A rare case of adrenocorticotropic hormone-producing Pheochromocytoma
S. Galan & P. Kann
Introduction: Cushings syndrome caused by adrenocorticotropic hormone-producing pheochromocytoma has been rarely reported. In such cases, the high production of ectopic adrenocorticotropic hormone produced by pheochromocytoma results in bilateral adrenocortical hyperplasia and thus in consecutive Cushings syndrome. We report a case of a 43-year-old patient with a 6 cm right-sided adrenal tumor producing both elevated levels of catecholamines and adrenocorticotropic hormone.
Case: The 43-year-old patient presented with a hypertensive crisis with blood pressure up to 230/160 mmHg, sinus tachycardia and pulmonary oedema. An arterial hypertension was 1 by the patient until the moment of admission. The abdominal ultrasonography and the later computerized tomography showed a 6 cm adrenal tumor with necrotic areas, typical for pheochromocytoma. Urinary catecholamines war extremely increased. The MIBG scan revealed a substantial uptake of the right adrenal tumor without any other pathological findings. The serum and urine cortisol concentrations were normal, but the cortisol was not at all suppressed on the overnight 2 mg and later 8 mg oral dexamethasone suppression test. After proper premedication the right adrenalectomy was performed. By means of histological examination the clinical, laboratory and imaging diagnosis of pheochromocytoma was confirmed. Furthermore, the cytoplasmatic staining revealed a positive reactivity for adrenocorticotropic hormone in about 5% of the cells. The laboratory test results concerning the preclinical Cushings syndrome normalized after performing the right adrenalectomy. The genetic analysis showed no genetic mutations.
Discussion: Adrenocorticotropic hormone-producing pheochromocytomas are rare entities, which should not be overlooked. When investigating an adrenal adenoma, an overproduction of all specific hormones, including of adrenocorticotropic hormone, should therefore be excluded.
Declaration of interest: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project.
Funding: This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.