ECE2020 Audio ePoster Presentations Adrenal and Cardiovascular Endocrinology (121 abstracts)
Diagnosis, treatment and survival of adrenocortical carcinoma: 28 years of experience
Diana Catarino 1 , Diana Silva 1 , Joana Guiomar 1 , Carolina Moreno 1 , Critina Ribeiro 1 , Leonor Gomes 1 , Lucia Fadiga 1 , Mariana Lavrador 1 , Inês Vieira 1 , Rui Caetano 2 , Arnaldo Figueiredo 3 & Isabel Paiva 1
1Centro Hospitalar e Universitário de Coimbra EPE, Coimbra, Portugal, Endocrinology, Diabetes and Metabolism Department; 2Centro Hospitalar e Universitário de Coimbra EPE, Coimbra, Portugal, Pathologic Anatomy Department; 3Centro Hospitalar e Universitário de Coimbra EPE, Coimbra, Portugal, Urology Department
Introduction: Adrenocortical carcinoma is a rare endocrine disease characterized by an aggressive behaviour with a poor prognosis. Clinical experience, even with a little number of patients, has enhanced knowledge about this malignancy, in most cases challenging.
Objective: Characterization of patients with adrenocortical carcinoma followed at the endocrine department of a hospital centre, between 1991 and 2019.
Methods: Retrospective cohort study, based on clinical records of patients with histopathologic confirmed result of adrenocortical carcinoma. Statistical analyses with SPSS v.25.
Results and conclusion: We obtained 34 patients: 79.4% female, mean age at the diagnosis 50.03 ± 13.02 years. Clinical presentation: 50% (n = 17) - symptoms of adrenal hormone excess, 32% (n = 11) - low back/abdominal pain or weight loss, 18% (n = 6) - adrenal incidentalomas. Computerized tomography scan (CT) data: mean tumour size - 10.5 ± 4.2 cm, necrosis imaging- 80%, CT density >10UH - 100%, additional invaded organs – 35.3% (mainly kidney and inferior cava vein), metastases at the diagnosis – 26.5%. Laboratory analysis: functional tumours (71%, n = 24); hypercortisolism – n = 11; hypercortisolism and androgen excess – n = 9; androgen excess – n = 3, hypercortisolism, androgen and mineralocorticoid excess – n = 1. Surgery treatment was performed on 88.2% (n = 30) of patients. Surgical approach: open surgery – 67% (n = 20), laparoscopic adrenalectomy – 32% (n = 10). Resection: R0 – 72.4%, R1 – 24.1%, R2 – 4.4%. ENSAT stage: 66.7% (n = 20) in the stage II, 20% (n = 6) in stage III, 13.3% (n = 4) in stage IV. Histopathologic analysis: median of weight tumour – 260.5 g(680 g), between 71-2600 g; mean Weiss-score −5.36 ± 1,55; mitotic rate (>5 per 50 high-power fields) – 73.3%, abnormal mitoses – 68.4%, cytoplasm with clear cells comprising 25% or less of the tumour – 84.6%, necrosis – 91.7%, capsular invasion – 62.5%, mean Ki-67 index −26.2 ± 22.8. Medical adjuvant therapy was performed on 70.6% (n = 4) patients: mitotane monotherapy (n = 12), mitotane and chemotherapy (n = 5), mitotane and metyrapone (n = 5), mitotane, chemotherapy and metyrapone (n = 2). Radiotherapy was performed only on one patient. Relative to mitotane therapy: median initial dose −1.5 g/day (1.5), median of maximum dose −5.4 g/day (3.0); mean of mitotane plasma level −14.8 ± 7.9 mg/l. Therapeutic mitotane plasma level was reached at 60% of patients. The mean overall survival was 52.2 ± 79.1 months and was higher in youngers patients (r = -0.09; P = 0.596). Actually, 12 patients are alive, 11 of them with complete remission. Despite the poor prognosis of this malignancy, the high survival rate and the high number of patients on complete remission found in this series (32.4%) is highlighted.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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