Please use this identifier to cite or link to this item: https://hdl.handle.net/2445/222397
Title: Spanish consensus on the diagnosis and management of adrenocortical carcinoma
Author: Araujo Castro, Marta
Álvarez Escolá, Cristina
Casteràs, Anna
Carmona Bayonas, Alberto
Chiara, María Dolores
Hanzu, Felicia A.
Hernando Cubero, Jorge
Vercher Conejero, José Luís
Rodríguez Fraile, Macarena
Gómez Dos Santos, Victoria
Jiménez Fonseca, Paula
Giraldo, Alexandra
Valdés, Nuria
Vidal, Oscar
Olmo García, Maribel del
Capdevila, Jaume
Keywords: Tractament adjuvant del càncer
Malalties de les glàndules suprarenals
Adjuvant treatment of cancer
Adrenal glands diseases
Issue Date: 11-Apr-2025
Publisher: Bioscientifica
Abstract: 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).
Note: Reproducció del document publicat a: https://doi.org/10.1530/ERC-25-0034
It is part of: Endocrine Related Cancer, 2025, vol. 32, num. 5
URI: https://hdl.handle.net/2445/222397
Related resource: https://doi.org/10.1530/ERC-25-0034
Appears in Collections:Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))
Articles publicats en revistes (IDIBAPS: Institut d'investigacions Biomèdiques August Pi i Sunyer)

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