The Unique Case of Adrenocortical Malignant and Functioning Oncocytic Tumour

Abstract

elevated blood pressure (180/120 mmHg), no features typical of Cushing's syndrome. Abnormal laboratory findings: oral glucose tolerance test revealed diabetes, elevated serum dehydroepiandrosterone-sulfate (1101.9 microg/dl; normal, 59-452), elevated serum cortisol following overnight 1 mg dexamethasone test (5.1 microg/dl; normal, <1.8), increased urinary excretion of 17- hydroxycorticosteroids (18.1 mg/24 h; normal, 2.0-7.0) with pathological response to high-dose dexamethason test (16.6 mg/24). On laparotomy, the lesion was considered unresectable because of evident - confirmed by intraoperative ultrasound - tumour infiltration of the inferior caval vein. The large biopsy specimen was obtained for histological examination in which tumour fulfilled criteria proposed by Bisceglia et al. for adrenocortical oncocytic borderline tumour. On immunohistochemistry, the lesion showed cytoplasmic reaction for cytokeratin, vimentin and synaptophysin. The presented case appears to be the first malignant and functioning adrenocortical oncocytic tumour reported and confirms the complexity of its biology.

Adrenal Oncocytic Neoplasm: A Systematic Review

“…According to the currently used classification, there are three histological categories: pure and benign oncocytoma, oncocytic neoplasm of uncertain malignant potential [36][37][38] and oncocytic carcinoma [39][40][41][42] .…”

Imaging appearance of bulk fat within an oncocytic adrenocortical neoplasm, a rare and potentially malignant tumour

“…In such cases, the lesion has been designated by some authors as an adrenocortical oncocytoma [4,5,9,11], analogous to the oncocytoma that is recognised in the kidney and salivary, thyroid and parathyroid glands [7]. However, oncocytic adrenocortical neoplasms have also presented in some cases as carcinomas with a malignant clinical course; for example, demonstrating invasion of the inferior vena cava [8,[12][13][14], local recurrence following resection [15,16] and bone, liver and lung metastases [15], with death as a reported outcome [17]. Thus, the major challenge in the management of these neoplasms is to distinguish benign from malignant lesions.…”

“…To assist in their categorisation, Bisceglia et al [17] proposed a classification system that divided oncocytic adrenocortical neoplasms into three broad groups on the basis of histological features with different prognostic implications; however, given the small number of lesions reported, as well as the short clinical follow-up available for most lesions, this classification system is considered controversial [15] and caution is still warranted when applying histological criteria to individual lesions [17]. Indeed, some reported cases in the literature have demonstrated a more aggressive clinical course than would be predicted using this classification system [8,12,13,17]. Until these lesions are better understood, long-term follow-up of oncocytic adrenocortical neoplasms is suggested [4,5].…”

Oncocytic adrenal cortical tumor with cytoplasmic inclusions and hyaline globules

“…Gradations of oncocyte-like features can be seen focally or diffusely in other tumors, and oncocytic carcinomas, both functional [1,11] and nonfunctional, have been reported. The present case is an unusual example of an apparently benign, clinically nonfunctional tumor that diffusely exhibited both well-developed oncocytic features and steroidogenic features in the same cells.…”