Adrenocortical Carcinoma: Updates of Clinical and Pathological Features after Renewed World Health Organisation Classification and Pathology Staging

Abstract

Adrenocortical carcinoma (ACC) is a heterogenous group of diseases with different clinical behaviour between adult and paediatric patients. In addition, three histological variants, oncocytic, myxoid and sarcomatoid are noted on the recent World Health Organisation (WHO) classification of ACC. A review of recent literature showed that the different types of ACC have distinctive demographic data, clinical presentation, pathology, biological behaviour, genomic and patients' prognosis. In addition, recent updates of pathology staging for ACC allow refinement of prognostic grouping for planning treatment of the patients with ACC. These advances in genomic, pathology and staging have driven the development of standardisation of pathology reporting. International standardisation of pathological reporting of adrenocortical carcinoma and adaption to local pathology communities provide universal platforms for clinicians and researchers involved in the management of patients with ACC. To conclude, all these advances in the field of pathology will improve development of management strategies including improvement of clinical care, development of prognostic markers and testing of novel therapeutic approaches for patients with adrenocortical carcinoma.

Keywords: adrenal; adrenocortical carcinoma; myxoid; oncocytic; pathology; sarcomatoid; staging.

Figure 1

Conventional adrenocortical carcinoma (ACC) showing variegated cut surface of yellow tumour with foci of haemorrhages and necrosis (arrows).

Figure 2

Histological features of ACC. (A) Tumour cells (T) with eosinophilic cytoplasm with thick fibrous bands (F). (B) Tumour cells (T) with necrosis (N) (haematoxylin and eosin stain) (scale bar–100 µm).

Figure 3

Adrenocortical carcinoma of high Ki-67 index with high percentage of nuclear stain (brown colour) for Ki-67 (3,3′-diaminobenzidine/haematoxylin stains) (blue arrows) (scale bar—100 µm).

Figure 4

Tumour stain positive (brown colour) for inhibin (3,3′-diaminobenzidine/haematoxylin stains) in cytoplasm of ACC (green arrows). The necrotic area is not stained up (blue arrow). (scale bar—100 µm).

Figure 5

Oncocytic adrenocortical carcinoma showing tan lobular tumour on macroscopic examination. The tumour is rimmed by normal adrenal cortex (yellow colour, arrows).

Figure 6

Oncocytic adrenocortical carcinoma: microscopic examination showing tumour cells with oncocytic (pink) cytoplasm (T) with necrosis (N) (haematoxylin and eosin stain) (scale bar—100 µm).

Figure 7

Survival analysis showing the difference in survival for patients with different types of adrenocortical carcinoma.