Giant nonfunctioning adrenal tumors: two case reports and review of the literature

Abstract

BackgroundThere are an estimated 1–2 cases per million per year of adrenocortical carcinoma in the USA. It represents a rare and aggressive malignancy; it is the second most aggressive endocrine malignant disease after anaplastic thyroid carcinoma. Non-secretory adrenal masses are diagnosed late due to a mass effect or metastatic disease or found incidentally (adrenal incidentalomas).Case presentationThe first case report describes a 39-year-old Greek woman who presented to our department with complaints of repeated symptoms of flatulence and epigastric discomfort over a few months. The second case report is about a 67-year-old Greek woman who presented to our department after being evaluated for fatigue, mass effect, and epigastric discomfort. Both of them were diagnosed as having a nonfunctioning adrenocortical carcinoma and underwent open adrenalectomy.ConclusionsApproximately 60% of patients with adrenocortical carcinoma present with symptoms and signs of hormonal secretion. Our cases’ adrenocortical carcinomas were not functional. Hormone secretion is not a discriminating feature between benign and malignant adrenocortical masses. The silent clinical nature of nonfunctioning adrenocortical carcinoma results in late diagnosis, while the majority of patients present with locally advanced and/or metastatic disease.Adrenocortical carcinoma is a rare endocrine tumor with a poor prognosis that can be diagnostically challenging and demands high clinical suspicion. The work-up for adrenal masses must include determination of whether the mass is functioning or nonfunctioning and whether it is benign or malignant.

Diagnosis of adrenocortical carcinoma with hypercortisolism in a patient presenting with hypokalemic metabolic alkalosis

“…Surveillance includes CT of the chest and abdomen every 3 months along with monitoring of steroid hormones. 8 Ultrasound has shown clinical significance during follow up of selected patients. 9 …”

Preoperative Transcatheter Arterial Embolization and En-Bloc Resection for Giant Non-Functioning Left Adrenocortical Carcinoma: A Case Report

“…Extension of ACC to surrounding structures and absence of a clear cleavage at preoperative diagnostic work-up should not represent a contraindication to an aggressive surgical approach: several experiences dealing with giant ACCs treated with multivisceral removal have been reported with satisfactory results [8,10,[12][13][14][15]. This surgical attitude has also been supported by larger consecutive series: data from the U.S. Adrenocortical Carcinoma Database show that 68 among 167 patients (40.7%) underwent adrenalectomy with some en-bloc resection of additional organs (AdEBR) and 26 patients among them had multiple organs removed (mainly kidney, left liver lobe, spleen, pancreatic tail); in this subgroup larger tumor (13 vs. 10 cm.…”

A case report on adrenocortical carcinoma

“…Adrenocortical carcinoma is a rare entity (incidence of 2 per 1 million). 2 It can occur at any age But bimodal distribution is more commonly observed in the first and fourth decades. Approximately 60% are functional with hormone overproduction.…”

“…1 It is the second most aggressive endocrine malignancy following anaplastic carcinoma of the thyroid. 2 A female predilection has been observed in the pattern of cases, with a bimodal age distribution. 60% tumours are functioning with Cushing's syndrome being the most common manifestation Followed by cushing's and virilisation syndrome.…”