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

Abstract Mixed corticomedullary tumors (MCMTs) are rare and comprise medullary and cortical cells in a single adrenal tumor. The mechanisms underlying their development have not been fully elucidated. Here, we report a case of MCMT in a 42-year-old woman. Based on the preoperative clinical findings, the patient was diagnosed as having a pheochromocytoma with subclinical Cushing syndrome. Postoperative pathological diagnosis revealed that the tumor demonstrated morphologically distinct medullary and cortical components, which produced catecholamines and cortisol, respectively. Hybrid tumor cells producing both catecholamines and cortisol were not detected. Adrenocorticotropin (ACTH)-positive tumor cells were identified to be present in the pheochromocytoma. This ectopic production of ACTH can contribute to an autonomous cortisol production in a paracrine manner. In addition, micronodules producing aldosterone were detected in the adrenal tissue adjacent to the tumor. The simultaneous development of these 2 lesions may not be correlated with each other; however, this case confirms the importance of a detailed histopathological examination of the adrenal lesions harboring complicated hormonal abnormalities by providing pivotal and indispensable information on their pathogenesis and the possible interaction of the hormones produced in the adrenal gland.

[1]  H. Falhammar,et al.  Ectopic ACTH- and/or CRH-producing pheochromocytomas. , 2020, The Journal of clinical endocrinology and metabolism.

[2]  M. Fujisawa,et al.  Adrenal Corticomedullary Mixed Tumor Associated With the FGFR4-G388R Variant , 2020, Journal of the Endocrine Society.

[3]  F. Beuschlein,et al.  Glucocorticoid Excess in Patients with Pheochromocytoma Compared with Paraganglioma and Other Forms of Hypertension , 2020, The Journal of clinical endocrinology and metabolism.

[4]  Claudia Ramírez-Rentería,et al.  From ACTH-Dependent to ACTH-Independent Cushing's Syndrome from a Malignant Mixed Corticomedullary Adrenal Tumor: Potential Role of Embryonic Stem Cells , 2020, Case reports in endocrinology.

[5]  Alaa Gabi,et al.  Severe Cushing Syndrome Due to an ACTH-Producing Pheochromocytoma: A Case Presentation and Review of the Literature , 2018, Journal of the Endocrine Society.

[6]  Hongting Zheng,et al.  Corticomedullary mixed tumour resembling a small adrenal gland-involvement of cancer stem cells: case report , 2017, BMC Endocrine Disorders.

[7]  H. Falhammar,et al.  Frequency of Cushing’s syndrome due to ACTH-secreting adrenal medullary lesions: a retrospective study over 10 years from a single center , 2016, Endocrine.

[8]  J. Bertherat,et al.  Role of ACTH in the Interactive/Paracrine Regulation of Adrenal Steroid Secretion in Physiological and Pathophysiological Conditions , 2016, Front. Endocrinol..

[9]  R. Kuick,et al.  Aldosterone-stimulating somatic gene mutations are common in normal adrenal glands , 2015, Proceedings of the National Academy of Sciences.

[10]  S. Aubert,et al.  Corticomedullary mixed tumor of the adrenal gland—a clinical and pathological chameleon: case report and review of literature , 2013, Updates in Surgery.

[11]  G. Karayannopoulou,et al.  Mixed corticomedullary adrenal carcinoma , 2013, Surgery Today.

[12]  Y. Homma,et al.  Dopamine‐secreting corticomedullary mixed tumor of the adrenal gland , 2012, International journal of urology : official journal of the Japanese Urological Association.

[13]  V. Livolsi,et al.  Mixed corticomedullary carcinoma of the adrenal gland: a case report. , 2012, Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists.

[14]  Hiroshi Iwasaki,et al.  CD133+ Cancer Stem Cell–like Cells Derived from Uterine Carcinosarcoma (Malignant Mixed Müllerian Tumor) , 2011, Stem cells.

[15]  P. Chu,et al.  Mixed cortical adenoma and composite pheochromocytoma-ganglioneuroma: an unusual corticomedullary tumor of the adrenal gland. , 2011, Annals of diagnostic pathology.

[16]  S. Momosaki,et al.  Case report: coexistence of pheochromocytoma and bilateral aldosterone-producing adenomas in a 36-year-old woman , 2010, Journal of Human Hypertension.

[17]  M. Mokni,et al.  Corticomedullary mixed tumor of the adrenal gland. , 2009, Annales d'endocrinologie.

[18]  H. Sasano,et al.  Coexistence of aldosterone-producing adrenocortical adenoma and pheochromocytoma in an ipsilateral adrenal gland. , 2009, Endocrine journal.

[19]  L. Thompson,et al.  Corticomedullary mixed tumor of the adrenal gland. , 2001, Annals of diagnostic pathology.

[20]  D. A. Mathison,et al.  Cushing's syndrome with hypertensive crisis and mixed adrenal cortical adenoma-pheochromocytoma (corticomedullary adenoma). , 1969, The American journal of medicine.