Endocrine Abstracts

Background: An adrenal incidentaloma (AI) is defined as an adrenal lesion that is discovered when a radiological study is performed for indications other than suspected adrenal disease. All patients with an AI should be evaluated for endocrine adrenal functioning and malignancy potential features. A combination of pheochromocytoma and Cushing’s syndrome in same adrenal gland is extremely rare. We report a case of AI with the evidence of both pheochromocytoma and sub-clinical Cushing’s syndrome.

Case report: A 68 year old female patient presented with an acute respiratory SARS-CoV2 infection. To stratify severity and disease extension, a Computed tomography was performed revealing chest ground-glass opacities, as well as a Right adrenal mass measuring 27×26×25 mm, a spontaneous density of 33UH, Absolute Wash out >50%. Left adrenal gland was normal; Without any clinical symptoms or biological hormonal findings suggesting of a functioning adrenal tumor. On endocrine evaluation a year later, the patient remains asymptomatic, however the laboratory data demonstrated 4 times raised 24-h urinary fractionated metanephrines with non-suppressible serum cortisol after 1 mg dexamethasone suppression test. She underwent right-sided adrenalectomy. The Anatomopathologic report confirmed a pheochromocytoma with a pass score at 3; a proliferative KI index of 5%; staining positive for chromogranin and synaptophysin. The Patient was discharged in good clinical condition.

Discussion: There are few case reports suggesting the different etiologies for this association, namely, pheochromocytoma secreting adrenocorticotropin hormone (ACTH) or its precursors, corticomedullary mixed tumors and focal adrenocortical hyperplasia. Pheochromocytoma may secrete various substances in addition to catecholamines. There are few cases reported of Cushing’s syndrome due to ACTH-secreting pheochromocytoma. In some of them, there was predominance of hyperpigmentation, hypokalemic alkalosis and evidence of contralateral adrenal gland hyperplasia on imaging, but these features were absent in our case. Mixed tumors involving the cortical and medullary components of the adrenal gland are quite rare. On histochemical staining, patient’s tumor was not a corticomedullary mixed tumor, but a pure pheochromocytoma. In some cases adrenocortical hyperplasia resulting from the pheochromocytoma’s paracrine stimuli was the source of autonomous cortisol production. As we do not have plasma ACTH levels, this hypothesis could not be completely ruled out.

Conclusions: The clinical aspects of this case suggest the importance of proper pre-operative recognition of the dual hormone secretion from adrenal mass, which, if goes unnoticed could lead to adrenal or hypertensive crisis in perioperative period.