DOI: 10.36347/sjmcr.2022.v10i02.015

Pages: 106-110

The most unusual cause of Cushing's syndrome (< 0.5% of cases) is a pheochromocytoma due to ectopic secretion of ACTH. Approximately 80% of cases are related to inappropriate corticotropin (ACTH) secretion and most of these patients have a pituitary adenoma (Cushing's disease). This is mostly a pulmonary or thymic carcinoid tumour, a pancreatic neuroendocrine tumour or a small cell lung cancer. 66 year old patient with a 20 years history of cannabis consumption, weaned 3 years ago. Admitted for etiological evaluation of a right adrenal mass, examination revealed abdominal pain over the last 6 months, intense in the right flank without any pressure sensation within a major weight loss context, asthenia and anorexia associated with generalized, disabling, and insominating skin pruritus; no Menard's Triad, no cramps or paresthesia. Pelvic abdominal CT scan: a 70×32 mm adrenal mass in contact with the inferior vena cava, segment I of the liver and the upper pole of the kidney with loss of the fatty border, it encircles the renal pedicle which remains permeable, associated with pre-aortic and aorto-caval adenopathies. Endocrine assessment: ACTH-dependant cushing syndrome. Management: due to the highly invasive nature of the tumour the patient benefited from a biopsy of the mass as well as a skin biopsy associated with heparinotherapy management of the partial thrombosis of the vena cava. Pathological and immunohistochemical examination of the mass biopsy: compatible with a pheochromocytoma with a score of Pass = 10, probably aggressive pheochromocytoma.