PECULIARITIES IN THE DIFFERENTIAL DIAGNOSIS OF ADRENOCORTICOTROPIC HORMONE-DEPENDENT CUSHING’S SYNDROME: A CASE REPORT AND LITERATURE REVIEW

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Laboratorinė medicina. 2021,
t. 23,
Nr. 2,
p. 86 -
95

Summary

Background. Adrenocorticotropic hormone-dependent Cushing’s syndrome, that belongs to the group of rare diseases (ORPHA: 99892), is a form of endogenous Cushing’s syndrome caused by abnormal production of andrenocorticotropic hormone: 80% of adrenocorticotropic hormone over-secretion cases are caused by pituitary adenoma, also known as Cushing’s disease, and 20% of cases are caused by ectopic adrenocorticotropic hormone secretion. Cushing’s syndrome is associated with poorer quality of life, higher morbidity and mortality rates compared to the general population. It is important to diagnose this syndrome and its causes as early as possible before chronic excess cortisol causes ir reversible changes.

Materials and methods. A twentythree- year-old clinical case of a patient is presented. Patient was diagnosed with adrenocorticotropic hormone-dependent Cushing’s syndrome, but difficulties arose in determining the etiology of the syndrome because the results of a high-dose dexamethasone suppression test suggested ectopic adrenocorticotropic hormone secretion, but after laboratory, imaging, and endoscopic examinations, the ectopic tumor could not be detected. Unable to completely rule out the adrenocorticotropic hormone secreting pituitary adenoma, pituitary magnetic resonance im aging was performed and a small lesion was found in the anterior- lower pituitary, similar to a microadenoma. The patient’s clinical case was discussed by a medical council and the pituitary adenoma was confirmed that had been removed by trans phenoidal surgery. Histological examination of the surgical material finally confirmed the diagnosis of pituitary adenoma.

Conclusion. Determining the etiology of adrenocorticotropic hormone-dependent Cushing’s syndrome is challenging because of factors such as cyclic and intermittent secretion of adrenocorticotropic hormone from secretory tumors, variable sensitivity and specificity of diagnostic tests, and often small size of the tumor making radiological detection difficult. The best results are achieved through a combination of several diagnostic tests and working in interdisciplinary team – this helps to make a correct diagnosis faster and apply the best treatment method.

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