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Impana Shetty, Sarah Fuller, Margarita Raygada, Maria J Merino, B J Thomas, Brigitte C Widemann, Karlyne M Reilly, Karel Pacak and Jaydira Del Rivero

Background Adrenocortical carcinoma (ACC) is a rare and aggressive cancer with an incidence of 0.7–2 cases per million persons per year. ACC generally has a poor prognosis with a 5-year survival rate of 20–25% ( 1 , 2 ). ACC is a challenging

Open access

S Hussain, E Panteliou, D M Berney, R Carpenter, M Matson, A Sahdev, M Bell, E O'Sullivan and W M Drake

Background Pure aldosterone-secreting adrenocortical carcinomas (ACCs) are extremely rare tumours. Their presentation may be indistinguishable from patients with primary aldosteronism secondary to a benign adrenocortical adenoma. Our case

Open access

Philip D Oddie, Benjamin B Albert, Paul L Hofman, Craig Jefferies, Stephen Laughton and Philippa J Carter

Background Adrenocortical carcinoma (ACC) is a rare, malignant tumor of the adrenal glands with a bimodal distribution, affecting young children and adults. In children, it can present with virilization secondary to increased androgen levels

Open access

Skand Shekhar, Sriram Gubbi, Georgios Z Papadakis, Naris Nilubol and Fady Hannah-Shmouni

Background The incidence of adrenocortical carcinoma (ACC) is about 1.5–2 cases per million person-years ( 1 ). Even though it can present at any age, the age distribution follows a bimodal pattern peaking before the age of 5 and again between

Open access

Angelo Paci, Ségolène Hescot, Atmane Seck, Christel Jublanc, Lionel Mercier, Delphine Vezzosi, Delphine Drui, Marcus Quinkler, Martin Fassnacht, Eric Bruckert, Marc Lombès, Sophie Leboulleux, Sophie Broutin and Eric Baudin

Background Monitoring plasma mitotane levels is recommended in the follow-up of patients with unresectable adrenocortical carcinoma (ACC) to look for a therapeutic window of 14–20mg/L to optimize benefit over risk and avoid toxicities ( 1

Open access

Ravi Kumar Menon, Francesco Ferrau, Tom R Kurzawinski, Gill Rumsby, Alexander Freeman, Zahir Amin, Márta Korbonits and Teng-Teng L L Chung

diagnosis of possible adrenocortical cancer with sub-clinical cortisol and androgen excess was made. Treatment The patient underwent an open adrenalectomy to remove the 9×10 cm adrenal mass. Histology confirmed adrenocortical carcinoma with a

Open access

Pedro Marques, Nicola Tufton, Satya Bhattacharya, Mark Caulfield and Scott A Akker

or cortisol ( 3 , 10 ). Adrenal tumours with mixed steroid-secreting pattern should be evaluated for malignancy ( 10 ). Adrenocortical carcinomas are often associated with elevated mineralocorticoid precursors, including DOC, whereas benign adrenal

Open access

Katia Regina Marchetti, Maria Adelaide Albergaria Pereira, Arnaldo Lichtenstein and Edison Ferreira Paiva

Paiva assisted with the final version. References 1 Libé R 2015 Adrenocortical carcinoma (ACC): diagnosis, prognosis, and treatment . Frontiers in Cell and Developmental Biology 3 45 . ( doi:10.3389/fcell.2015.00045 ) 2 Ishikura

Open access

N F Lenders and J R Greenfield

in Table 2 . Marked elevation of tetrahydro-11 deoxycortisol (THS) was noted, which is associated with adrenocortical carcinoma. Figure 1 CT adrenals demonstrating left adrenal tumour. Table 1 Initial serum laboratory data

Open access

Vasileios Chortis, Christine J H May, Kassiani Skordilis, John Ayuk, Wiebke Arlt and Rachel K Crowley

use of cross-sectional imaging ( 1 ). Although the majority of AIs represent benign adenomas, a substantial minority consist of malignant tumours, including adrenocortical carcinomas, phaeochromocytomas and adrenal metastases from other primary