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Endocrine. 2019 Apr 12. doi: 10.1007/s12020-019-01918-9. [Epub ahead of print]

Adrenocortical carcinoma: presentation and outcome of a contemporary patient series.

Author information

1
Endocrinology, Abdominal Center, Helsinki University Hospital and University of Helsinki, Helsinki, Finland.
2
Department of Surgery, Helsinki University Hospital and University of Helsinki, Helsinki, Finland.
3
HUS Medical Imaging Centre, Radiology, Helsinki University Hospital and University of Helsinki, Helsinki, Finland.
4
Children's Hospital, Helsinki University Hospital and University of Helsinki, Helsinki, Finland.
5
Department of Biostatistics, University of Turku and Turku University Hospital, Turku, Finland.
6
Department of Pathology, University of Helsinki and HUSLAB, Helsinki, Finland.
7
Endocrinology, Abdominal Center, Helsinki University Hospital and University of Helsinki, Helsinki, Finland. camilla.schalin-jantti@hus.fi.

Abstract

BACKGROUND:

Adrenocortical carcinoma (ACC) is a rare endocrine carcinoma with poor 5-year survival rates of < 40%. According to the literature, ACC is rarely an incidental imaging finding. However, presentation, treatment and outcome may differ in modern series.

DESIGN AND METHODS:

We studied all patients (n = 47, four children) from a single centre during years 2002-2018. We re-evaluated radiologic and histopathological findings and assessed treatments and outcome. We searched for possible TP53 gene defects and assessed nationwide incidence of ACC.

RESULTS:

In adults, incidental radiologic finding led to diagnosis in 79% at median age of 61 years. ENSAT stage I, II, III and IV was 19%, 40%, 19% and 21%, respectively. Nonenhanced CT demonstrated > 20 Hounsfield Units (HU) for all tumours (median 34 (21-45)), median size 92 mm (20-196), Ki67 17% (1-40%), Weiss score 7 (4-9) and Helsinki score 24 (4-48). ACC was more often found in the left than the right adrenal (p < 0.05). One child had Beckwith-Wiedemann and one a TP53 mutation. In adults, the primary tumour was resected in 88 and 79% received adjuvant mitotane therapy. Median hospital stay was significantly shorter in the laparoscopic vs. open surgery group (4 (3-7) vs. 8 (5-38) days, respectively; p < 0.001). In 3/4 patients, prolonged remission of > 5 to > 10 years was achieved after repeated surgery of metastases. Overall 5-year survival was 67%, and 96% vs. 26% for ENSAT stage I-II vs. III-IV (p < 0.0001). ENSAT stage and Ki67 predicted survival, type of surgery did not. Mitotane associated with better survival.

CONCLUSIONS:

Contemporary ACC predominantly presents as an incidental imaging finding, characterised by HU > 20 on nonenhanced CT but variable tumour size (20-196 mm). Malignancy cannot be ruled out by small tumour size only. The 5-year survival of 96% in ENSAT stage I-III compares favourably to previous studies.

KEYWORDS:

Adrenocortical carcinoma; Complications; ENSAT stage; Hounsfield units; Mitotane; Surgery; Survival

PMID:
30980285
DOI:
10.1007/s12020-019-01918-9

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