Difficult Diagnosis and Management of Concealed Cushing

Title:Difficult Diagnosis and Management of Concealed Cushing

Volume: 24

Author(s): Antonio Stefano Salcuni*, Francesca Marchese, Miriam Cellini, Alessandro Brunetti, Elda Kara, Jacopo Manso, Veronica Tonelli, Claudia Cipri, Maria Carpentieri, Silvia Maria Sciannimanico, Silvia Galasso, Sandra Agus and Fabio Vescini

Affiliation:

• Department of Endocrinologia, SOC Endocrinologia, Ospedale Santa Maria della Misericordia, Udine, Italy

Abstract: Background: Adrenocorticotropic Hormone (ACTH)-secreting tumors account for 5- 10% of Cushing syndrome cases and are often difficult to diagnose and treat.

Case Report: A 44-year-old man presented with arterial hypertension and weight gain. On the physical examination, he exhibited central obesity, abdominal striae rubrae, and facial plethora. Due to the clinical suspicion of Cushing syndrome, the Nugent test and Liddle-1 test were performed, which showed a lack of cortisol suppression. ACTH levels were also high (138 pg/mL), so pituitary MRI and dynamic tests were performed, including the Corticotropin-releasing Hormone (CRH) stimulation test and Liddle-2. MRI showed a 3 mm pituitary microadenoma, but hormonal testing suggested ectopic ACTH production. Chest CT detected a 10-mm nodule in the upper lobe of the right lung, suspicious for a carcinoid tumor. However, the nodule did not exhibit any enhancement on 68-Gallium-DOTATOC PET-CT, and further, 18-FDG PET-CT was inconclusive. In addition, the nodule was deemed non-biopsiable due to its location. Meanwhile, the patient developed osteoporosis, resulting in two vertebral fractures and one rib fracture, which was treated with zoledronate. Furthermore, the patient developed acute aortic insufficiency. During bioprosthetic valve replacement, the thoracic surgeon performed wedge resection of the right upper lung lobe. The histological examination of the lesion revealed a typical lung carcinoid (1.2x0.9 cm, pT1bNXR0, Ki671%, ACTH positive in 95% of neoplastic elements). ACTH levels dropped to 4 pg/mL on the fourth postoperative day.

Conclusion: ACTH-secreting tumors are particularly challenging diseases. A comprehensive hormonal and instrumental valuation is often required, necessitating a multidisciplinary approach.

Cite this article as:

Salcuni Antonio Stefano*, Marchese Francesca, Cellini Miriam, Brunetti Alessandro, Kara Elda, Manso Jacopo, Tonelli Veronica, Cipri Claudia, Carpentieri Maria, Sciannimanico Silvia Maria, Galasso Silvia, Agus Sandra and Vescini Fabio, Difficult Diagnosis and Management of Concealed Cushing, Endocrine, Metabolic & Immune Disorders - Drug Targets 2024; 24 () . https://dx.doi.org/10.2174/0118715303322153240513103529