Sistema de Salut de Catalunya
Institut Català de la Salut
[Araujo-Castro M] Endocrinology & Nutrition Department, Hospital Universitario Ramón y Cajal Madrid, Madrid, Spain. Instituto de Investigación Biomédica Ramón y Cajal (IRYCIS), Madrid, Spain. [Álvarez-Escola C] Endocrinology & Nutrition Department, Hospital Universitario La Paz, Madrid, Spain. [Casteràs A] Servei d’Endocrinologia i Nutrició, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Carmona-Bayonas A] Medical Oncology Department, Hospital General Universitario Morales Meseguer, University of Murcia, IMIB, Murcia, Spain. [Chiara MD] Health Research Institute of the Principality of Asturias, Oviedo, Spain. Institute of Oncology of the Principality of Asturias, University of Oviedo, Oviedo, Spain. [Hanzu FA] Endocrinology & Nutrition Department, Hospital Clinic, University of Barcelona, IDIBPAS, CIBERDEM, Barcelona, Spain. [Hernando J, Capdevila J] Servei d’Oncologia Mèdica, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Gastrointestinal and Endocrine Tumor Unit, Vall Hebron Institute of Oncology (VHIO), Barcelona, Spain. [Giraldo A] Servei d’Oncologia Radioteràpica, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Vall Hebron Institute of Oncology (VHIO), Barcelona, Spain
Vall d'Hebron Barcelona Hospital Campus
2025-06-04T06:23:14Z
2025-06-04T06:23:14Z
2025-05
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).
Article
Published version
English
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
Bioscientifica
Endocrine-Related Cancer;32(5)
https://doi.org/10.1530/ERC-25-0034
Attribution 4.0 International
http://creativecommons.org/licenses/by/4.0/
Articles científics - HVH [3437]
