Adrenocortical carcinoma and the genetic relationship: a literature review
DOI:
https://doi.org/10.52076/eacad-v3i2.204Keywords:
Adrenocortical carcinoma; Adrenal cortex; Endocrinology; Genetic predisposition to disease; Paraneoplastic endocrine syndromes.Abstract
Introduction: Adrenal tumors are very common, affecting 3% to 10% of the human population, and most are small non-functional benign adrenocortical adenomas (ACA). ACC, in contrast, is a very rare disease. Methodology: This is a systematic literature review of a quantitative type using the following descriptors: Adrenocortical Carcinoma, Adrenal Cortex, Endocrinology, Genetic Predisposition to Disease and Paraneoplastic Endocrine Syndromes. Result: Patients with ACC rarely present with classic tumor symptoms such as cachexia or night sweats. Paraneoplastic syndromes are uncommon. However, tumor-associated hypoglycemia is a well-described phenomenon, historically called Anderson syndrome, which can be attributed to IGF-2-mediated hypoglycemia. Discussion: Proper preoperative assessment and operative planning by a surgeon experienced in the resection of malignant adrenal tumors are of utmost importance to ensure the optimal outcome. Consideration of the surgical anatomy, the potential complications of the surgical intervention, the expected results including recovery time and the various intervention options are important with regard to the beneficial application of surgical management strategies for patients with ACC. Conclusion: While it is true that it is the only drug approved for the treatment of ACC, it also has limited efficacy and may interfere with trials with new therapeutic agents. However, we will await further discussions on the scientific and ethical perspectives of this issue.
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Copyright (c) 2022 João Victor Carvalho Paz; Alexandre Augusto Gomes Alves; Beatriz da Cruz Alves; Camila Melo de Freitas; Amanda de Morais Melo; Denise Ribeiro Guimaraes Borges Salgado; Ana Paula Beatriz Mendes Silva; Vinícius Santos Mendes Restier; Lucas Hewitson Froes Santos ; Maria Laura Nunes Machado de Barros; Danielle Cavalcante Cruz Almeida; Dyego Mondego Moraes; Ana Paula Diniz Alves; Jonhnny Welton Feitosa Melo; Cleison de Souza Avelar
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