Clinical and Pathological Features of Metastatic Adrenocortical Carcinoma
Authors: Guiomar Joana Reis , Silva Diana Festas , Catarino Diana Filipa , Moreno Carolina , Fadiga Lúcia , Lavrador Mariana , Vieira Inês , Araújo Bárbara , Araújo Cátia , Caetano Rui , Figueiredo Arnaldo , Paiva Isabel 
Published: May 2021

Abstract
Introduction: Adrenocortical carcinoma (AAC) is a rare and aggressive disease, associated with a poor prognosis. Surgery with complete resection (R0) remains the only curative treatment. However, even after complete resection, most patients present with distant metastatic disease. The aim of this study is to determine clinical and pathological features of metastatic disease in AAC. Materials and methods: Retrospective cohort study in 34 patients with AAC followed in our centre since 1991 until 2019. Selected patients with metastatic disease (n=21) and without metastatic disease (=13). Descriptive and comparative data analyses. Statistics: SPSS®v.23, with the variables: age, sex, clinical signs and symptoms, hormonal activity, imaging and pathological characteristics, surgical procedure, postoperative adjuvant treatments and overall survival. Results: 27 (79%) female and 7 (21%) male patients were included in our study, with a median age of 50 ± 13 years at the time of diagnosis. 21 patients (61,2%) presented with metastatic disease (38% of witch at the time of diagnosis) representing the metastatic disease group. 13 (38,8%) patients had no metastases until the collected data (group without metastatic disease). In the comparative analyses between the two groups, patients with metastatic disease had significantly more laparotomy procedures (71,2% n=15 vs 15,4% n=2; p&lt;0,05), bigger tumours (≥ 12cm) (52,4% n=11 vs 23% n=3; p&lt;0,05) and higher Ki67 (34,18% vs 1%, p&lt;0,05). Postoperatively, the metastatic group had higher LDH (LDH at 6 months) (582 ± 502 vs 181 ± 47; p&lt;0,05) and lower overall survival (months) (22,9 ± 4,69 vs 237,16 ± 44,42; p&lt;0,05). Patients with metastatic disease had more constitutional symptoms (weight loss and asthenia) (33,3% n = 7 vs 15,4% n = 2; p = 0.092) and incomplete surgical recessions (R1/R2) (42,8% n = 9 vs 15,4% n = 2; p=0.18), however, without statistical significance. There were no differences regarding: age, sex, hormonal activity, imaging characteristics and post-surgical medical treatment. Conclusion: In this study, the adrenocortical carcinoma metastasis rate was 61,2% with an overall survival of 23 months in the metastatic group. Laparotomy surgeries, tumour size ≥12cm and higher KI67 are features significantly associated with metastatic disease in adrenocortical carcinoma. Constitutional symptoms and incomplete surgical recessions are more common in metastatic patients, however without statistical significance, in this cohort.
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