THYMIC CARCINOID WITH ADRENOCORTICOTROPIC HORMONE-PRODUCING ECTOPIC CUSHING SYNDROME AND EMPTY SELLA

Abstract

Objective: Ectopic Cushing's syndrome secondary to thymic carcinoid is a rare disorder that may be difficult to diagnose and manage.

Methods: We describe a case of severe Cushing's syndrome secondary to a large adrenocorticotropic hormone (ACTH) producing thymic carcinoid in a patient with history of primary hyperaldosteronism.

Results: A 43-year-old female with a 20-year history of an aldosterone-secreting adrenocortical adenoma status post right adrenalectomy presented with acute onset of proximal muscle weakness, swelling, facial hirsutism, and severe hypokalemia. Ectopic Cushing's Syndrome was suspected based on the sudden symptom onset and markedly elevated 24-hr urine cortisol and ACTH levels. MRI revealed an empty pituitary sella and a large (7.3 cm) mediastinal mass visible on chest CT. The mass was resected by video-assisted thoracoscopic surgery, resulting in resolution of symptoms and cortisol levels. Pathology assessment confirmed well-differentiated thymic carcinoid with positive ACTH staining.

Conclusion: The case highlights clinical features, challenges in diagnostic work up, treatment modalities, and associated endocrine findings in a thymic carcinoid abutting the heart and presenting with ectopic ACTH secretion.

Keywords: ACTH; EAS; Thymic carcinoid; ectopic Cushing’s syndrome; empty sella; hyperaldosteronism; neuroendocrine carcinoma of thymus.

Figure 1.

Left panel: magnetic resonance imaging (MRI) pituitary with empty sella (red arrow). Right panel: computer tomography (CT) chest with 7.3 cm mediastinal mass (red arrow).

Figure 2.

Left panel: Diffusively positive ACTH staining of tissue from mediastinal mass. Right panel: Positive immunostaining of isolated cell culture. Green: ACTH; blue: DAPI (4',6-diamidino-2-phenylindole).