Mixed corticomedullary adrenal carcinoma

Abstract

Truly mixed corticomedullary tumors (MCMTs) are extremely rare and present as a single tumor mass composed of an intimately admixed population of both adrenal cortical cells and pheochromocytes. The current study describes the first case of a mixed corticomedullary adrenal carcinoma. In addition, we also review the published data on MCMTs to determine their clinical features, biochemical characteristics, pathological findings, and management. In order to compose this review, a search of the international literature for MCMTs was conducted. Fifteen related articles were found. The clinical and pathological information was obtained for all reported cases. MCMTs were found almost exclusively in females. In the vast majority of patients, the symptoms were related to the tumor's hormone hypersecretion. Hypertension and diabetes were present in 80 and 40 % of cases, respectively. Cushing's syndrome was reported in eight cases (53.33 %). A final diagnosis was made in all cases after surgery based on the pathological results. As of the writing of this article, all published cases of MCMTs had benign clinical behavior, with no instances of metastasis or death due to the tumor. MCMTs are currently considered to be benign tumors. Ours is the first case of malignant MCMT reported in the literature. The potential for malignancy should therefore be considered for these tumors.

Adrenal Cushing syndrome with detectable ACTH from an unexpected source

“…Cortisol and catecholamines also normalised postoperatively. Similar to our patient, in seven of eight published cases, patients presented with a detectable serum ACTH, despite a hypercortisolaemic state from an adrenal source, in which ACTH is usually suppressed 2–4 6–7 10 15. The detectable ACTH level is not grossly elevated, as is usually seen from a pituitary or ectopic source 16.…”

“…The majority (71%) of patients had elevated cortisol, 12% had elevated aldosterone and 59% had elevated catecholamines. Of the 12 previous cases with elevated cortisol, 8 report ACTH levels and the vast majority (7/8, 88%) had unsuppressed ACTH levels, despite a hypercortisolaemic state 2–4 6–8 10 15…”

Adrenal cortical and chromaffin stem cells: Is there a common progeny related to stress adaptation?

“…Supported by the existence of cortico-chromaffin hybrid cells (Bornstein et al, 1991) and mixed corticomedullary tumors (Michalopoulos et al, 2013), we here challenge the dogma of a strictly separated progeny of the two systems. Because of the rarity of mixed adrenal tumors and thereby limited amount of literature describing this, it is still not possible to say if they derive from a common precursor and further investigation will be needed in order to solve this question.…”

“…However, little is known about interactions between progenitors in the adrenal cortex and medulla, or if to some degree there is a progeny capable of bipotent fates generating both cortical as well as medullary cell types. Alluding to this notion, an extremely rare form of corticomedullary adrenal tumors are characterized by a single mass composed of mixed cell populations with attributes characteristic of both cortical and medullary origin (Michalopoulos et al, 2013). One hypothesis for their origin proposed by Lee et al suggests a genetic mutation in an adrenal stem cell giving rise to cells with this mixed character (Lee et al, 2008).…”

Adrenocorticotropic hormone-producing pheochromocytoma: analysis of clinical cases

“…Микст-опухоли -это смешанные новообразования, содержащие элементы двух и более опухолей различного эмбрионального происхождения. Микст-опухоли надпочечников встречаются крайне редко -в литературе на сегодняшний день описано 16 случаев, и все были выявлены у женщин [24].…”