Radical surgical management of Li-Fraumeni syndrome: a case report
DOI:
https://doi.org/10.34119/bjhrv8n4-045Keywords:
Li-Fraumeni syndrome, vertical rectus abdominis myocutaneus flap, osteosarcomaAbstract
Li-Fraumeni syndrome is a condition associated with TP53 gene mutation, and its associated with the early development of multiple neoplasias. There are criteria for suspicion and clinical diagnosis; however, a definitive diagnosis requires confirmation of the genetic mutation through molecular testing. The treatment of neoplasias in these patients is a challenge because chemotherapy and radiotherapy usually used in adjuvant or neoadjuvant therapy increase the risk of a second primary cancer, this limits therapeutic options and cases must be discussed in multidisciplinary sessions for adequate management. Genetic counseling is especially important for secondary and primary prevention in family groups. The present case reports an adult man with three metacronic neoplasms treated surgically with a reconstructive technique that was adapted to the patient's context and comprehensive management was based on decisions of a multidisciplinary team.
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