European Society of Endocrine Surgeons (ESES) and European Network for the Study of Adrenal Tumours (ENSAT) recommendations for the surgical management of adrenocortical carcinoma

Abstract

Background: Radical surgery provides the best chance of cure for adrenocortical carcinoma (ACC), but perioperative surgical care for these patients is yet to be standardized. Methods: A working group appointed jointly by ENSAT and ESES used Delphi methodology to produce evidence-based recommendations for the perioperative surgical care of patients with ACC. Papers were retrieved from electronic databases. Evidence and recommendations were classified according to the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) system, and were discussed until consensus was reached within the group. Results: Twenty-five recommendations for the perioperative surgical care of patients with ACC were formulated. The quality of evidence is low owing to the rarity of the disease and the lack of prospective surgical trials. Multi-institutional prospective cohort studies and prospective RCTs are urgently needed and should be strongly encouraged. Conclusion: The present evidence-based recommendations provide comprehensive advice on the optimal perioperative care for patients undergoing surgery for ACC.

European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors

“…Complete resection is of utmost importance for all ACCs and successful surgery is a prerequisite for cure. As the diagnosis of ACC might only become apparent after histological analysis, it remains imperative for all adrenalectomies (laparoscopic or open) in patients with a reasonable suspicion for ACC to respect the principles of oncological surgery in order to ensure complete resection (R0 status) (185,186).…”

Treatment and management of adrenal cancer in a specialized Australian endocrine surgical unit: approaches, outcomes and lessons learnt

“…Our approach to advanced disease was aggressive: complete en bloc surgical resection where feasible which is supported by the international literature . There is evidence that complete resection of primary adrenal disease confers survival benefits even in the presence of metastatic disease .…”

“…It is an orphan cancer for which outcomes and survival rates have not improved in the past decades. Many of the guidelines for diagnosis and management arise from European and American centres, that have the advantage of analysing large aggregated numbers of patients. The European Society of Endocrinology recently published consensus guidelines advocating for en bloc resection of suspected ACC with locoregional lymphadenectomy in localized disease.…”

Surgical resection of adrenocortical carcinoma with invasion into the inferior vena cava: a case report and literature review

“…They drew a conclusion that complete resection of ACC with IVC tumor thrombus seemed justified as the survival was similar to the patients without IVC tumor thrombus. The clinical guideline for ACC recommends that the surgery should be performed by surgeons with expertise in adrenal surgery (open and laparoscopic) and with a volume of more than 15 adrenalectomies per year (benign and malignant) . In our case, the patient underwent radical surgery and reconstruction of the IVC with the use of cardiopulmonary bypass.…”