Li-Fraumeni Syndrome Treatment & Management

Updated: Mar 02, 2016
  • Author: ; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Treatment

Medical Care

No clear evidence suggests that individuals with Li-Fraumeni syndrome (LFS) diagnosed with cancers should be treated differently from other patients with cancer through the modalities of chemotherapy or surgery. Radiation therapy should be used with caution owing to concerns about increased risk for radiation-induced second primary tumors in Li-Fraumeni syndrome patients. Specifics of therapy are related to the type of cancer.

Based on expert opinions and limited evidence, the National Comprehensive Cancer Network (NCCN) has provided an algorithm for the testing and management of Li-Fraumeni syndrome in adults.

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Surgical Care

Physicians should discuss risk-reducing, prophylactic mastectomy with Li-Fraumeni syndrome women. It should be considered on a case-by-case basis depending on the degree of cancer risk and reconstructive options available.

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Consultations

Genetic counseling for families with Li-Fraumeni syndrome should be provided to ensure appropriate understanding of potential risk and possible evaluation of genetic predisposition markers.

In addition, Li-Fraumeni syndrome patients can develop high levels of anxiety, depression, and clinically relevant distress. Psychological monitoring is important, and appropriate support should be provided.

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