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Horm Metab Res 2021; 53(11): 709-716
DOI: 10.1055/a-1666-9026
Endocrine Care

Adrenocortical Cancer: A 20-Year Experience of a Single Referral Center in Prognosis and Outcomes

Christos Parianos
1   Department of Biological Chemistry, National and Kapodistrian University of Athens, Athens, Greece
2   Department of Surgery, General Hospital of Athens “G. Gennimatas”, Athens, Greece
,
Georgios Kyriakopoulos
1   Department of Biological Chemistry, National and Kapodistrian University of Athens, Athens, Greece
3   Department of Pathology, Evaggelismos Hospital, Athens, Greece
,
Ioannis D. Kostakis
4   Department of Hepato-Pancreato-Biliary Surgery and Liver Transplantation, Royal Free Hospital, London, UK
,
Narjes Nasiri-Ansari 
1   Department of Biological Chemistry, National and Kapodistrian University of Athens, Athens, Greece
,
Chrysanthi Aggeli
2   Department of Surgery, General Hospital of Athens “G. Gennimatas”, Athens, Greece
,
Anastasia Dimitriadi
5   Department of Pathology, General Hospital of Athens “G. Gennimatas”, Athens, Greece
,
Anna Angelousi
6   Unit of Endocrinology, First Department of Internal Medicine, Laiko Hospital, National and Kapodistrian University of Athens, Athens, Greece
,
Athanasios G. Papavassiliou
1   Department of Biological Chemistry, National and Kapodistrian University of Athens, Athens, Greece
,
Gregory A. Kaltsas
7   Unit of Endocrinology, First Department of Propaedeutic Internal Medicine, Laiko Hospital, National & Kapodistrian University of Athens, Athens, Greece
,
George Zografos‡
2   Department of Surgery, General Hospital of Athens “G. Gennimatas”, Athens, Greece
,
Eva Kassi‡
1   Department of Biological Chemistry, National and Kapodistrian University of Athens, Athens, Greece
7   Unit of Endocrinology, First Department of Propaedeutic Internal Medicine, Laiko Hospital, National & Kapodistrian University of Athens, Athens, Greece
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Abstract

Adrenocortical carcinoma (ACC) is a rare but very aggressive endocrine malignancy with poor survival. Histopathology is important for diagnosis, while in some cases immunohistochemical markers and gene profiling of the resected tumor may be superior to current staging systems to determine prognosis. We aimed to present the 20-year experience at a tertiary hospital in patients with ACCs and correlate the immunohistochemical characteristics of ACCs with the clinical and morphological characteristics of the tumors and the survival of the patients. Forty-five patients with ACC were included in the study. All the resections were R0. The tumor size and weight, the disease stage (ENSAT classification), Weiss score and Helsinki score were examined along with immunohistochemical expression of inhibin-A, melan A, calretinin, Ki67, synaptophysin, p53, vimentin, CKAE1/AE3. The male to female ratio was 1:1.37. The median age at diagnosis was 55.5 years (IQR 19–77). The median size of ACCs was 9 cm (IQR 3.5–22 cm) and the median weight 127 g (IQR 18–1400 g). The median follow up period was 18 months (IQR 1–96). Ki67 varied from<1% to 75% (median: 16.4%). The expression of melan-A and lower expression of Ki-67 (≤4) were independently associated with longer OS time (p=0.01 and p=0.04, respectively). In multivariable analysis, tumor volume>400 cm3 (p=0.046), Weiss score>5 (p=0.007) and overexpression of p53 (p=0.036) were independent risk factors for shorter survival. Adrenocortical carcinoma is a rare and very aggressive endocrine malignancy. The most important factors that determine long-term prognosis of ACC are the disease stage at diagnosis, the Weiss score, and the Ki67 index. Immunohistochemical markers such as melan A could also serve as prognostic factors.

Key words

ACC - melan A - p53 - Weiss score - Helsinki score

Supplementary Material



Publication History

Received: 19 April 2021

Accepted after revision: 01 October 2021

Article published online:
05 November 2021

© 2021. Thieme. All rights reserved.

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