2026-04-17T03:51:52Zhttps://recercat.cat/oai/request

oai:recercat.cat:11351/131962025-06-14T04:13:46Zcom_2072_378070com_2072_378040col_2072_378092

Spanish consensus on the diagnosis and management of adrenocortical carcinoma Chiara, María-Dolores Hanzu, Felicia Alexandra Hernando, Jorge Araujo Castro, Marta Alvarez-Escola, Cristina Casteràs, Anna Carmona-Bayonas, Alberto Giraldo Marin, Alexandra Capdevila Castillon, Jaume Decisió, Presa de Glàndules suprarenals - Càncer - Tractament Glàndules suprarenals - Càncer - Diagnòstic DISEASES::Neoplasms::Neoplasms::Neoplasms by Site::Endocrine Gland Neoplasms::Adrenal Gland Neoplasms::Adrenal Cortex Neoplasms::Adrenocortical Carcinoma Other subheadings::Other subheadings::/diagnosis DISEASES::Neoplasms::Neoplasms::Neoplasms by Site::Endocrine Gland Neoplasms::Adrenal Gland Neoplasms::Adrenal Cortex Neoplasms::Adrenocortical Carcinoma Other subheadings::Other subheadings::/therapy PSYCHIATRY AND PSYCHOLOGY::Behavior and Behavior Mechanisms::Psychology, Social::Group Processes::Consensus ENFERMEDADES::neoplasias::neoplasias::neoplasias por localización::neoplasias de las glándulas endocrinas::neoplasias de las glándulas suprarrenales::neoplasias de la corteza suprarrenal::carcinoma corticosuprarrenal Otros calificadores::Otros calificadores::/diagnóstico ENFERMEDADES::neoplasias::neoplasias::neoplasias por localización::neoplasias de las glándulas endocrinas::neoplasias de las glándulas suprarrenales::neoplasias de la corteza suprarrenal::carcinoma corticosuprarrenal Otros calificadores::Otros calificadores::/terapia PSIQUIATRÍA Y PSICOLOGÍA::conducta y mecanismos de la conducta::psicología social::procesos de grupo::consenso Adrenalectomy; Adrenocortical carcinoma; Mitotane Adrenalectomia; Carcinoma adrenocortical; Mitotà Adrenalectomía; Carcinoma adrenocortical; Mitotano Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with an estimated incidence of 0.7-2 cases per million/year. The rarity of this disease, coupled with limited preclinical models and clinical trials, has hindered progress, resulting in poor outcomes, with a 5-year survival rate of approximately 35%. Currently, the only available curative treatment is complete surgical resection of the adrenal tumor. For unresectable or metastatic ACC, the current standard therapeutic modalities are mitotane, chemotherapy, radiotherapy and locoregional treatments; however, these are noncurative. Mitotane has an adrenolytic and anti-steroidogenic effect, and it is used in the adjuvant setting for high-risk patients, as systemic therapy for metastatic disease, and/or to control hormonal secretion. While key pathways in ACC pathogenesis have been identified as potential therapeutic targets, results with targeted therapies remain modest, showing that there is a clinical unmet need for novel treatments or new combinations of exiting drugs. Effective management requires a multidisciplinary team of experts to optimize outcomes for patients. This article presents a multidisciplinary consensus on the diagnosis, management, prognosis and follow-up of patients with ACC, and the approach to two special contexts, ACC in pregnant women and hormone-producing ACC. The consensus was coordinated by the Spanish Society of Endocrinology and Nutrition (SEEN) and the Spanish Group of Neuroendocrine and Endocrine Tumors (GETNE), with contribution from experts from related societies including the Spanish Association of Surgeons (AEC), Spanish Society of Urology (AEU), Anatomic-Pathology (SEAP), Nuclear Medicine (SEMNIM), Medical Oncology (SEOM) and Radiotherapeutic Oncology (SEOR). This work was funded by Sociedad Española de Endocrinología y Nutrición (SEEN) and the Spanish Group of Neuroendocrine and Endocrine Tumors (GETNE). 2025-06-14T04:13:46Z 2025-06-14T04:13:46Z 2025-06-14T04:13:46Z 2025-06-04T06:23:14Z 2025-06-04T06:23:14Z 2025-05 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion http://hdl.handle.net/11351/13196 Endocrine-Related Cancer;32(5) https://doi.org/10.1530/ERC-25-0034 Attribution 4.0 International http://creativecommons.org/licenses/by/4.0/ info:eu-repo/semantics/openAccess Bioscientifica Scientia