Corticomedullary mixed tumour resembling a small adrenal gland-involvement of cancer stem cells: case report

Abstract

BackgroundAdrenal corticomedullary mixed tumours are very rare. Its mechanism is rarely reported. Here we report the first case of a corticomedullary mixed tumour resembling a “small adrenal gland” with distinct arrangement of the cortical and medullary layers. We further hypothesize regarding the tumorigenic mechanism of this tumour.Case presentationA 58-year man had been diagnosed with diabetes and hypertension for 3 years. His 24-h urine vanillylmandelic acid (VMA) levels were slightly elevated. An abnormal circadian cortisol rhythm was noted, and his cortisol levels were not suppressed by dexamethasone. Abdominal computed tomography (CT) revealed a right adrenal gland lesion (diameter, 30 × 38 mm), while an enhanced CT showed enhancement and hypervascularization. The tumour was positive for adrenocorticotropic hormone, chromogranin A (CGA), and steroidogenic factor-1 (SF-1) on the tumour surface. Acetaldehyde dehydrogenase 1(ALDH1), CD44, CD133, Nestin, Nerve growth factor receptor (NGFR), and Sex determining region y-box 9(SOX9) staining were positive. Although administration of medications for diabetes and hypertension was stopped until surgery was performed, the blood sugar level and blood pressure were maintained after surgery.ConclusionsThis is the first report about a possible mechanism by which cancer stem cells induce adrenal corticomedullary tumours.

Adrenal Corticomedullary Mixed Tumor Associated With the FGFR4-G388R Variant

“…Furthermore, during pregnancy, the placenta is a source of ACTH and CRH, which exaggerates the circulating ACTH levels synergistically with ACTH induced by hyper catecholamine and may result in the proliferation of the cortical components within the pheochromocytomas. Another hypothesis is that tumor stem cells may contribute to the development of these 2 distinct tumor cells [ 5 ]. In the current study, whole exome analysis revealed the FGFR4 -G388R variant as a possible pathogenic gene alteration, and p-S727 STAT3, a downstream signal of the FGFR4 -G388R variant, was observed mainly in the adrenocortical adenoma component, suggesting that the FGFR4 -G388R variant and downstream signaling may play a role in the development of adrenocortical adenoma components.…”

From ACTH-Dependent to ACTH-Independent Cushing’s Syndrome from a Malignant Mixed Corticomedullary Adrenal Tumor: Potential Role of Embryonic Stem Cells

“…Mixed corticomedullary adrenal tumors (MCATs) are very rare and present as a single heterogeneous mass composed of intimately admixed adrenocortical and medullary cells [1][2][3][4][5][6][7][8][9]. e medullary component usually consists of pheochromocytes, whereas the cortical component is usually an adrenocortical adenoma or less frequently an adrenocortical carcinoma [1][2][3][4][5][6][7][8][9]. Clinical and/or biochemical evidence of cortisol hypersecretion, putatively by the cortical component, has been documented in about half of the reported cases of mixed corticomedullary adrenal tumors [1][2][3][4][5][6][7][8][9].…”

“…e medullary component usually consists of pheochromocytes, whereas the cortical component is usually an adrenocortical adenoma or less frequently an adrenocortical carcinoma [1][2][3][4][5][6][7][8][9]. Clinical and/or biochemical evidence of cortisol hypersecretion, putatively by the cortical component, has been documented in about half of the reported cases of mixed corticomedullary adrenal tumors [1][2][3][4][5][6][7][8][9]. ese tumors are especially rare in young patients.…”

“…ese tumors are especially rare in young patients. On the contrary, catecholamine hypersecretion by the pheochromocytoma component appears to be less frequent [1][2][3][4][5][6][7][8][9]. A significant proportion of MCAT is clinically and biochemically nonfunctioning lesions.…”

Mixed Corticomedullary Tumor Accompanied by Unilateral Aldosterone-Producing Adrenocortical Micronodules: A Case Report

“…Mixed corticomedullary tumors (MCMTs) are composed of medullary and cortical cells in a single adrenal tumor [ 1 ], which can produce catecholamines, cortisol, or both [ 2 ]. MCMTs are extremely rare because the adrenal medulla and cortex have different embryological origins, namely, the neuroectoderm and mesoderm, respectively.…”