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[Fulminant hypercorticism due to ACTG producing pheochromocytoma].
- Source :
-
Problemy endokrinologii [Probl Endokrinol (Mosk)] 2023 Nov 02; Vol. 70 (3), pp. 55-66. Date of Electronic Publication: 2023 Nov 02. - Publication Year :
- 2023
-
Abstract
- Endogenous hypercorticism (EH) is a severe symptom complex caused by hypercortisolemia; according to the etiology, ACTH-dependent and ACTH-independent variants are distinguished, which, according to the literature, occur in 70-80% and 20-30% of cases, respectively. A rare cause of ACTH-dependent endogenous hypercorticism is ACTH-ectopic syndrome (ACTH-ES) (about 15-20% of cases). ACTH-ES is a syndrome of adrenocorticotropic hormone (ACTH) hyperproduction by neuroendocrine tumors of extrahypophyseal origin. Various tumors can secrete ACTH: bronchopulmonary carcinoid, small cell lung cancer, less frequently, thymus carcinoid, islet cell tumors and pancreatic carcinoid, medullary thyroid cancer, carcinoid tumors of the intestine, ovaries, as well as pheochromocytoma (PCC).This publication presents a clinical case of rarely detected paraneoplastic ACTH production by pheochromocytoma. The patient had clinical manifestations of hypercorticism, therefore, she applied to the Russian National Research Center of Endocrinology of the Ministry of Health of Russia. During the examination Cushing's syndrome (CS) was confirmed, multispiral computed tomography (MSCT) of the abdominal cavity revealed a voluminous formation of the left adrenal gland. Additional examination recorded a multiple increase in urinary catecholamine levels. Subsequently, the patient underwent left-sided adrenalectomy. The diagnosis of pheochromocytoma was confirmed morphologically, immunohistochemical study demonstrated intensive expression of chromogranin A and ACTH by tumor cells.
- Subjects :
- Humans
Female
Cushing Syndrome etiology
Cushing Syndrome pathology
Adrenocorticotropic Hormone blood
Adrenocorticotropic Hormone metabolism
Adult
ACTH Syndrome, Ectopic etiology
ACTH Syndrome, Ectopic diagnosis
ACTH Syndrome, Ectopic pathology
Middle Aged
Pheochromocytoma complications
Pheochromocytoma pathology
Pheochromocytoma metabolism
Adrenal Gland Neoplasms complications
Adrenal Gland Neoplasms pathology
Adrenal Gland Neoplasms metabolism
Subjects
Details
- Language :
- Russian
- ISSN :
- 2308-1430
- Volume :
- 70
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- Problemy endokrinologii
- Publication Type :
- Academic Journal
- Accession number :
- 39069773
- Full Text :
- https://doi.org/10.14341/probl13374