Adrenal Cushing syndrome with detectable ACTH from an unexpected source

Lwin, Thinzar M.; Galal, Nehal; Gera, Shweta; Marti, Jennifer L.

doi:10.1136/bcr-2016-216965

Cited by 8 publications

(10 citation statements)

References 16 publications

(13 reference statements)

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“…Four cases were found in other languages (Chinese, Japanese, Korean, and Russian); these were not included in our study [ 54–57 ]. Other mechanisms for the development of ectopic Cushing syndrome from a pheochromocytoma have been reported, such as corticotropin-releasing hormone secretion [ 58–60 ] or mixed corticomedullary tumors [ 61 , 62 ]. Other cases have been reported as part of the MEN 2A syndrome with bilateral masses [ 63 , 64 ].…”

Section: Discussionmentioning

confidence: 99%

Severe Cushing Syndrome Due to an ACTH-Producing Pheochromocytoma: A Case Presentation and Review of the Literature

Gabi

Milhem

Tovar

et al. 2018

Journal of the Endocrine Society

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Adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome is rarely caused by a pheochromocytoma. We present a case of a 46-year-old woman who developed severe hypertension, hypokalemia, and typical Cushingoid features. Investigations revealed extremely high metanephrine, cortisol, and ACTH levels. Imaging showed a 3.8-cm left adrenal mass. Preoperative control of hypertension and hypokalemia was very challenging. The patient was cured after surgical removal of the adrenal mass. We followed this by a review of the literature using the databases Google Scholar and PubMed. A total of 58 cases have been reported to date. In summary, ACTH-producing pheochromocytoma is a rare condition that poses a clinical challenge in the perioperative period. It is important that physicians be aware of such a condition because early recognition and treatment are crucial to decrease morbidity and mortality.

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Section: Discussionmentioning

confidence: 99%

Severe Cushing Syndrome Due to an ACTH-Producing Pheochromocytoma: A Case Presentation and Review of the Literature

Gabi

Milhem

Tovar

et al. 2018

Journal of the Endocrine Society

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“…As conceptualized in an old study, steroidogenic precursors (21-deoxycortisol) secreted by ACC may antagonize the glucocorticoid feedback at the hypothalmic-pituitary level [36,37]. Although the intra-adrenal source of ACTH has been reported in PBMAH and mixed cortico-medullary adrenal tumors (MCMT), the evidence to support the production of ACTH or ACTH-like substances from ACC is negligible [38,39]. The evaluation for the possible role of heterophile antibodies was not performed in our study.…”

Section: Discussionmentioning

confidence: 83%

Clinical Spectrum of Adrenal Cushing’s Syndrome and the Caution for Interpretation of Adrenocorticotrophic Hormone: A Single-Center Experience

Gosavi¹,

Lila²,

Memon³

et al. 2022

Horm Metab Res

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To describe the differences in presentation, biochemistry, and radiological evaluation of various etiologies of adrenal Cushing’s syndrome (CS) from a single center. To emphasize caution for interpretation of plasma adrenocorticotropic hormone (ACTH), as a spuriously unsuppressed ACTH level by immunometric assay may lead to therapeutic misadventures in adrenal CS. Design: Retrospective, single-center, observational study. Methods: Fifty-eight adrenal CS patients [Adrenocortical carcinoma (ACC), n=30; Adenoma (ACA), n=15; Primary pigmented nodular adrenocortical disease (PPNAD), n=10; ACTH independent macronodular adrenal hyperplasia (AIMAH), n=3) evaluated at a tertiary care center in western India between January 2006 to March 2020 were included. Data on demography, clinical evaluation, biochemistry, imaging, management, histopathology, and outcome were recorded in a standard format and analyzed. Results: Cortisol secreting ACC presented at 38(1–50) years with abdominal mass in 26/30 (86.7%) and 16/30 (53.3%) had metastases at presentation. ACA with autonomous cortisol excess presented at 25(4.9–40) years with discriminating features of CS in 14/15 (93.3%), sex steroid production in 2/15, unenhanced HU <10 in only one, and relative washout >40% in 8/11 (72.7%). One ACA and eight ACC patients had plasma ACTH (by Siemens Immulite assay) > 20 pg/ml, despite hypercortisolemic state. Conclusions: Cortisol-secreting ACC and ACA most often present with mass effects and florid CS, respectively. Baseline HU has low sensitivity to differentiate cortisol-secreting ACA from ACC. Plasma ACTH measured by Seimens Immulite is often unsuppressed, especially in ACC patients, which can be addressed by measuring ACTH by more accurate assays.

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“…Further, adrenocortical cells produced cortisol in most cases and aldosterone in two cases, and only one case produced dehydroepiandrosterone sulfate (DHEA-sulfate). Seven of 12 previous cases with elevated cortisol had unsuppressed ACTH levels, despite a hypercortisolemic state (19). The detectable ACTH level was not grossly elevated, as is usually seen from pituitary or ectopic sources (19).…”

Section: Clinical Manifestations and Laboratory Findingsmentioning

confidence: 83%

“…The detectable ACTH level was not grossly elevated, as is usually seen from pituitary or ectopic sources (19). We added six subsequent cases from the reports after Lwin et al (19) and found that 71% (10/14) of MCMTs had subclinical Cushing syndrome with ectopic ACTH. In cases with Cushing syndrome, ectopic ACTH syndrome was defined as ACTH dependent when the plasma ACTH level was >15 pg/ml (reference range: 10-50 pg/ml) and ACTH independent when the plasma ACTH level was <5 pg/ml based on a previous report (23).…”

Section: Clinical Manifestations and Laboratory Findingsmentioning

confidence: 93%

“…Seven of 12 previous cases with elevated cortisol had unsuppressed ACTH levels, despite a hypercortisolemic state (19). The detectable ACTH level was not grossly elevated, as is usually seen from pituitary or ectopic sources (19). We added six subsequent cases from the reports after Lwin et al (19) and found that 71% (10/14) of MCMTs had subclinical Cushing syndrome with ectopic ACTH.…”

Section: Clinical Manifestations and Laboratory Findingsmentioning

confidence: 98%

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Mixed corticomedullary tumor of the adrenal gland

et al. 2022

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Mixed corticomedullary tumor (MCMT) of the adrenal gland is an extremely rare tumor characterized by an admixture of steroidogenic cells and chromaffin cells in a single tumor mass simultaneously producing adrenocortical hormones and catecholamines; it is associated with ectopic adrenocorticotropic hormone (ACTH) in some cases. We reviewed and summarized clinicopathological data of 28 MCMTs, including four metastatic tumors in 26 previous reports. These reports included 21 females and 7 males, and the average tumor sizes were 4.8 ± 2.5 cm and 12.6 ± 6.4 cm in the non-metastatic and metastatic groups, respectively (P<0.001). The clinical manifestations and laboratory data were as follows: Cushing or subclinical Cushing syndrome, 58% (14/24); hypertension, 71% (17/24); elevated adrenocortical hormones, 75% (18/24); elevated catecholamines, 75% (18/24); and ectopic ACTH, 71% (10/14). All four patients with metastatic MCMTs had poor prognoses and elevated adrenocortical hormone levels; however, only two patients had elevated catecholamine levels. Immunohistochemistry was essential for the pathologic diagnosis of MCMTs. In this study, using an improved technique, we detected ectopic ACTH-producing cells in the same paraffin-embedded sections reported to be negative in our previous reports. As MCMT is composed of cells with embryologically different origins, its pathogenesis has been explained by various hypotheses. We compared MCMT to the adrenal gland of birds and the early stage of human fetuses, in which nests of chromaffin cells and steroidogenic cells admix without the formation of cortex and medulla. MCMT is characterized by the immaturity of organogenesis and might be classified as an adrenal embryonal tumor.

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Adrenal Cushing syndrome with detectable ACTH from an unexpected source

Cited by 8 publications

References 16 publications

Severe Cushing Syndrome Due to an ACTH-Producing Pheochromocytoma: A Case Presentation and Review of the Literature

Severe Cushing Syndrome Due to an ACTH-Producing Pheochromocytoma: A Case Presentation and Review of the Literature

Clinical Spectrum of Adrenal Cushing’s Syndrome and the Caution for Interpretation of Adrenocorticotrophic Hormone: A Single-Center Experience

Mixed corticomedullary tumor of the adrenal gland

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