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.

Highlights

  • There are an estimated 1–2 cases per million per year of adrenocortical carcinoma in the USA

  • Nonfunctioning adrenocortical carcinoma (ACC) is correlated with a poor prognosis due to the late diagnosis, local invasion, or recurrence and distant metastases [1, 3, 4]

  • We present the cases of two women who were referred to our Department of surgery and were diagnosed as having a large nonfunctioning ACC

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Summary

Conclusions

ACC is a rare and aggressive malignancy. Non-secretory adrenal masses are diagnosed late by a mass effect, metastatic disease, or found incidentally. We present the cases of two women who presented to our department after being evaluated for fatigue, mass effect, and epigastric discomfort. Both of them were diagnosed as having a nonfunctioning ACC and underwent open adrenalectomy. IP wrote the manuscript and collected the data. PS is the director of the Department of Surgery and the consultant surgeon who provided the cases. All authors read and approved the final manuscript. Ethics approval and consent to participate Our case report obtained ethics approval from the ethics committee of our hospital and the patients gave their informed consent to participate. Consent for publication Written informed consent was obtained from the patients for publication of this case report and any accompanying images. Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations

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