Concurrent pheochromocytoma and cortical carcinoma of the adrenal gland

Adrenocortical carcinoma and succinate dehydrogenase gene mutations: an observational case series

“…However, considering the rarity of ACC as well as SDHx mutations in the population, the co-occurrence in our cases argues for a true association rather than a chance event. It should be noted, that in addition to our observations, one single case report in 2011 described both ACC and pheochromocytoma in a patient with no known pathogenic germline mutations in SDHB, SDHC, or SDHD (17); however, germline SDHA testing and genetic tumor testing were not conducted. It is important to note, that the absolute risk increase for ACC in SDHx mutation gene carriers does not reach a level that would justify targeted ACC surveillance in SDHx mutation carriers; however, ACC should be a considered as a potential rare manifestation of SDHx mutations, and the role of SDHx mutations in the pathogenesis of ACC should be further investigated.…”

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

“…Mixed corticomedullary tumors are extremely rare, including the present one, and there are only 26 cases reported in the English literature since its initial description in 1969 by Mathison et al [16]. However, the medullary component has usually been reported as a benign pheochromocytoma [17], and the adrenocortical component can be either an adenoma [1-3, 6, 7, 9] or a carcinoma [4,5,8]. ere have been only 3 previously reported cases of malignant, mixed adrenocortical tumors [4,5,8].…”

“…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.…”