Serum inhibin pro-αC is a tumor marker for adrenocortical carcinomas

ObjectiveThe insufficient diagnostic accuracy for differentiation between benign and malignant adrenocortical disease and lack of sensitive markers reflecting tumor load emphasize the need for novel biomarkers for diagnosis and follow-up of adrenocortical carcinoma (ACC).DesignSince the inhibin α-subunit is expressed within the adrenal cortex, the role of serum inhibin pro-αC as a tumor marker for ACC was studied in patients.MethodsRegulation of adrenal pro-αC secretion was investigated by adrenocortical function tests. Serum inhibin pro-αC levels were measured in controls (n=181) and patients with adrenocortical hyperplasia (n=45), adrenocortical adenoma (ADA,n=32), ACC (n=32), or non-cortical tumors (n=12). Steroid hormone, ACTH, and inhibin A and B levels were also estimated in patient subsets.ResultsSerum inhibin pro-αC levels increased by 16% after stimulation with ACTH (P=0.043). ACC patients had higher serum inhibin pro-αC levels than controls (medians 733 vs 307 ng/l,P<0.0001) and patients with adrenocortical hyperplasia, ADA, or non-adrenocortical adrenal tumors (148, 208, and 131 ng/l, respectively,P=0.0003). Inhibin pro-αC measurement in ACC patients had a sensitivity of 59% and specificity of 84% for differentiation from ADA patients. Receiver operating characteristic analysis displayed areas under the curve of 0.87 for ACC vs controls and 0.81 for ACC vs ADA (P<0.0001). Surgery or mitotane therapy was followed by a decrease of inhibin pro-αC levels in 10/10 ACC patients tested during follow-up (P=0.0065).ConclusionsInhibin pro-αC is produced by the adrenal gland. Differentiation between ADA and ACC by serum inhibin pro-αC is limited, but its levels may constitute a novel tumor marker for ACC.

Download Full-text

Adrenal involvement in MEN1. Analysis of 715 cases from the Groupe d'étude des Tumeurs Endocrines database

Acta Endocrinologica ◽

10.1530/eje-11-0679 ◽

2012 ◽

Vol 166 (2) ◽

pp. 269-279 ◽

Cited By ~ 94

Author(s):

B Gatta-Cherifi ◽

O Chabre ◽

A Murat ◽

P Niccoli ◽

C Cardot-Bauters ◽

...

Keyword(s):

Adrenal Tumors ◽

Limited Data ◽

Phenotype Correlation ◽

Adrenal Tumours ◽

Risk Condition ◽

Tumeurs Endocrines ◽

Adrenocortical Carcinomas ◽

Multicentre Cohort

ObjectiveLimited data regarding adrenal involvement in multiple endocrine neoplasia type 1 (MEN1) is available. We describe the characteristics of MEN1-associated adrenal lesions in a large cohort to provide a rationale for their management.MethodsAnalysis of records from 715 MEN1 patients from a multicentre database between 1956 and 2008. Adrenal lesions were compared with those from a multicentre cohort of 144 patients with adrenal sporadic incidentalomas.ResultsAdrenal enlargement was reported in 20.4% (146/715) of patients. Adrenal tumours (>10 mm in size) accounted for 58.1% of these cases (10.1% of the whole patient cohort). Tumours were bilateral and >40 mm in size in 12.5 and 19.4% of cases respectively. Hormonal hypersecretion was restricted to patients with tumours and occurred in 15.3% of them. Compared with incidentalomas, MEN1-related tumours exhibited more cases of primary hyperaldosteronism, fewer pheochromocytomas and more adrenocortical carcinomas (ACCs; 13.8 vs 1.3%). Ten ACCs occurred in eight patients. Interestingly, ACCs occurred after several years of follow-up of small adrenal tumours in two of the eight affected patients. Nine of the ten ACCs were classified as stage I or II according to the European Network for the Study of Adrenal Tumors. No evident genotype/phenotype correlation was found for the occurrence of adrenal lesions, endocrine hypersecretion or ACC.ConclusionsAdrenal pathology in MEN1 differs from that observed in sporadic incidentalomas. In the absence of relevant symptoms, endocrine biology can be restricted to patients with adrenal tumours and should focus on steroid secretion including the aldosterone–renin system. MEN1 is a high-risk condition for the occurrence of ACCs. It should be considered regardless of the size of the tumour.

Download Full-text

Laparoscopic Adrenalectomy for Potentially Malignant Adrenal Tumors Greater than 5 Centimeters

The Journal of Clinical Endocrinology & Metabolism ◽

10.1210/jc.2005-2420 ◽

2006 ◽

Vol 91 (8) ◽

pp. 3080-3083 ◽

Cited By ~ 61

Author(s):

Chun-Hou Liao ◽

Shih-Chieh Chueh ◽

Ming-Kuen Lai ◽

Po-Jen Hsiao ◽

Jun Chen

Keyword(s):

Tumor Size ◽

Laparoscopic Adrenalectomy ◽

Malignant Tumors ◽

Adrenal Tumors ◽

Benign Tumors ◽

Conversion To Open Surgery ◽

Open Conversion ◽

Potentially Malignant ◽

Adrenocortical Carcinomas

Abstract Purpose: Laparoscopic adrenalectomy (LA) is controversial for large, potentially malignant tumors. We report a series of LA or hand-assisted LA for large (>5 cm) adrenal tumors. Patients and Methods: Among 210 LAs performed in 6 yr, 39 patients had potentially malignant tumors greater than 5 cm in diameter. Their perioperative and follow-up data were retrospectively analyzed. Results: All 39 patients had successful LAs without perioperative mortality, conversion to open surgery, or capsular disruption during dissection. The mean tumor size was 6.2 cm (range, 5–12 cm), operative time 207 min (115–315 min), and blood loss 75 ml (minimal–1400 ml). Complications included one intraoperative diaphragmatic perforation, three mild wound infections, and one pneumonia. Preoperatively there were 27 nonfunctioning tumors, seven pheochromocytomas, three cortisol-secreting tumors, and two virilizing tumors. Final pathology revealed eight malignant (four adrenocortical carcinomas and four metastatic carcinomas) and 31 benign tumors (14 cortical adenomas, eight pheochromocytomas, six myelolipomas, and three ganglioneuromas). Median follow-up was 39 months. Four patients (two adrenocortical carcinomas, one metastatic hepatoma, and one lymphoma) died 24, 10, 9, and 3 months after surgery, respectively. A hand-assisted device was used in 10 patients. Only the tumor size was larger and length of postoperative hospital stay longer for those in the hand-assisted group. Conclusions: LA is a reasonable option for selected large adrenal tumors when complete resection is technically feasible and there is no evidence of local invasion. Hand-assisted LA is a good alternative to open conversion if a difficult dissection is encountered intraoperatively.

Download Full-text

Pathological Findings in the Adrenal Glands of 80 Dogs

Acta Scientiae Veterinariae ◽

10.22456/1679-9216.88860 ◽

2018 ◽

Vol 46 (1) ◽

pp. 7

Author(s):

Elisângela Olegário Da Silva ◽

Giovana Wingeter Di Santis ◽

Selwyn Arlington Headley ◽

Ana Paula Frederico Rodrigues Loureiro Bracarense

Keyword(s):

Adrenal Gland ◽

Adrenocortical Carcinoma ◽

Adrenal Glands ◽

Clinical Signs ◽

Adrenal Tumors ◽

Endocrine Disorders ◽

Adrenocortical Adenoma ◽

Primary Tumors ◽

Nodular Hyperplasia ◽

Adrenocortical Carcinomas

Background: The adrenal glands development important endocrine functions and can be affected by primary or secondary diseases. These adrenal gland pathologies may induce clinical syndromes resulting from abnormalities in the production and secretion of hormones. Data about pathological changes in dogs are scarce. Therefore, the aim of the present study was to identify and evaluate the histopathological and epidemiological features of adrenal changes in dogs submitted to necropsy examination from 2005 to 2016 in a Veterinary Teaching Hospital, Londrina, Paraná, Brazil.Material, Methods & Results: During this period, 80 animals presented alterations of adrenal gland, representing 5.5% of all necropsied dogs. The pure breed dogs representing 58.6% and mixed breed 41.4%; 53.4% were female and 46.6% were male. The non-neoplastic adrenal lesions were more frequently (57.5%) compared to the neoplastic changes (42.5%). Most of the adrenal glands lesions occurred in older dogs (60%), following by the middle aged (31.25%) and young dogs (8.75%). The main non-neoplastic lesions observed in the adrenal glands were of hyperplastic (69.5%) and circulatory (26%) origin, among the earlier, 68.8% were diffuse hyperplasia and 31.2% nodular hyperplasia. The nodular hyperplasia was classified as micronodular multifocal in 40% of the glands with nodular hyperplasia, macronodular multifocal in 30%, micronodular diffuse in 20%, and micronodular focal in 10%. The neoplasms observed were adrenocortical adenoma (ACA) in 44.1%, pheochromocytomas in 23.5% and adrenocortical carcinomas (ACC) in 11.7% of the dogs with adrenal tumors. Metastasis from other primary tumors were observed in 20.6%.Discussion: The data in veterinary literature about the frequency of changes in adrenal gland of dogs are scarce and focus features of diagnosis by ultrasound examination. Most of the adrenal changes observed in the present study were incidental findings observed during the necropsy examination and no specific clinical signs were observed. Non-neoplastic adrenal lesions, mainly of hyperplastic origin, were more frequently observed compared to the neoplastic changes. Differing from previous studies, the diffuse hyperplasia was the most common non neoplastic finding in the adrenal glands. Considering the subclassification of nodular hyperplasia, the multifocal micronodular and macronodular multifocal pattern were the most frequent. In veterinary literature, there are no data about frequency of nodular hyperplastic subtypes. However, in humans the subclassification of nodular hyperplasia is associated to development of endocrine disorders. Previous studies reported lower incidence of neoplastic changes in adrenal gland of dogs compared to the present results. The adrenocortical adenoma was the most common primary tumor, followed by the adrenocortical carcinoma, pheochromocytoma and adrenocortical carcinoma. Congestion and hemorrhage were common findings observed in the adrenal gland primary tumors. On the other hand, necrosis and inflammatory infiltrate were observed only in the adrenocortical carcinomas. These histopathological features may be used as tool in the differential diagnosis between well differentiated adrenocortical carcinoma and adenoma. The histopathological examination was fundamental to differential and definitive diagnosis of all canine adrenal gland disorders observed.

Download Full-text

Chromogranin a Measurement in Neuroendocrine Tumors

The International Journal of Biological Markers ◽

10.1177/172460089801300102 ◽

1998 ◽

Vol 13 (1) ◽

pp. 3-9 ◽

Cited By ~ 14

Author(s):

L. Ferrari ◽

E. Seregni ◽

A. Martinetti ◽

B Van Graafeiland ◽

S. Nerini-Molteni ◽

...

Keyword(s):

Tumor Marker ◽

Neuroendocrine Tumors ◽

Chromogranin A ◽

High Sensitivity ◽

Biologically Active ◽

Proliferative Index ◽

Favorable Prognosis ◽

Blood Marker ◽

Active Substances

Neuroendocrine tumors (NETs) are rare neoplasms characterized by a low proliferative index and, in some cases, a favorable prognosis. These tumors often overproduce and release biologically active substances that are responsible for severe syndromes. Tumor marker measurement provides the clinician with useful information for the management of NET patients. The substances released by overproducing tumors are currently used as biomarkers, but there is a need for sensitive markers also for the “biochemically silent” NETs. The most effective and reliable blood marker available today is chromogranin A (CgA). Because of its high sensitivity and specificity, this glycoprotein can be used for the diagnosis, prognosis and follow-up of NETs. Furthermore, CgA measurement can be used for monitoring those tumors not overproducing or releasing any hormones or biological amines. This paper is a synthetic review on the value of CgA in NET management and reports our experiences with CgA measurement in NET patients.

Download Full-text

Adrenal Cortex-Sparing Surgery for Bilateral Multiple Pheochromocytomas in a Patient with Von Hippel-Lindau Disease

Case Reports in Medicine ◽

10.1155/2012/659104 ◽

2012 ◽

Vol 2012 ◽

pp. 1-5 ◽

Cited By ~ 1

Author(s):

Tarık Esen ◽

Ömer Acar ◽

Ahmet Tefekli ◽

Ahmet Musaoğlu ◽

İzzet Rozanes ◽

...

Keyword(s):

Adrenal Cortex ◽

Adrenocortical Function ◽

Renal Masses ◽

Von Hippel Lindau ◽

Pancreatic Masses ◽

Adrenal Pheochromocytoma ◽

Von Hippel Lindau Disease ◽

Vhl Disease

Pheochromocytomas can be a part of familial neoplastic syndromes, in which case they tend to be multiple and involve both adrenal glands. Therefore, sparing adrenocortical function represents a major concern while dealing with these hereditary lesions. Herein, we describe the clinical characteristics and the management strategy of a patient with von Hippel-Lindau (VHL) disease who had multiple, bilateral pheochromocytomas as well as bilateral renal masses, pancreatic masses, and a paracaval mass. Only a portion of the left adrenal gland has remained in situ after two consecutive open surgeries and a percutaneous radiofrequency ablation which have been performed to treat the various components of this syndrome. No adrenal or extra-adrenal pheochromocytoma recurrences have been detected during a follow-up period of more than 2 years. Pancreatic and adrenal functions were normal throughout the postoperative period and never necessitated any replacement therapy. Adrenal cortex-sparing surgery is a valid option for VHL disease patients who present with synchronous bilateral adrenal pheochromocytomas.

Download Full-text

Virtual monoenergetic dual-energy CT reconstructions at 80 keV are optimal non-contrast CT technique for early stroke detection

The Neuroradiology Journal ◽

10.1177/19714009211047449 ◽

2021 ◽

pp. 197140092110474

Author(s):

Doris Dodig ◽

Zrinka Matana Kaštelan ◽

Nina Bartolović ◽

Slaven Jurković ◽

Damir Miletić ◽

...

Keyword(s):

Diagnostic Accuracy ◽

Symptom Onset ◽

Brain Oedema ◽

Dual Energy ◽

Characteristic Analysis ◽

Stroke Incidence ◽

Ct Technique ◽

Contrast Ct ◽

Early Stroke

Background Virtual monoenergetic (VM) dual-energy computed tomography (DE-CT) enables grey-to-white matter contrast-to-noise ratio optimization, potentially increasing ischaemic brain oedema visibility. The aim of this study was to compare the diagnostic accuracy of VM and standard DE-CT reconstructions for early stroke detection. Methods Consecutive patients with non-contrast DE-CT of the brain scanned within 12 h of stroke symptom onset were prospectively included in the study. Patients with other significant brain pathology were excluded. Two radiologists jointly evaluated standard and VM reconstructions (from 40 to 190 keV at increments of 10 keV) for early stroke signs on a four-point Likert scale: (a) stroke definitely present, (b) stroke probably present, (c) probably no stroke, and (d) definitely no stroke. Follow-up imaging and clinical data served as the standard of reference. Diagnostic accuracy was evaluated by receiver operating characteristic analysis. Results Stroke incidence among 184 patients was 76%. In 64 patients follow-up imaging served as the standard of reference: ischemic brain oedema detection was significantly more accurate on VM reconstructions at 80 keV compared with standard DE-CT reconstructions (area under the curve (AUC) = 0.821 vs. AUC = 0.672, p = 0.002). The difference was most prominent within the first 3 h after symptom onset (at 11%, AUC = 0.819 vs. AUC = 0.709, p = 0.17) and in patients with National Institutes of Health Stroke Scale above 16 (at 37.5%, AUC = 1 vs. AUC = 0.625, p = 0.14). Conclusion VM DE-CT reconstructions at 80 keV appear to be the optimal non-contrast CT technique for diagnosing early ischaemic stroke, particularly within the first 3 h after symptom onset and in severely ill patients.

Download Full-text

The role of imaging in congenital adrenal hyperplasia

Arquivos Brasileiros de Endocrinologia & Metabologia ◽

10.1590/0004-2730000003371 ◽

2014 ◽

Vol 58 (7) ◽

pp. 701-708 ◽

Cited By ~ 6

Author(s):

Sara Reis Teixeira ◽

Paula Condé Lamparelli Elias ◽

Marco Túlio Soares Andrade ◽

Andrea Farias Melo ◽

Jorge Elias Junior

Keyword(s):

Congenital Adrenal Hyperplasia ◽

Adrenal Tumors ◽

Adrenal Hyperplasia ◽

Salt Wasting ◽

Testicular Adrenal Rest Tumors ◽

High Incidence ◽

Adrenal Rest ◽

Hormonal Evaluation

Congenital adrenal hyperplasia (CAH) is an autossomic recessive disorder caused by impaired steroidogenesis. Patients with CAH may present adrenal insufficiency with or without salt-wasting, as well as various degrees of virilization and fertility impairment, carrying a high incidence of testicular adrenal rest tumors and increased incidence of adrenal tumors. The diagnosis of CAH is made based on the adrenocortical profile hormonal evaluation and genotyping, in selected cases. Follow-up is mainly based on hormonal and clinical evaluation. Utility of imaging in this clinical setting may be helpful for the diagnosis, management, and follow-up of the patients, although recommendations according to most guidelines are weak when present. Thus, the authors aimed to conduct a narrative synthesis of how imaging can help in the management of patients with CAH, especially focused on genitography, ultrasonography, computed tomography, and magnetic resonance imaging.

Download Full-text