Reznik at Unité Fonctionnelle d'Endocrinologie et Maladies Métaboliques, Department of Medicine, CHU Côte de Nacre; Email: reznik-y{at}chu-caen.fr)

(A Rod and M Voicu contributed equally to this work)

Abstract

Context: Ectopic ACTH syndrome (EAS) is principally associated with aggressive malignant tumors but also with neuroendocrine tumors of good prognosis. Recently, rare nonhepatocytic nested stromal and epithelial tumors (NSET) were characterized by their possible association with Cushing's syndrome of which biochemical and physiopathological features were still incompletely studied.

Objective: To describe the clinical and hormonal characteristics of an EAS originating from a liver NSET and further understand the mechanism of cortisol overproduction.

Design and setting: This is a clinical case report from the Endocrinology Department of Caen University Hospital, France.

Patient and intervention: A 17-year-old female patient was found to have a large liver NSET with mild Cushingoid clinical features and intense biological hypercortisolism but moderate ACTH secretion. Resection of the tumor was curative with a 30-month follow-up.

Results: The epithelial component of the tumor coexpressed ACTH mildly, corticotropin-releasing hormone (CRH) strongly, and 11β-hydroxysteroid dehydrogenase at a level comparable with normal human hepatocytes.

Conclusions: Liver NSET is a new cause of EAS, which may evoke hypercortisolism by multiple biochemical pathways.