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.

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.

scholarly journals Analysis between Adrenal MRI Mass Size and 1 mg Dexamethasone Suppression Test in Patients with Adrenal Incidentaloma

2019 ◽  
Author(s):  
Pinar Karakaya ◽  
Bulent Yaprak ◽  
Bahar Ozdemir ◽  
Hakan Kocoglu
Keyword(s):  
Adrenal Incidentaloma ◽  
Dexamethasone Suppression Test ◽  
Test Results ◽  
Resonance Imaging ◽  
Mass Size ◽  
Adrenal Masses ◽  
Suppression Test ◽  
Magnetic Resonance Imaging Mri ◽  
Dexamethasone Suppression ◽  
Adrenal Nodule

This study aimed to investigate the correlation of adrenal magnetic resonance imaging (MRI) mass size and 1 mg dexamethasone suppression test results in patients with adrenal incidentaloma diagnosis. Eighty-six patients (82.6% female) with incidentally discovered adrenal masses, diagnosed and treated in the Department of Endocrinology Bakirkoy Dr. Sadi Konuk Education and Research Hospital between June and August 2017, were included in the analysis. All the patients evaluated for their hormonal functionality. 24-h urinary excretion of cortisol did not correlate with tumor size. But there was a positive correlation between longer adrenal nodule diameter and metanephrine, and there was a negative correlation between shorter adrenal nodule diameter and renin levels. We found that smaller and clinically “silent” tumors often do not demonstrate subclinical hormonal activity.

scholarly journals Ectopic ACTH Syndrome Emerging 5 Years after the Diagnosis of Neuroendocrine Tumor

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Minghao Liu ◽  
Diane Hamele-Bena ◽  
John Ausiello ◽  
Gabrielle Page-Wilson
Keyword(s):  
Neuroendocrine Tumor ◽  
Serum Cortisol ◽  
Urinary Free Cortisol ◽  
Ectopic Acth Syndrome ◽  
Ectopic Acth ◽  
Free Cortisol ◽  
Metastatic Lung ◽  
Suppression Test ◽  
Dexamethasone Suppression ◽  
Retrospective Examination

Ectopic ACTH syndrome (EAS) arising years after the diagnosis of a neuroendocrine tumor (NET) is exceedingly rare. We describe a case of EAS occurring five years after the diagnosis of a metastatic lung NET in a 61-year-old woman. She presented with severe hypokalemia but was not overtly Cushingoid on exam. Serum cortisol was 61mcg/dL after an overnight 1mg dexamethasone suppression test (<1.8mcg/dL) and urinary free cortisol was 7544 mcg/24h (<45mcg/24h), establishing the diagnosis of Cushing’s syndrome. Plasma levels of peptides which have been associated with EAS, Agouti-related peptide (AgRP) and the ACTH precursors POMC (31-kDa) and pro-ACTH (22-kDa), were elevated. Metyrapone was initiated, but hypercortisolism persisted and the patient succumbed to pneumonia shortly after presentation. Retrospective examination of biopsy tissues showed rare ACTH immunoreactivity at the time of initial diagnosis, followed by staining in a greater proportion of cells as the disease progressed, consistent with EAS arising years after the diagnosis of NET. Given the increase in mortality associated with EAS, this unusual case highlights the importance of early detection and raises the possibility that early immunohistochemical stains for ACTH and measurements of ACTH precursors may facilitate the identification of NETs at high risk for EAS.

scholarly journals Pulvinar sign in a case of anti-HU paraneoplastic encephalitis

2016 ◽  
Vol 29 (6) ◽  
pp. 436-439 ◽  
Author(s):  
Pierre-Luc Gamache ◽  
Maude-Marie Gagnon ◽  
Martin Savard ◽  
François Émond
Keyword(s):  
Magnetic Resonance Imaging ◽  
Differential Diagnosis ◽  
Magnetic Resonance ◽  
Clinical Manifestations ◽  
Resonance Imaging ◽  
Paraneoplastic Encephalitis ◽  
Jakob Disease ◽  
Magnetic Resonance Imaging Mri ◽  
Work Up

This article reports the case of a 68-year-old patient with anti-HU antibodies paraneoplastic encephalitis. The clinical manifestations were atypical and the paraclinical work-up, notably the magnetic resonance imaging (MRI) showing bilateral posterior thalamic hyperintensities (pulvinar sign), misleadingly pointed towards a variant Creutzfeld–Jakob disease. After presenting the case, the differential diagnosis of the pulvinar sign is discussed along with other important diagnostic considerations.

Reappraisal of left‐sided gallbladder and its accompanying anomalies: a report of two cases and literature review

2005 ◽  
Vol 46 (3) ◽  
pp. 233-236 ◽  
Author(s):  
T. ‐C. Wu ◽  
R. ‐C. Lee ◽  
J. ‐H. Chiang ◽  
C. ‐Y. Chang
Keyword(s):  
Magnetic Resonance Imaging ◽  
Portal Vein ◽  
Three Dimensional ◽  
Ligamentum Teres ◽  
Hepatobiliary Surgery ◽  
Resonance Imaging ◽  
Magnetic Resonance Imaging Mri ◽  
Anatomical Information ◽  
Preoperative Work Up ◽  
Work Up

We report two cases of coexistent left‐sided gallbladder and right‐sided ligamentum teres with portal vein anomalies documented by magnetic resonance imaging (MRI) and three‐dimensional (3D) computed tomography during arterial portography (CTAP). Reformatted 3D MR and CTAP images provide an informative illustration of the accompanying portal vein anomalies. This important anatomical information is useful in preoperative work‐up of hepatobiliary surgery.

Musculotendinous Disorders of the Abdomen and Pelvis

2017 ◽  
Vol 21 (04) ◽  
pp. 403-414
Author(s):  
William Palmer ◽  
Miriam Bredella ◽  
Arvin Kheterpal
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Groin Pain ◽  
Iliotibial Band ◽  
Normal Anatomy ◽  
Resonance Imaging ◽  
Injury Mechanisms ◽  
Common Causes ◽  
Magnetic Resonance Imaging Mri ◽  
Work Up

AbstractMusculotendinous disorders of the abdomen and pelvis are common causes of pain in both athletes and nonathletes. Magnetic resonance imaging (MRI) is the modality of choice in the work-up of these patients. This article focuses on the MRI appearance of normal anatomy and spectrum of musculotendinous disorders in the abdomen and pelvis including muscle strains and tears, avulsions and apophysitis, muscular contusions, athletic groin pain, and gluteal aponeurotic/proximal iliotibial band injuries. Normal biomechanics and injury mechanisms are discussed.

scholarly journals Sinonasal Chondrosarcoma, an Unusual Location

2020 ◽  
Author(s):  
Oumniya Abouhanine ◽  
Aicha Merzem ◽  
Vianney Ndayishimiye ◽  
Omar Amriss ◽  
Hasnaa Belgadir ◽  
...  
Keyword(s):  
Hard Palate ◽  
Locally Advanced ◽  
Long Bones ◽  
Resonance Imaging ◽  
Cross Sectional ◽  
Extension Work ◽  
Cross Sectional Imaging ◽  
Magnetic Resonance Imaging Mri ◽  
Left Maxillary Sinus ◽  
Work Up

Introduction: Chondrosarcoma is a tumour with a cartilaginous matrix frequently encountered in long bones and the pelvis with rare sinonasal location. Patient and methods: We report the case of a 25-year-old patient who was referred to us for an extension work-up for sinonasal chondrosarcoma confirmed by anatomopathological examination. Results: Facial magnetic resonance imaging (MRI) confirmed by a CT scan showed an osteolytic tumour process of the hard palate and walls of the left maxillary sinus that was locally advanced. Conclusion: The sinonasal location of a chondrosarcoma is rare. Characterized by cross-sectional imaging, confirmation was provided by histological assessment.

The Case of the Missing Nose: Congenital Arhinia Case Presentation and Management Recommendations

2020 ◽  
Vol 129 (7) ◽  
pp. 645-648
Author(s):  
Andrew K. Fuller ◽  
Hilary C. McCrary ◽  
M. Elise Graham ◽  
Jonathan R. Skirko
Keyword(s):  
Neonatal Period ◽  
Rare Condition ◽  
Male Infant ◽  
Nasal Patency ◽  
Review Of The Literature ◽  
Resonance Imaging ◽  
Case Presentation ◽  
Magnetic Resonance Imaging Mri ◽  
Management Recommendations ◽  
Work Up

Objectives: To discuss the presentation and management of infants with arhinia or congenital absence of the nose. Methods: This case report describes an infant with arhinia that was diagnosed prenatally. In addition to a discussion of the case, a review of the literature was completed to define appropriate postnatal work-up and management. Results: The patient is a term male infant, diagnosed with arhinia on ultrasound and magnetic resonance imaging (MRI) performed at 21-weeks gestational age. Upon birth, the patient was subsequently intubated, followed by tracheostomy due to complete nasal obstruction. Through a genetics evaluation, the patient was found to be heterozygous for the SMCHD1 gene, with hypomethylation at the D4Z4 locus. Plans for reconstruction will be based on future imaging and the development of any nasal patency, however, the patient’s family plans to utilize a prosthetic nose until the patient is older. Conclusion: Arhinia is a rare condition causing respiratory distress in the neonatal period. While stabilization of the airway is the first priority, further management is not clearly defined given the rarity of the malformation. This case discusses stabilization of the airway with a review of treatment and reconstructive options.

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.

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