ECE2020 Audio ePoster Presentations Adrenal and Cardiovascular Endocrinology (121 abstracts)
Treatment outcomes in 198 patients of the portuguese adrenocortical carcinoma register
Tiago Nunes da Silva 1 , Diana Catarino 2 , Isabel Paiva 2 , Carolina Chaves 1,3 , Claudia Costa 4 , Raquel Castro 5 , Ana Paula Marques 6 , Pedro Souteiro 7 , Maria Pereira 8 , Catarina Machado 9 , Carlos Belo 10 , Helder Simões 1 , Luisa Cortes 11 , Bernardo Pereira 3 , João Anselmo 3 , Inês Sapinho 12 , Ana Gonçalves Ferreira 13 , Maria Teresa Pereira 14 , Liliana Fonseca 14 , Isabel Fernandes 5 , Ana Rita Elvas 15 , Sara Donato 1 , Anabela Martins 16 , Laura Fajar 17 , Mariana Martinho 15,18 , Susana Garrido 18 & Marta Ferreira 9
1Institute of Oncology Francisco Gentil, Endocrinology, Lisboa, Portugal; 2Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal; 3Hospital do Divino Espírito Santo, Ponta Delgada, Portugal; 4IPO Porto, Porto, Portugal; 5Hospital de Santa Maria, Lisboa, Portugal; 6Hospital Pedro Hispano, Senhora da Hora, Portugal; 7Saint John Hospital, Porto, Portugal; 8Braga Public Hospital, Braga, Portugal; 9Centro Hospitalar de Vila Nova de Gaia/Espinho Espinho, Portugal; 10Hospital Egas Moniz, Lisboa, Portugal; 11Hospital Curry Cabral, Lisboa, Portugal; 12Hospital Fernando da Fonseca, Amadora, Portugal; 13Hospital Garcia de Orta, Almada, Portugal; 14Hospital Geral de Santo António, Porto, Portugal; 15Ipo Coimbra - Instituto Português De Oncologia De Coimbra Francisco Gentil, E.P.E., Coimbra, Portugal; 16Hospital da Luz Lisboa, Lisboa, Portugal; 17Hospital Viana ULSAM, Viana do Castelo, Portugal; 18Centro Hospitalar do Tâmega e Sousa, Portugal
Introduction: Adrenocortical carcinoma (ACC) is a rare entity, usually associated with a poor prognosis. There are still many areas of uncertainty regarding its approach.
Methods: A Portuguese ACC register was established in 2020, including information from 24 endocrine centres. Kaplan-Meier was used to calculate overall survival and recurrence; log- rank test to assess survival and progression free survival (PFS) differences between groups; cox-regression analysis to assess predictors of survival and PFS. Alfa level of significance was 0.05.
Results: 198 patients (63% female) with a mean age at diagnosis of 51.96 ± 15.1 years old presented with constitutional symptoms in 62.8%, hormonal hypersecretion in 47.2% and incidentally in 31.82%. The mean tumour size was 11.1 cm ± 5.09 with evidence of adenopathy in 21.8%, local invasion in 41.6% and distant metastasis in 47.9%. Surgery was performed in 88% of patients with negative resection margins in 61.44%. Mean Weiss score was 4.94 ± 1.6 and mean ki67 17.5%.ENSAT staging was: I – 1.6%; II – 30%; III – 20%; IV – 47.9%. Mitotane was used in 71% of patients, adjuvant radiotherapy was employed in 11%, and different modalities of chemotherapy in 19%. During follow-up: 32% remained disease-free; 27% recurred (63% at the adrenal bed, followed by lung and liver metastasis in 37% and 29%) and 42% progressed (the majority in the lungs and liver 56%, followed by the adrenal bed in 30%). One and five-year survival was 72.7 and 35.7%. One and five-year survival was 92.8/59.5% for ENSAT stage 2; 74.5/34.6% for ENSAT stage III and 35.8/9.9% for ENSAT stage IV. One and five-year PFS was, for stage II, 89.5%/56.5%; for stage III, 54.6/31.9%, and for stage IV, 33.0/0%. Patients with incidentally discovered tumours, lower ENSAT stages, R0 surgery, ki67 < 20%, low-grade tumours, stage IV patients treated with mitotane irrespective of other chemotherapy, and patients treated with radiotherapy had better survival and PFS in univariate analysis (P < 0.05). Tumour stage III and IV (HR 4.65 and 13.59, P < 0.001), age at diagnosis (HR 0.25, P = 0.031) and incidentally discovered tumours (HR 0.44, p = 0.034) were the only independent predictors of survival. Adrenal bed radiotherapy showed a trend towards improved survival (HR 0.25, P = 0.061). Stage IV was the only independent predictor of PFS (HR 18.36, P < 0.001).
Conclusions: This is the first characterisation of treatment outcomes of the Portuguese ACC population. Overall survival and its predictors are consistent with published literature, except for age, which impact is still uncertain.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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