Abstract
Introduction: Adrenocortical carcinoma (ACC) is a rare tumor, often discovered at an advanced stage and associated with poor prognosis. Treatment is guided by staging according to the European Network for the Study of Adrenal Tumors (ENSAT) classification. Surgery is the treatment of choice for ACC. The aim of this review is to provide a complete overview on surgical approaches and management of adrenocortical carcinoma. Methods: This comprehensive review has been carried out according to the PRISMA statement. The literature sources were the databases PubMed, Scopus and Cochrane Library. The search thread was: ((surgery) OR (adrenalectomy)) AND (adrenocortical carcinoma). Results: Among all studies identified, 17 were selected for the review. All of them were retrospective. A total of 2498 patients were included in the studies, of whom 734 were treated by mini-invasive approaches and 1764 patients were treated by open surgery. Conclusions: Surgery is the treatment of choice for ACC. Open adrenalectomy (OA) is defined as the gold standard. In recent years laparoscopic adrenalectomy (LA) has gained more popularity. No significant differences were reported for overall recurrence rate, time to recurrence, and cancer-specific mortality between LA and OA, in particular for Stage I-II. Robotic adrenalectomy (RA) has several advantages compared to LA, but there is still a lack of specific documentation on RA use in ACC.
Highlights
Adrenocortical carcinoma (ACC) is a rare malignancy with an estimated incidence of1–2 cases per million persons annually [1,2,3,4,5]
The suspicions of ACC for an adrenal lesion are driven by tumor size (>6 cm), radiological signs of malignancy of 18 FDG-PET uptake, presence of local invasion or distant metastases and typical hormonal secretions
Surgery is the treatment of choice for ACC (Stage I–III) whereas for Stage IV
Summary
1–2 cases per million persons annually [1,2,3,4,5]. It is more frequent in women than in men, with an average age at presentation in the fifth to seventh decades [6,7,8]. But not constant, adrenocortical hormone production (mainly cortisol), the clinical presentation of ACC is extremely variable, and most patients (80%) [13] are asymptomatic at the time of diagnosis. Symptomatic ACC may be diagnosed during clinical evaluation of hypercortisolism, hyperandrogenism, or due to “mass effect”
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