A unique case of a benign adrenocortical tumor with triple secretion of cortisol, androgens, and aldosterone. Development of multiple sclerosis after surgical removal of the tumor

Abstract

We present a 39-year old female with a benign adrenal tumor characterized by autonomous secretion of cortisol, androgens, and aldosterone. The patient presented with a 4-year history of hypertension and severe hirsutism. Baseline investigations revealed elevated testosterone, androstendione, and 17OH progesterone with normal levels of dehydroepi androsterone sulfate. CT of the adrenals revealed a 2.5x3.0 cm tumor with characteristics of an adenoma on the left adrenal gland. Pelvic ultrasound was normal. Further investigations revealed suppressed basal ACTH levels, loss of diurnal rhythm of cortisol, and failure to suppress on low dose dexamethasone suppression test, suggesting autonomous cortisol secretion by the tumor. She had an exaggerated response of 17OH progesterone to ACTH, implying reduced 21-hydroxylase activity. An elevated plasma aldosterone concentration to plasma renin activity ratio was suggestive of hyperaldosteronism, which was confirmed by failure of aldosterone to suppress to a formal saline infusion test. Complete clinical and biochemical remission of the disease was observed after left adrenalectomy. Histology confirmed the presence of an adrenocortical adenoma. The patient developed multiple sclerosis 6 months after the operation. The flare-up of an autoimmune disease (multiple sclerosis) postoperatively could be coincidental or possibly related to the high normalization of the high cortisol levels acting as a precipitating factor.

Emergence of De Novo Conditions Following Remission of Cushing Syndrome: A Case Report and Scoping Review

“…We identified 11 cases of neurological disorders, including idiopathic intracranial hypertension (IIH) ( n = 5) [ 15 , 16 , 41 , 42 , 43 ], multiple sclerosis ( n = 2) [ 44 , 45 ], lymphocytic hypophysitis ( n = 2) [ 46 , 47 ], acute disseminated encephalomyelitis ( n = 1) [ 48 ] and myasthenia gravis ( n = 1) [ 49 ]. IIH has been associated with both primary adrenal insufficiency and steroid withdrawal [ 15 ].…”

A Large Benign Adrenocortical Adenoma Cosecreting Testosterone and Cortisol

“…However, Kamenicky et al showed that even benign cortisol-secreting adenomas are able to secrete normal to low normal levels of androgens which typically do not cause virilization ( 11 ). There are rare case reports of adenomas that cause both Cushing disease and virilization ( 13-18 ).…”

A pedunculated aldosterone‐producing adenoma drained by an extra vein causing puzzling results of adrenal vein sampling

“…This tumour, in contrast to our case, also produced cortisol. 11 Immunohistochemical investigation revealed that the adenoma stained positive not only for CYP11B2 but also for CYP11B1. This costaining has been found in only 6% of patients with PA caused by ATPase or CACNA1D mutations compared to 49% and 45% in patients with a KCNJ5 mutant or wild-type tumour, respectively.…”