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Clinical and Pathological Features of Metastatic Adrenocortical Carcinoma

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 st...

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Detalles Bibliográficos
Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8089528/
http://dx.doi.org/10.1210/jendso/bvab048.159
Descripción
Sumario: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<0,05), bigger tumours (≥ 12cm) (52,4% n=11 vs 23% n=3; p<0,05) and higher Ki67 (34,18% vs 1%, p<0,05). Postoperatively, the metastatic group had higher LDH (LDH at 6 months) (582 ± 502 vs 181 ± 47; p<0,05) and lower overall survival (months) (22,9 ± 4,69 vs 237,16 ± 44,42; p<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. Unless otherwise noted, all abstracts presented at ENDO are embargoed until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. Abstracts presented at a news conference are embargoed until the date and time of the news conference. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.