Adrenocortical carcinoma is a malignant tumor with a poor prognosis and a frequent metastatic extension. In very rare cases, a cardiac metastatic disease may occur, and surgical resection is essential for its management. MR-guided stereotactic radiotherapy is an attractive radiotherapy modality for the treatment of mobile thoracic tumors, enabling the target to be monitored continuously during irradiation, while the dosimetric plan can be adapted daily if necessary. We report here the case of a patient with intracardiac metastasis secondary to malignant adrenocortical carcinoma, treated with magnetic resonance imaging-guided stereotactic radiotherapy.

The adrenocortical carcinoma (ACC) is a primary malignant tumor developed from the adrenal cortex, defined by a Weiss score≥3. Its prognosis is poor and depends mainly on the stage of the disease at diagnosis. Care is organized in France by the multidisciplinary expert centers of the national ENDOCAN-COMETE \"Adrenal Cancers\" network, certified by the National Cancer Institute. This document updates the guidelines for the management of ACC in adults based on the most robust data in the literature. It\'s divided into 11 chapters: (1) circumstances of discovery; (2) pre-therapeutic assessment; (3) diagnosis of ACC; (4) oncogenetics; (5) prognostic classifications; (6) treatment of hormonal hypersecretion; (7) treatment of localized forms; (8) treatment of relapses; (9) treatment of advanced forms; (10) follow-up; (11) the particular case of ACC and pregnancy. R0 resection of all localized ACC remains an unmet need and it must be performed in expert centers. Flow-charts for the therapeutic management of localized ACC, relapse or advanced ACC are provided. It was written by the experts from the national ENDOCAN-COMETE network and validated by all French Societies involved in the management of these patients (endocrinology, medical oncology, endocrine surgery, urology, pathology, genetics, nuclear medicine, radiology, interventional radiology).

BACKGROUND: The objective of this publication is to recall the initial work-up when faced with an adrenal incidentaloma and, if necessary, to establish the oncological management of an adrenal malignant tumor.
METHODS: The multidisciplinary working group updated French urological guidelines about oncological assessment of the adrenal incidentaloma, established by the CCAFU in 2020, based on an exhaustive literature review carried out on PubMed.
RESULTS: Although the majority of the adrenal masses are benign and non-functional, it is important to investigate them, as a percentage of these can cause serious endocrine diseases or be cancers. Malignant adrenal tumors are mainly represented by adrenocortical carcinomas (ACC), malignant pheochromocytomas (MPC) and adrenal metastases (AM). The malignancy assessment of an adrenal incident includes a complete history, a physical examination, a biochemical/hormonal assessment to look for subclinical hormonal secretion. Diagnostic hypotheses are sometimes available at this stage, but it is the morphological and functional imaging and the histological analysis, which will make it possible to close the malignancy assessment and make the oncological diagnosis.
CONCLUSIONS: ACC and MPC are mainly sporadic but a hereditary origin is always possible. ACC is suspected preoperatively but the diagnosis of certainty is histological. The diagnosis of MPC is more delicate and is based on clinic, biology and imagery. The diagnosis of certainty of AM requires a percutaneous biopsy. At the end, the files must be discussed within the COMETE - adrenal cancer network (Appendix 1).

BACKGROUND: - The objective of this publication is to recall the initial oncological management of adrenal incidentalomas.
METHODS: - The multidisciplinary working group updated french urological guidelines established by the CCAFU in 2018, based on an exhaustive literature review carried out on PubMed.
RESULTS: - Although the majority of the adrenal masses are benign and non-functional, it is important to investigate them, as a percentage of these can cause serious endocrine diseases or be cancers. Malignant adrenal tumors are mainly represented by Adrenocortical Carcinomas (ACC), malignant pheochromocytomas (MPC) and adrenal metastases (AM). The malignancy assessment of an adrenal incident includes a complete history, a physical examination, a biochemical / hormonal assessment to look for subclinical hormonal secretion. Diagnostic hypotheses are sometimes available at this stage, but it is the morphological and functional imaging and the histological analysis which will make it possible to close the malignancy assessment and make the oncological diagnosis.
CONCLUSIONS: - AC and MPC are mainly sporadic but a hereditary origin is always possible. ACC is suspected preoperatively but the diagnosis of certainty is histological. The diagnosis of MPC is more delicate and is based on clinic, biology and imagery. The diagnosis of certainty of AM requires a percutaneous biopsy. At the end, the files must be discussed within the COMETE - adrenal cancer network (Appendix 1).

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.

This article has been retracted: please see Elsevier Policy on Article Withdrawal (http://www.elsevier.com/locate/withdrawalpolicy). Cet article est retiré de la publication à la demande des auteurs car ils ont apporté des modifications significatives sur des points scientifiques après la publication de la première version des recommandations. Le nouvel article est disponible à cette adresse: doi:10.1016/j.purol.2019.01.011. C’est cette nouvelle version qui doit être utilisée pour citer l’article. This article has been retracted at the request of the authors, as it is not based on the definitive version of the text because some scientific data has been corrected since the first issue was published. The replacement has been published at the doi:10.1016/j.purol.2019.01.011. That newer version of the text should be used when citing the article.

BACKGROUND: Malignant tumours of the adrenal gland are adrenocortical carcinomas (ACC), malignant phaeochromocytomas (MPC) or metastatic tumours. The objective of this article is to propose guidelines for the management of these tumours.
METHODS: A review of the literature was performed by selecting articles on malignant tumours of the adrenal gland published in PUBMED.
RESULTS: Abdominal computed tomography is the reference first-line examination. A diameter > 6 cm, a heterogeneous appearance, irregular margins, spontaneous high density (> 20 HU) and delayed wash-out are radiological signs of malignancy. MRI can be used to characterize the tumour with a sensitivity of 89% and a specificity of 99%. Hormone assays and an endocrinology consultation are recommended before any management. When ACC is suspected, (18)FDG-PET is the reference scintigraphic examination, while the isotope of choice for MPC is (18)F-DOPA, which is more sensitive than MIBG. These scintigraphic examinations have a sensitivity close to 100% and allow staging of distant metastases. Percutaneous biopsy has a limited place in the diagnostic work-up. It is only indicated in the case of suspected adrenal metastasis after having excluded phaeochromocytoma and must not be performed in the case of suspected adrenocortical carcinoma. Surgery is first-line treatment for localized and resectable tumours, but is rarely curative due to the high recurrence rate. For ACC, adjuvant therapy by mitotane or adjuvant radiotherapy can be proposed. Metabolic radiotherapy with (131)I-MIBG can be proposed for the treatment of MPC. First-line chemotherapy is indicated in the case of advanced disease or unresectable tumour. Surgical treatment of adrenal metastasis by adrenalectomy is recommended depending on the type and prognosis of the primary cancer.
CONCLUSIONS: Preoperative laboratory, morphological and scintigraphic assessment is essential before any management. First-line treatment is surgical when the tumour is resectable, but must be completed by adjuvant therapy to limit the risk of recurrence.