scholarly journals A rare case of ectopic ACTH syndrome with rhabdomyolysis

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Wei Qiang ◽  
Sucai Song ◽  
Tianjun Chen ◽  
Zhe Wang ◽  
Jun Feng ◽  
...  
Keyword(s):  
Rare Case ◽  
Rare Complication ◽  
Clinical Manifestations ◽  
Dexamethasone Suppression Test ◽  
Ectopic Acth Syndrome ◽  
High Dose ◽  
Ectopic Acth ◽  
Supraclavicular Fossa ◽  
Suppression Test ◽  
Dexamethasone Suppression

Abstract Background Manifestations of hypokalaemia in ectopic adrenocorticotropic hormonesyndrome(EAS) vary from mild muscle weakness to life-threatening arrhythmia. Herein, we present a rare case of EAS with concomitant rhabdomyolysis(RM) as a result of intractable hypokalaemia. Case presentation A 64-year-old man was admitted for limb weakness and facial hyperpigmentation for 2 weeks. Lab tests revealed intractable hypokalaemia (lowest at 1.8 mmol/L) and metabolic alkalosis. The diagnosis of RM was based on a creatine kinase(CK)level of 5 times the upper limit. The elevated CK and myohemoglobin (Mb) levels returned to within the normal range after the alleviation of hypokalaemia. The patient was diagnosed with ACTH-dependent Cushing’s syndrome (CS) based on unsuppressed serum cortisol after a low-dose dexamethasone suppression test(LDDST) and remarkably elevated ACTH levels. The diagnosis of EAS was made based on the results of a high-dose dexamethasone suppression test(HDDST) and bilateral inferior petrosal sinus sampling(BIPSS). Multiple lymph nodes in the left supraclavicular fossa, right root of neck, mediastinum and bilateral hili of the lung were found with abnormal uptake of 68Ga-DOTA-NOC. Mediastinoscopic lymph node biopsy was performed. The pathological diagnosis was small-cell and large-cell neuroendocrine carcinoma with positive ACTH staining. The patient was prescribed mifepristone and received one cycle of chemotherapy. The patient could not tolerate subsequent chemotherapy and died of dyscrasia. Conclusions RM is a rare complication of EAS with insidious onset and atypical clinical manifestations. Serum potassium levels should be vigilantly monitored to avoid RM in EAS.

Nocturnal high-dose dexamethasone suppression test in the aetiological diagnosis of Cushing's syndrome

1985 ◽  
Vol 109 (2) ◽  
pp. 158-162 ◽  
Author(s):  
O. D. Bruno ◽  
Maria A. Rossi ◽  
Liliana N. Contreras ◽  
R. M. Gómez ◽  
G. Galparsoro ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Dexamethasone Suppression Test ◽  
High Dose ◽  
Ectopic Acth ◽  
High Dose Dexamethasone ◽  
Power Of The Test ◽  
Suppression Test ◽  
Aetiological Diagnosis ◽  
Dexamethasone Suppression

Abstract. Seventeen patients with well-proven Cushing's syndrome (13 with Cushing's disease, 3 with adrenal tumour and 1 presenting ectopic ACTH syndrome caused by bronchial carcinoid) were investigated by using a single-dose 8 mg dexamethasone nocturnal test. The results obtained were compared with those of the classical 8 mg Liddle's test, metyrapone stimulation, plasma ACTH concentration and with the final diagnosis reached through surgery, pathologic anatomy, and/or clinical and biochemical follow-up of the patients after treatment. The diagnostic efficacy or the predictive power of the test (defined as the ratio between the number of cases in which the diagnosis was correctly predicted and the total number of cases), was at least 82.4% vs 84.6% for the classical 8 mg Liddle's test. This percentage increased to 94.1% when the results of repeated tests on three patients with conflicting data were included. It is concluded that the nocturnal high-dose dexamethasone suppression test is a valuable tool in the aetiological diagnosis of Cushing's syndrome.

scholarly journals Ectopic ACTH syndrome: our experience with 25 cases

2006 ◽  
Vol 155 (5) ◽  
pp. 725-733 ◽  
Author(s):  
Luiz Roberto Salgado ◽  
Maria Candida B Villares Fragoso ◽  
Mirta Knoepfelmacher ◽  
Marcio Carlos Machado ◽  
Sorahia Domenice ◽  
...  
Keyword(s):  
Outcome Data ◽  
Pancreatic Tumors ◽  
Ectopic Acth Syndrome ◽  
Dynamic Tests ◽  
Resonance Imaging ◽  
Ectopic Acth ◽  
Suppression Test ◽  
Magnetic Resonance Imaging Mri ◽  
Work Up ◽  
Dexamethasone Suppression

Objective: Ectopic ACTH syndrome (EAS) occurs in about 5–10% of all patients with ACTH-dependent hypercortisolism with most of them caused by intrathoracic neoplasms. It may be associated with overt malignancies or with occult and indolent tumors. We assessed the accuracy of dynamic tests, inferior petrosal sinus sampling (IPSS) using desmopressin, and imaging in the work-up diagnosis of EAS. Design and subjects: Tumor markers, imaging, and outcome data from 25 patients (13F/12M) aged 18–72 years. High dexamethasone suppression test (HDDST), desmopressin test, GHRP-6 test, corticotropin-releasing hormone (CRH) test, IPSS, computed tomography (CT), magnetic resonance imaging (MRI), and 111In-pentetreotide scintigraphy were revised. Results: In 5 out of 20 patients HDDST was positive. In 13 patients who underwent desmopressin test, ACTH- and cortisol-positive responses were seen in six and five patients respectively. GHRP-6 test was positive in two out of three cases. Two patients underwent CRH test with negative response. In the seven patients submitted to IPSS using desmopressin in six of them, none had ACTH gradients. CT was positive in 15 out of 21 patients and MRI in 8 out of 17 cases. 111In-pentetreotide scintigraphy was positive in three out of five patients. Fourteen patients had intrathoracic tumors, five had pheochromocytomas, three had pancreatic tumors, one had a glomic tumor, and had three occult tumors. Six out of 11 patients with metastasis died and 3 others without metastasis died. Conclusions: IPSS with desmopressin was helpful for differential diagnosis. Patients initially harboring occult carcinoids may also exhibit severe hypercortisolism and those harboring tymic carcinoids had poor prognoses when compared with bronchial carcinoids and pheocromocytomas.

How early can one diagnose Cushing’s disease? An early diagnosis in a case of prepubertal Cushing’s disease

2014 ◽  
Vol 27 (11-12) ◽  
pp. 1043-1047 ◽  
Author(s):  
Julia Hoppmann ◽  
Isabel V. Wagner ◽  
Gudrun Junghans ◽  
Stefan A. Wudy ◽  
Michael Buchfelder ◽  
...  
Keyword(s):  
Early Diagnosis ◽  
Cushing’S Disease ◽  
Dexamethasone Suppression Test ◽  
Cushing's Disease ◽  
High Dose ◽  
Thyroid Function Tests ◽  
Free Cortisol ◽  
Suppression Test ◽  
Cortisol Levels ◽  
Dexamethasone Suppression

Abstract Background: Cushing’s disease is very rare in children, and the diagnosis is frequently delayed by several years. Objective: We report a case of prepubertal Cushing’s disease with a medical history of only 9 months. This case illustrates the difficulties involved in diagnosing children at the early stage of the disease. Case presentation: An 8-year-old prepubertal boy presented with rapid weight gain accompanied by a decreasing growth velocity and hirsutism. Thyroid function tests and growth factor levels were normal, thus excluding hypothyroidism and growth hormone deficiency. Cushing’s syndrome was confirmed by elevated 24-h urinary free cortisol levels, increased diurnal cortisol levels, and a lack of cortisol suppression in the low-dose dexamethasone suppression test. Further tests to investigate the source of the hypercortisolism showed the following results: Basal morning adrenocorticotropic hormone (ACTH) was normal. The high-dose dexamethasone suppression test led to a 51% decrease in cortisol level. In the corticotropin-releasing hormone (CRH) test, ACTH and cortisol increased only by 28%. Repeated magnetic resonance imaging (MRI) finally revealed a microadenoma in the anterior pituitary, thus establishng the diagnosis of Cushing’s disease. Upon diagnosis, the patient underwent transsphenoidal surgery. Histological analysis confirmed an ACTH-secreting pituitary adenoma. Conclusion: This case illustrates the difficulties associated with the clinical, biochemical, and radiological diagnoses of Cushing’s disease in children. Early diagnosis remains a challenge because test results often do not match standard diagnostic criteria.

scholarly journals An Optimized Pathway for the Differential Diagnosis of ACTH-Dependent Cushing’s Syndrome Based on Low-Dose Dexamethasone Suppression Test

2021 ◽  
Vol 12 ◽  
Author(s):  
Kang Chen ◽  
Shi Chen ◽  
Lin Lu ◽  
Huijuan Zhu ◽  
Xiaobo Zhang ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Cushing’S Syndrome ◽  
Low Dose ◽  
Serum Cortisol ◽  
Cushing's Syndrome ◽  
Dexamethasone Suppression Test ◽  
High Dose ◽  
Free Cortisol ◽  
Suppression Test ◽  
Dexamethasone Suppression

ContextTraditionally, low-dose dexamethasone suppression test (LDDST) was used to confirm the diagnosis of Cushing’s syndrome (CS), and high-dose dexamethasone suppression test (HDDST) was used to differentiate Cushing’s disease (CD) and ectopic adrenocorticotropin (ACTH) syndrome (EAS), but some studies suggested that HDDST might be replaced by LDDST. For the differential diagnosis of CS, dexamethasone suppression test was usually combined with other tests such as bilateral petrosal sinus sampling (BIPSS) and pituitary magnetic resonance imaging, but the optimal pathway to incorporate these tests is still controversial.ObjectivesTo develop an optimized pathway for the differential diagnosis of CD and EAS based on LDDST.Design and SettingSingle-center retrospective study (2011–2019).PatientsTwo hundred sixty-nine CD and 29 EAS patients with pathological diagnosis who underwent consecutive low- and high-dose DST.ResultsFor the differential diagnosis of CD and EAS, the area under curve (AUC) of LDDST using urine free cortisol (0.881) was higher than that using serum cortisol (0.685) (p < 0.001) in head-to-head comparison among a subgroup of 108 CD and 10 EAS. The AUC of LDDST (0.883) was higher than that of HDDST (0.834) among all the included patients. With the cutoff of <26%, the sensitivity and specificity of LDDST were 39.4% and 100%. We designed a new pathway in which BIPSS was only reserved for those patients with unsuppressed LDDST and adenoma <6mm, yielding an overall sensitivity of 97.7% and specificity of 86.7%.ConclusionLDDST had similar value to HDDST in differentiating CD and EAS using the specific cutoff point. The pathway that combined LDDST and BIPSS could differentiate CD and EAS accurately.

scholarly journals Selective blood sampling from the inferior petrosal sinuses for differential diagnostics of ACTH-dependent hypercortisolism (the original clinical experience)

2012 ◽  
Vol 58 (2) ◽  
pp. 29-33 ◽  
Author(s):  
A V Dreval' ◽  
I V Komerdus ◽  
A V Murzina ◽  
O A Nechaeva ◽  
I N Demidov ◽  
...  
Keyword(s):  
Clinical Experience ◽  
Primary Diagnosis ◽  
Dexamethasone Suppression Test ◽  
Blood Sampling ◽  
Immunohistochemical Method ◽  
Differential Diagnostics ◽  
High Dose ◽  
High Dose Dexamethasone ◽  
Suppression Test ◽  
Dexamethasone Suppression

This paper summarizes the original clinical experience of the authors concerning differential diagnostics of ACTH-dependent hypercortisolism. A total of 8 patients were available for the estimation of the potential of such diagnostics with the use of the high-dose dexamethasone suppression test, pituitary MRI, and selective blood sampling from the inferior petrosal sinuses for the determination of the ACTH concentration gradient between central and peripheral compartments. It turned out that neither the high-dose dexamethasone suppression test nor pituitary MRI provided unambiguous information about the source of ACTH hypersecretion whereas the use of selective blood sampling allowed to confirm the primary diagnosis of Cushing's disease in 4 patients and revise it in 2 others. In all the patients having the diagnosis established based on the results of selective blood sampling, it was confirmed after transsphenoidal adenomectomy. At the same time, the diagnosis of ACTH-ectopic syndrome was confirmed by an immunohistochemical method only in 1 of the 2 patients. Thus, the results of the present study indicate that selective blood sampling from the inferior petrosal sinuses is a valuable diagnostic tool which should be recommended for a wider application in endocrinological practice. However, this method failed to reveal lateralization of the tumours in all the examined patients.

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