Abstract
Adrenal cortical carcinoma is a rare endocrine tumor for which complete surgical resection is the only potentially curative treatment. Accurate preoperative evaluation (biochemical and radiographic) of the patient who presents with an adrenal mass maximizes the opportunity for the patient to undergo a complete, margin-negative resection of the primary tumor, which is the most powerful prognostic variable for long-term survival. The response to chemotherapy or mitotane is modest in patients with advanced disease. Hopefully, an improved understanding of the molecular pathogenesis of this challenging tumor will lead to the development of more effective therapies in the future.
Résumé
Le cancer de la corticosurrénale est une tumeur endocrine rare pour laquelle seule la résection chirurgicale complète offre la possibilité de guérison. C’est par une évaluation préopératoire précise (biochimique et radiographique) d’un patient présentant une masse de la surrénale qu’on s’assure d’une résection complète, avec des marges libres, ce qui est le facteur pronostique le plus puissant pour évaluer la survie à long terme. La réponse à la chimiothérapie ou au mitotane est modeste chez la plupart de patients ayant une maladie avancée. On espère que grâce à une compréhension de la pathogenèse moléculaire de cette tumeur, on pourra développer de nouvelles thérapies plus efficaces dans l’avenir.
Resumen
El carcinoma adrenocortical es un tumor endocrino raro para el cual la resección quirúrgica total es la única modalidad terapéutica potencialmente curativa. La debida evaluación preoperatoria (bioquímica y radiográfica) del paciente que presenta una masa suprarrenal maximiza la posibilidad de lograr la resección completa del tumor primario con márgenes libres, que es la variable pronóstica de mayor importancia en lo relativo a supervivencia a largo plazo. La respuesta a la quimioterapia o al mitotane es modesta en los casos de enfermedad avanzada. Se guarda la esperanza de que una mejor comprensión de la patogénesis molecular de este tumor lleve al desarrollo de nuevas y más efkaces formas de tratamiento.
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Published Online: June 27, 2001
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Dackiw, A.P.B., Lee, J.E., Gagel, R.F. et al. Adrenal cortical carcinoma. World J. Surg. 25, 914–926 (2001). https://doi.org/10.1007/s00268-001-0030-7
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DOI: https://doi.org/10.1007/s00268-001-0030-7