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Endocrine Abstracts (2020) 70 AEP116 | DOI: 10.1530/endoabs.70.AEP116

1Coimbra Hospital and University Centre, Endocrinology Department, Coimbra, Portugal; 2Faculty of Medicine of the University of Coimbra, Endocrinology Department, Coimbra, Portugal; 3Coimbra Hospital and University Centre, Pathological Anatomy Department, Coimbra, Portugal; 4Coimbra Hospital and University Centre, Urology Department, Coimbra, Portugal


Introduction: Adrenocortical carcinoma (ACC) is a rare and aggressive tumor, with a poor prognosis and median survival of 3–4 years. Complete surgical resection is the only possibility of cure. The rate of post-surgical recurrence is high, so adjuvant treatment with mitotane plays a key role.

Objectives: To evaluate predictive factors of response to adjuvant treatment with mitotane in monotherapy in patients that underwent surgical resection of ACC.

Material and Methods: Retrospective study of medical records of ACC patients from March/1991 to May/2019. Analysis of clinical, biochemical, imaging and anatomopathological variables (Ki-67 and Weiss score) and their predictive role in response to adjuvant treatment with mitotane. Some patients did not have a description of all Weiss criteria. Results with P < 0.05 were considered statistically significant.

Results: Of the 34 patients, 22 were excluded (4 were not operated and 18 received mitotane in combination with chemotherapy and/or metyrapone and/or radiotherapy). Twelve patients were included. The average age at diagnosis was 50.5 ± 14.6 years, with 83.3% being female. At presentation 50.0% of patients had symptoms of hormonal hypersecretion, 25.0% had constitutional symptoms and 25.0% were diagnosed as incidentaloma. The laparotomy was the preferred surgical approach (n = 7; 58.3%). Complete tumor resection occurred in 8 patients (72.7%). The initial median dose of mitotane was 1.5 g/day (IQR:1.5), with an average maximum dose of 4.4 ± 2.6 g/day (minimum: 2.0; maximum: 8.0). The average time interval to reach the maximum daily dose of mitotane was 4.7 ± 3.9 months, with an average maximum mitotanemia of 14.5 ± 7.7 mg/l. 83.3% of patients reported treatment-limiting adverse effects, with a predominance of gastrointestinal effects. The median duration of therapy was 12 months (IQR:38.0; minimum: 1.0/maximum: 68.0). 25.0% had a complete response, without evidence of disease until the present time. The median survival was 18 months (IQR:38.3; minimum: 4.0/maximum: 113.0). In the univariate analysis, only the presence of atypical mitoseswas significant. The disease persisted in all cases with it vs 33% in the absence of atypia (P = 0.023). Gender, age, other imaging and anatomopathological characteristics, hormonal statusin the preoperative period, initial dose of mitotane, level of mitotanemia, and time to maximum dose or duration of therapy, were not predictive of the response.

Conclusions: In this study, the presence of atypical mitoses was the only significant survival predictor. Mitotanemia at the lower limit of the therapeutic window or even lower and the small sample size conditioned by the rarity of the pathology may have contributed to the results.

Volume 70

22nd European Congress of Endocrinology

Online
05 Sep 2020 - 09 Sep 2020

European Society of Endocrinology 

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