[French ccAFU guidelines - Update 2018-2020: Adrenal cancer]

To update French oncology guidelines concerning adrenal cancer.

Comprehensive Medline search between 2016 and 2018 upon diagnosis, treatment and follow-up of adrenal cancer to update 2013 guidelines. Level of evidence was evaluated according to AGREE-II.

Adrenal cancers are mainly represented by adrenocortical carcinomas (AC), malignant pheochromocytomas (MPC) and adrenal metastases (AM). Medical background of these tumors is either the exploration of hormonal or tumor symptoms, or an adrenal incidentaloma. Etiological explorations are based on hormonal biochemical assessment, morphological and functional imaging and histological analysis. AC and MPC are mostly sporadic but hereditary origin is still possible. The suspicion of AC is driven mainly by radiological signs of malignancy, signs of local invasion or distant metastasis, and type of hormonal secretion but the accurate diagnosis is histological. The diagnosis of MPC is clinical, biological and radiological. The diagnosis of MS involves a percutaneous biopsy. Medical files for primitive adrenal cancer should be discussed within the COMETE - Adrenal Cancer Network (Appendix 1). Oncological adjuvant treatments are specific for the histological type. In the AC, their indication depends on the risk of recurrence and is based on mitotane, external radiotherapy or chemotherapy. In the MPC, it is based on internal radiotherapy and chemotherapy. Metastatic forms treatment is exceptionally surgical. Debulking is uncommon. For metastatic unresectable AC, treatment is based on mitotane monotherapy or triple chemotherapy. For metastatic unresectable MPC, treatment is based on exclusive metabolic radiotherapy or triple chemotherapy. Recurrences are frequent and sometimes delayed, which justifies a close and long follow-up.

The curative treatment of Adrenal cancers is surgical provided. This treatment is rarely sufficient alone, the prognosis is then pejorative.