The American Association of Endocrine Surgeons, Patient Education Site

Adrenocortical carcinoma
(adrenal cancer)

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What is the chance that this is genetic?

Most adrenocortical carcinoma (ACC) are sporadic (i.e. they are NOT due to an inherited genetic problem). However, adrenocortical carcinoma can occur in families with two specific genetic syndromes: Li-Fraumeni and Beckwith-Wiedemann Syndrome.

Li-Fraumeni Syndrome is caused by a mutation in a gene that normally prevents tumor growth called TP53. Patients with Li-Fraumeni syndrome have a higher risk of many cancers including breast cancer, soft tissue sarcomas, brain tumors, osteosarcoma, leukemia, and ACC. Many physicians believe than any child with ACC should undergo testing to determine if they have this gene mutation. In adults without a family history of ACC, TP53 may be mutated in up to 25% of tumors. In these non-genetic cases, even if the mutation is present, it cannot be passed on to children. As a result, most patients without a known family history of Li-Fraumeni Syndrome do not need to be tested for TP53 mutations.

Beckwith-Wiedemann Syndrome is caused by a mutation in the gene that controls production of a protein insulin-like growth factor 2 (IGF2). IGF2 overproduction can also be found in sporadic (non-inherited) ACC. Researchers believe that too much IGF2 causes tumor cells to grow abnormally. Several clinical trials are in progress to treat ACC with drugs that block this biochemical pathway. At this time, most patients without a known family history Beckwith-Wiedemann Syndrome do not need to be tested for these mutations.

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