AN UNUSUAL SITE OF ADRENOCORTICAL CARCINOMA

Abstract

INTRODUCTION/BACKGROUND
Adrenocortical carcinoma (ACC) is a rare malignancy with an incidence of 0.5–2 cases per million per year. Typically, ACC originates in the adrenal glands. Although exceedingly rare, ectopic presentations can occur due to developmental anomalies and rarely may arise from an adrenal rest. These adrenal rests are usually clinically silent, but on rare occasions, may undergo malignant transformation and hormonal secretion.

CASE
We report the case of a 33-year-old female with underlying hypertension and diabetes who had an incidentally discovered right adnexal mass which was asymptomatic during a routine medical checkup. She underwent complete laparoscopic tumour resection without complications. Comprehensive histopathologic evaluation revealed a low-grade ectopic ACC arising from an adrenal rest. Postoperative imaging demonstrated no residual tumor and normal adrenal glands. She remains under active surveillance.

The case highlights the diagnostic challenge posed by an ectopic ACC masquerading as an adnexal mass. Detailed histopathologic and immunohistochemical analyses are essential in accurately determining tumor origin, thus guiding optimal management strategies. Adrenal rests have been described within the retroperitoneum, broad ligament, testis, ovaries and inguinal region. Due to limited data, the management of ectopic ACC is generally considered similar to that of eutopic tumors. Complete surgical resection is still the mainstay of treatment for both eutopic and ectopic ACC. Long-term follow-up and close monitoring are imperative given the risk of recurrence.

CONCLUSION
This case underscores the importance of maintaining a high index of suspicion, as many ectopic adrenocortical rests are under-recognized due to their small size and low clinical relevance. Awareness of ectopic adrenal rests is crucial to correctly identify sources of adrenocortical hormone production, avoid misinterpretations in the diagnostic workup of intraabdominal masses, and to evaluate for possible malignant transformation.