Adrenocortical carcinoma masquerading as Cushing's disease

Jarial, Kush Dev Singh; Walia, Rama; Kumar, Santosh; Bhansali, Anil

doi:10.1136/bcr-2016-217519

Cited by 4 publications

(4 citation statements)

References 9 publications

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“…Adrenal CS cases reported in the literature (ODST serum cortisol value > 5 μg/dl and/or 24-hour UFC more than the upper limit of normal) with unsuppressed ACTH are summarized in ▶ Table 3. Unsuppressed ACTH in adrenal CS may prompt unnecessary inves- tigations like MRI pituitary, IPSS, CT thorax and neck, and/or nuclear scans, and in unfortunate instances, even an unnecessary pituitary surgery [7][8][9]. Unsuppressed ACTH has been reported mostly with ACA, whereas in our series, we observed eight patients of ACC had plasma ACTH > 20 pg/ml.…”

Section: Discussioncontrasting

confidence: 46%

“…Inappropriate sample collection and storage conditions may lead to falsely low ACTH levels due to degradation of this labile analyte, which may erroneously suggest an ACTH-independent CS. On the other hand, adrenal CS may be misdiagnosed as ACTH-dependent by an unsuppressed plasma ACTH, as reported in a few case reports/series [7][8][9][10]. Here, we describe our experience of adrenal CS from a single center, emphasizing caution for interpretation of ACTH.…”

Section: Introductionmentioning

confidence: 78%

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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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Section: Discussioncontrasting

confidence: 46%

Section: Introductionmentioning

confidence: 78%

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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“…Because of decreased glucocorticoid negative feedback effects, plasma ACTH levels will be inappropriately normal or elevated (generally > 20 pg/mL) in ACTH-dependent causes and low (generally < 10 pg/mL) in ACTH-independent causes of CS (70,71). Thirty percent of the patients with CS have ACTH levels in the "gray zone" (5-20 pg/mL) and should have repeat testing and consideration of adrenal imaging to detect possible adrenal pathology (72). ACTH-dependent CS comprises 80% to 85% of all CS cases.…”

Section: Acth-dependent Csmentioning

confidence: 99%

Approach to the Patient: Diagnosis of Cushing Syndrome

Savas

Mehta

Agrawal

et al. 2022

The Journal of Clinical Endocrinology &Amp; Metabolism

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Cushing’s syndrome results from supraphysiological exposure to glucocorticoids and is associated with significant morbidity and mortality. The pathogenesis includes administration of corticosteroids (exogenous Cushing’s syndrome) or autonomous cortisol overproduction, whether or not adrenocorticotropin hormone (ACTH) dependent (endogenous Cushing’s syndrome). An early diagnosis of Cushing’s syndrome is warranted, however, in clinical practice very challenging partly due to resemblance with other common conditions (i.e. pseudo-Cushing’s syndrome). Initial workup should start with excluding local and systemic corticosteroid use. First-line screening tests including the 1-mg dexamethasone suppression test, 24-hour urinary free cortisol excretion, and late-night salivary cortisol measurement should be performed to screen for endogenous Cushing’s syndrome. Scalp-hair cortisol/cortisone analysis helps in the assessment of long-term glucocorticoid exposure as well as in detection of transient periods of hypercortisolism as observed in cyclical Cushing’s syndrome. Interpretation of results can be difficult due to individual patient characteristics and hence requires awareness of test limitations. Once endogenous Cushing’s syndrome is established, measurement of plasma ACTH concentrations differentiates between ACTH-dependent (80-85%) or ACTH-independent (15-20%) causes. Further assessment with different imaging modalities and dynamic biochemical testing including bilateral inferior petrosal sinus sampling helps further pinpoint the cause of Cushing’s syndrome. In this issue of ‘Approach to the patient’ the diagnostic workup of Cushing’s syndrome is discussed with answering the questions when to screen, how to screen and how to differentiate the different causes. In this respect, latest developments in biochemical and imaging techniques are discussed as well.

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“…While the unavailability of corticotrophin-releasing hormone in many countries mandates pituitary or adrenal imaging to explore the underlying condition in this scenario, the presence of pituitary incidentalomas poses a risk of wrong localisation. [ 2 ] Considering the potential role of DHEAS in reflecting plasma ACTH levels over a longer period, the present study aimed to explore the performance of DHEAS to distinguish adrenal adenomas with ‘grey zone’ ACTH levels from ACTH-dependent CS.…”

mentioning

confidence: 99%

Low DHEAS Level: A Surrogate Marker of Adrenal Cushing Syndrome with Grey Zone ACTH Levels

Dhar,

Banerjee,

Bhattacharjee

et al. 2023

Indian Journal of Endocrinology and Metabolism

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Adrenocortical carcinoma masquerading as Cushing's disease

Cited by 4 publications

References 9 publications

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

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

Approach to the Patient: Diagnosis of Cushing Syndrome

Low DHEAS Level: A Surrogate Marker of Adrenal Cushing Syndrome with Grey Zone ACTH Levels

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